The New-born: A Practical Guide: Paediatric Problems in General Practice 9781487578381

Citation preview

Paediatric Problems In General Practice THE NEW-BORN

Paediatric Problems in General Practice

THE NEW-BORN

A practical guide

J.B. J. McKENDRY B.A.,M.D.

Assistant Physician, Hospital for Sick Children, Toronto Associate in the Department of Paediatrics University of Toronto

J. D. BAILEY M.D., F.R.C.P.(C.)

Physician, Hospital for Sick Children, Toronto Assistant Professor of Paediatrics University of Toronto

UNIVERSITY

OF

TORONTO

PRESS

@ UNIVERSI1Y OF TORONTO PRESS 1965

Printed in Canada Reprinted in 2018 ISBN 978-1-4875-7910-4 (paper) Published and distributed in the U.S.A. by CHARLES C TIIOMAS

PUBLISHER

Bannerstone House 301-327 East Lawrence Avenue, Springfield, Illinois, U.S.A. Natchez Plantation House 735 North Atlantic Boulevard, Fort Lauderdale, Florida, U.S.A.

To the physician who first examines the baby

Preface

interns, and students have led us to believe that there is a need for a book in paediatrics that is neither a classical text nor a handbook but one that is a guide in the management of paediatric problems. The author of a classical medical textbook usually makes an anatomical guide the basis of his description since his work must cover fully each system of the body. On the other hand, the writer of a handbook must always keep the reference aspect prominent and so enumerates diagnostic tests, differential diagnoses, and dosages of drugs. This book was written with the patient always in mind and we have tried to present an orderly investigation of situations which may confront the doctor, stressing the urgency of early diagnosis for which specific treatment is available. It is the major problems which are discussed here, and although no attempt has been made to include all neonatal diseases, some of the rarer entities are described since early recognition is important for successful management. The book begins with "Care of the Full-Term Baby," and then discusses the major problems of new-borns. The chapter on "Congenital Anomalies" reviews the main disabilities that can be prevented or treated during the first month of life. Some chapters include definition of terminology; some present physiological, aetiological, and pathological considerations; but all are organized so that the

DISCUSSIONS WITH GENERAL PRACTITIONERS,

viii

PREFACE

practical aspects of management are readily apparent. There is one section dealing with procedures and another on dosages of drugs, including antibiotics. The book should serve as a guide to the physician when anticipating or meeting a problem or as a reference for management after examining the patient. We have tried to make the discussion practical and readable, and to give a chronological organization to investigative procedures. Since the availability of trained personnel varies greatly from hospital to hospital, we have not attempted to designate duties in all situations. It is assumed that the most qualified person in any particular situation is the one responsible for care. Although the management of the major problems in the new-born period has been outlined in some detail, a consultation with a paediatrician or an experienced colleague is warranted in any serious neonatal illness. To perform and interpret many of the procedures requires a period of supervised training. Furthermore, where facilities are limited, the early transfer of the patient to a centre equipped for investigative procedures may be imperative. We hope this book may prove useful to the general practitioners who are responsible for such a large percentage of paediatric care. It should be helpful also to the medical student, who usually studies the common problems of general practice in his final-year training in medicine. Paediatricians also may find the book a readily available source of initial reference for the major neonatal problems. J. B. J. MCKENDRY J. D. BAILEY

Acknowledgments

their gratitude to the staff of the Hospital for Sick Children, Toronto, for much information gained through years of pleasant associations. Particularly as interns, we profited from the excellent teaching of many outstanding clinicians in this hospital. Our contemporaries and the Hospital residents have also provided a continuous fund of knowledge. In this endeavour the suggestions of the medical and nursing staffs from the Queensway General and Humber Memorial hospitals have been most helpful. The clerical assistance and editorial guidance have played a large part in the completion of this volume. THE AUTHORS ACKNOWLEDGE

Contents

PREFACE ACKNOWLEDGMENTS

1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17.

A General Philosophy of Paediatrics Care of the Full-Term Baby Formula Feeding Care of the Premature Infant Rashes and Skin Infections Dysphagia, Vomiting, and Diarrhoea The Ill Baby Intravenous Therapy Respiratory Distress Cyanosis Jaundice Twitching and Convulsions Medications and the Mother Congenital Defects Procedures Dosages of Drugs Antimicrobial Drugs for the Premature and Full-Term Infant INDEX

vii

ix 3

6 22 29 41 48 68 80 88 100 110 127 137 140 162 176 181 186

Paediatric Problems In General Practice THE NEW-BORN

1. A General Philosophy of Paediatrics

as a routine examination of an infant or a child. When well, a child always presents the challenge of normal variations of mental and physical growth and development in an ever-changing personality. Normality is neither a point nor a line but a broad band, the outside limits of which take a lifetime of experience, reading, and projection to discover. When ill, a child is a diagnostic challenge and the satisfaction of seeing the sick infant restored to health is a reward in itself. Ignorance of complexities of disease seldom is costly to the patient, but errors in the simpler cases resulting from failure to consider the possibility of correctible conditions has, at times, been disastrous for the patient or at best prolonged his illness unnecessarily. Errors of simplicity come from overconfidence in past clinical experience, failure to obtain a detailed history, careless physical examination, and a neglect of simple procedures. Some examples may be helpful. While nothing can supplant clinical experience in the search of the cause of illness, one must be alert continually for the unexpected variation. The new-born member of a family afflicted with nasopharyngitis may develop a fever that is considered to be the result of a throat infection. The infant may actually have meningitis. An error in diagnosis is made if the physician, too ready to accept the familial pattern of disease, allows successive examinations in the THERE IS NO SUCH EXERCISE

4

THE NEW-BORN

group setting to become increasingly more cursory. The danger lies in the fact that the clinical evaluation of the siblings suggests nasopharyngitis and the physician fails to consider any other diagnosis in the baby. Careful examination likely would disclose an infant who is more irritable or more lethargic than is usually seen with nasopharyngitis. In addition the fontanelle would have an abnormal fullness or tenseness. A probing, detailed history is most important. A child may have a localized pneumonia which never resolves completely and often recurs in a recognizable exacerbation. The physician becomes concerned that his choice of antibiotics has been wrong. A detailed history may suggest that initially the patient became ill after a choking spell. Unless the doctor considers early the possibility of a foreign body in the bronchus, irreparable damage to the lung may be done before a bronchoscopic examination is performed and the offending foreign body removed. An examination of the ear drum may be neglected because of the small calibre of the canal and obstructing cerumen. An adequate otoscopic examination may disclose an obvious otitis media, which will explain the cause of the colic and sleeplessness in a previously happy baby. Neglecting to visualize the ocular fundi, to take the blood pressure, and to perform a rectal examination when necessary are other common failures of physical examination. Neglect of simple procedures, at worst, may be lethal to the child or, at best, humiliating to the doctor. A child who is ill with abdominal pain, even localized to the right lower quadrant, may have diabetes mellitus rather than appendicitis. A urinalysis would have made the doctor substitute insulin for the scalpel. In the course of dealing with complicated diagnostic problems it is very difficult to change one's line of thinking, and the likelihood of an exact diagnosis being established

A GENERAL PHILOSOPHY OF PAEDIATRICS

S

at a time when specific treatment might do the most good is often in inverse ratio with the time interval. When a doubtful situation thus arises, a consultation is essential. This reappraisal of the illness should bring either new information to light or reassurance to the physician. The recognition that a consultation is required in such cases is the mark of a wise physician. Finally, infants and children, in contrast to adults, have generally a recuperative capacity that is efficient and fastacting. It is the task of the doctor who is treating a child not to impair the natural healing processes by over-zealous investigation and excessive treatment. Nor should the physician's judgment be influenced by the anxiety of parents who wish that something be done quickly and actively. The role of the physician is to instruct as well as to prescribe.

2. Care of the Full-Term Baby

ALTHOUGH MOST NURSERIES function from day to day on established routines, the physician-in-charge and the headnurse of each should review from time to time the various techniques and procedures for the care of the new-born in the light of current knowledge. Most important in this care is the health of the nursing staff. Members of this staff with colds, diarrhoea, or skin infections should not report for duty. Periodic nasal cultures will identify chronic carriers of pyogenic staphylococci, who should be excluded from the nursery. Epidemic infections in the new-born nursery can be minimized by a meticulously clean and healthy nursing staff.

VITAMIN K

Most authorities recommend that no vitamin K be administered to the mother on admission and that all babies should be given 1 mg. of vitamin K1 oxide (Mephyton, Konakion) intramuscularly in the delivery room. Some obstetrical departments give the mother 5 mg. of vitamin K1 oxide intramuscularly on admission. If birth occurs within 3 hours or more than 10 hours after this injection, the baby is given 1 mg. of vitamin K1 oxide intramuscularly. The efficacy of this method of protecting the baby against hypoprothrombinaemia depends on the variables of time of administration and degree of placental transfer.

CARE OF THE FULL-TERM BABY

7

CARE OF THE BABY IN THE DELIVERY ROOM

After delivery, the doctor holds the baby by the ankles and in this dependent position allows fluid to drain from the baby's mouth as he strips the mucus from the throat. A light cord clamp is applied about 1 inch from the umbilicus. Spontaneous respirations should be established by 1 minute following delivery; if they are not, resuscitative measures must be taken. (See p. 162, "Resuscitation.") If blood incompatibility between infant and mother is suspected, the cord clamp should be applied 2 to 2½ inches from the umbilicus to facilitate replacement transfusion if required. Cord blood from infants of Rh negative mothers is sent to the laboratory for blood grouping and direct Coombs' testing. Two drops of 1 % silver nitrate ophthalmic solution are

TABLE I APGAR CHART

Heart

rate (

Respiratory effort (

Muscle tone (

)

)

None detectable (

)

Absent (

)

Slow and irregular (

)

Some flexion of extremities ( )

Active motion (

)

Grimace (

Cry (

)

Flaccid (

)

Response to stimulation ( )

No response (

)

Colour

Blue-pale (

)

(

)

2

1

0 Below 100 (

Over 100 )

)

)

Body pink, extremities blue (

SCORE TOTAL AGE IN MINUTES WHEN EVALUATION WAS MADE

)

(

)

Good cry (

)

Completely ) pink (

8

IBE NEW-BORN

instilled in each eye, and then the eyes are bathed with normal saline. After any necessary resuscitative procedures have been carried out, an Apgar ratingt is done (Table I). It is important to note the time when the evaluation was made. All babies with a rate of seven or below must be carefully observed by nursery staff. Finally, either a string of identification beads or a plastic name tag is attached to the baby's neck or wrist by the nurse. CARE OF THE BABY IN THE NEW-BORN NURSERY

As soon as the mother has recovered from the anaesthetic (which usually takes about 30 minutes), and when she has seen the baby, he is wrapped in a blanket and carried to the new-born nursery. Premature and ill babies are transferred immediately in an incubator. The baby is placed on his side. The nurse examines the cord stump for bleeding, notes the colour of the baby, any respiratory distress, and any gross abnormalities such as asymmetrical facial movements, limb paresis, and the type and force of the baby's cry. Respiratory distress requires specific and prompt management. (See p. 88, "Respiratory Distress.") · THE INITIAL BATH

If activity and breathing patterns are normal the nurse may bathe the baby 20 to 30 minutes after birth. Bathing should be postponed if the baby has had a difficult delivery since exposure might add to the baby's difficulty. The vemix caseosa is removed with cotton batting; mineral oil may be used, if necessary, to clean axillae, groin, and skin folds. The baby is bathed using a teaspoonful of 3 % hexachlorophene which is rubbed gently over the baby's body

CARE OF mE FULL-TERM BABY

9

with a soft face cloth. The hexachlorophene is rinsed off with sterile water, the baby is dried with a soft clean towel, and an alcohol wipe is applied to the cord stump to ensure that it is clean and dry. The skin of a few babies may become excessively dry or develop a blushing papular rash after the use of hexachlorophene. In these cases this solution is discontinued and bathing is performed with plain water or mineral oil. Nivea cream may be applied after bathing to the dry areas. BABY'S TEMPERATURE

The baby's temperature is taken on admission to the nursery; a rectal thermometer is used, the tip being inserted about 1½ inches into the rectum. Often the nurse makes a diagnosis of imperforate anus when she is unable to insert the thermometer into the anus. If the temperature is below 36° C. (96.8° F.) the baby is placed in an incubator until the temperature stabilizes between 36.5-37.5° C. (97.799.50 F.). The temperature is taken once daily unless some condition such as respiratory distress or fever develops, in which case the baby's temperature is taken every 4 hours. WEIGHING THE BABY

The nurse weighs the baby daily at bath time. If the gain or loss is excessive, the baby should be examined for signs of oedema or dehydration. The full-term baby usually loses 5 to 6 oz. within the first 3 to 4 days, although occasionally he may lose up to 10 per cent of his birth weight. After a stationary weight on the fifth day, he gains at the rate of 5 to 8 oz. a week for the first month. EYE CARE

None is given unless a silver nitrate reaction occurs. This reaction, characterized by an injection of the conjunctivae and a purulent discharge, develops 12 to 24 hours after

10

THE NEW-BORN

instillation. Treatment consists of bathing the baby's eyes with normal saline every 4 hours for 2 days. If the discharge persists beyond 2 days a culture should be made and a topical antimicrobial solution (e.g., 30% sulphacetamide) prescribed. Meanwhile, if the inflammation is severe, intramuscular aqueous penicillin should be given pending the result of the culture. (Seep. 183, "Dosage of Drugs.") ISOLATION

Babies born outside the maternity department are kept in the isolation nursery for 72 hours. If the baby and mother show no signs of infection and are afebrile, the baby may be transferred to the main nursery. Babies of mothers who are infectious are kept in the isolation nursery during the mother's entire stay in hospital. A nurse, other than the regular nursery staff, must care for the baby in the isolation unit. Babies from the nursery who develop thrush, infected umbilical stump, impetigo, diarrhoea, or a profuse discharge from eyes that is not considered to be a silver nitrate reaction, should be isolated in this unit. NASOPHARYNGEAL SUCTION

Suction apparatus should be readily available in all rooms. Routine suctioning of the baby should be avoided unless the baby is having difficulty breathing or swallowing. Unnecessary suction of the nasopharynx at times may produce a laryngeal spasm if the tip of the rubber catheter stimulates the vocal cords. Excessive mucus, requiring frequent suctioning, suggests an oesophageal atresia. EXAMINATION OF THE NEW-BORN INFANT

The doctor should examine the baby carefully and completely as soon as possible after birth. At times this may be done in the delivery room, but generally it is more con-

CARE OF THE FULL-TERM BABY

11

venient and satisfactory to perform the examination in the nursery. The baby must be completely undressed. A general appraisal is made of the baby regarding his cry, activity, colour, and character and type of breathing. A weak or high-pitched cry, listlessness, pallor, cyanosis, apnoea, or hyperpnoea require investigation. Facial asymmetry will be apparent when the baby cries. If the baby is quiet, it is opportune to determine the position of the heart and the cardiac rate. Any murmur heard over the precordium or back should be recorded and assessed as to its significance in relation to the clinical state. The absence of femoral pulsations should be investigated. If the baby has excessive mucus in the mouth or pharynx, it is advisable to pass a #5 French rubber catheter into the baby's stomach to ensure patency of the oesophagus after removing the excess mucus. Choanal atresia which causes respiratory distress may be excluded as a possibility by passing the same catheter into the nasopharynx through each nostril. The length of the baby should be recorded: normal is 48 to 5 3 centimetres ( 19-21 inches). The head circumference should be measured: normal is 33.5 to 37 centimetres (13 .2-14.5 inches). These measurements are useful later in assessing linear growth and head size. If the patency of the coronal or sagittal suture is in doubt, radiograms of the skull should be taken to exclude craniostenosis. The size and tension of the anterior and posterior fontanelles and the presence of a cephalhaematoma should be recorded. Abnormal size, structure, and location of pinnae may be associated with other congenital anomalies. The eyes should be examined for cataracts or coloboma. A large eyeball may indicate congenital glaucoma or tumour; a small eye may indicate toxoplasmosis. An ophthalmoscopic examination of the retina should be done in cases of suspected intracranial injury or disease. The

12

THE NEW-BORN

baby should be examined for evidence of cleft palate, harelip, and mucous cyst under the tongue, the presence of teeth and any cardinal signs of mongolism. The size, character, and location of birth-marks should be recorded for future reference since change in the lesion may influence definitive treatment at a later date. Abnormal sounds in lung fields or enlargement of liver, spleen, or kidneys require investigation. The genitalia should be examined carefully to ascertain that the correct sex has been allotted to the baby. If any doubt exists after careful examination, an expert opinion should be sought and the necessary investigation be done before a sex is assigned to the baby. (See p. 148, "Congenital Defects.") Comparative size and movement ( active and passive) of all limbs should be noted. Bilateral dislocation of the hips should be suspected if instability of hip joint is demonstrable or if abduction of both flexed thighs is limited. Unilateral dislocation of the hip may be suspected by the difference in abduction of the two thighs. The manipulation, however, should be gentle. Radiographs of hips should be taken in all clinically apparent or suspected cases. However, not all cases of congenital dislocation of hips are evident at birth; some infants require repeated examinations until 6 months of age. Both feet should be examined for intrinsic forefoot adduction. Unilateral forefoot adduction will be noted on a comparative basis, but bilateral forefoot adduction should not escape notice. If the posterior half of the foot is held stable between the thumb and index finger of one hand, the other hand may be used to assess the degree of flexibility of the midtarsal joint by abducting the forefoot. Gross clubbing of the feet ( talipes equinovarus) is readily apparent, but minor degrees sometimes escape notice. The foot should be forcibly dorsiflexed in such a manner that the fifth digit approximates the fibula. If this manoeuvre

CARE OF THE FULL-TERM BABY

13

is accomplished readily any degree of inversion, adduction, and pes cavus of the foot can be excluded. Talipes valgus (abduction) deformities are more difficult to assess since the feet of many babies tend to be "floppy" for a few weeks. The Moro reflext is useful in assessing damage to the central nervous system, Erb's palsyt and upper limb injury. In an injury of limb or Erb's palsy there is a unilateral response whereas in central nervous system injury there is a minimal or total lack of response. Gross defects of muscle such as diastasis recti of the anterior abdominal wall should be noted. Some of the entities which may be discovered during this examination are as follows: 1. Skull

suture)

(a) Cranio-stenosis ( fontanelle absent, prominent ( b) ( c) ( d) ( e)

Hydrocephalus Microcephaly Cephalhaematoma Crepitations suggestive of a skull fracture

2. Eyes Cataract, cloudiness of cornea, coloboma, Brushfield spots, or microphthalmia. 3. Ears Abnormal shape, size, or position which on occasion is associated with kidney disease, deafness, or chromosomal defects.

4. Mouth and Pharynx (a) Cleft Palate (b) Harelip ( c) Macroglossia

tThis dagger indicates a term, the definition of which will be given at the end of a chapter.

14

THE NEW-BORN

( d) Mucous cyst in the region of the tongue (e) Teeth (if loose should be removed)

(f) Choanal atresia ( usually associated with difficult breathing) (g) Tracheo-oesophageal fistula ( usually associated with excessive oral mucus)

5. Neck (a) Torticollis ( not usually diagnosed until about

3 weeks of age) (b) Webbing (suggestive of Turner's syndrome) ( c) Cystic hygroma (d) Goitre 6. Heart (a) Dextracardia ( b) A murmur suggestive of a valvular defect ( c) A murmur suggestive of a patent ductus

arteriosus ( d) The absence of femoral pulsation suggestive

of coarctation of the aorta or aortic atresia. 1. Chest (a) Tachypnoea, tugging of chin, and indrawing

suggestive of respiratory distress ( b) The presence of rales suggestive of a neonatal pneumonia or aspiration ( c) Presence of bowel sounds diagnostic of a diaphragmatic hernia 8. Abdomen (a) Large liver suggestive of a haemolytic incompatibility, cardiac failure, or infection ( b) Enlarged spleen suggestive of a haemolytic incompatibility or infection

CARE OF THE FULL-TERM BABY

15

( c) Umbilical hernia or diastasis recti ( d) Congenital hydronephrosis may appear at birth as a large abdominal mass 9. Anus Imperforate or displaced anus

IO. Limbs (a) Dislocation of hip ( b) Forefoot adduction ( c) Club feet I I. Genitalia

(a) Hypospadias ( b) Cryptorchidism ( c) Hypertrophy of clitoris ( d) Labial adhesions The physician delivering the baby should note if there are two (normal) or one (abnormal) umbilical arteries. A single umbilical artery may be associated with congenital abnormalities of the gastro-intestinal, cardiac, or central nervous system. FEEDING OF FULL-TERM BABIBS

Babies under 6 pounds are fed every 3 hours, babies over 6 pounds every 4 hours unless there are some extenuating circumstances. The feeding should supply about 3 oz. of fluid per pound per day and 50 calories per pound per day. BREAST FEEDING

No mother should be forced against her will to breast feed her baby, but the physician has a duty to enumerate the advantages of natural feeding. Maternal illnesses such as tuberculosis and badly inverted nipples are medical contra-indications. Economic considerations, social commit-

16

THE NEW-BORN

ments, a maternal repugnance and paternal refusal are, however, the commonest obstacles to breast feeding. The success of breast feeding depends on the desire of the mother, the encouragement of the physician, and the advice and help of a sympathetic, well-trained, experienced nurse. Babies are put to breast once when 8 hours old, then twice on the second and third days. Other feedings, which are given in the nursery, may be 5 % glucose water or a supplementary milk feeding depending on the baby's weight. On the fourth day the baby is put to breast every 4 hours, 5 feedings a day ( 5 % glucose water is offered at the 2 A.M. feeding). BREAST CARE

The mother should wash her nipples with sterile water and cotton batting prior to nursing. Boracic acid solutions should not be used. The baby may have difficulty sucking the nipple of a breast that is too full or too hard. If a little milk is removed by pumping, the baby is able to grasp the nipple better. Engorged breasts are treated by supporting them with a binder and applying ice packs. A breast pump sometimes will help a flat nipple to become everted, but if the nipples are badly inverted breast feeding is impossible. Tender nipples may be treated by an application of Masse cream and a reduction in feeding time. Cracked nipples are treated by stopping the baby nursing temporarily, and expressing the milk with a breast pump. Painting the crack with tincture benzoin compound will facilitate the healing. SUPPLEMENTARY FEEDINGS

If the baby is receiving 2 oz. or more each feeding from the mother by the end of the fourth day and the baby's weight is satisfactory, 5% glucose water may be used as a

CARE OF THE FULL-TERM BABY

17

supplement after nursing. However, if the baby is small and losing too much weight, a supplementary milk feeding should be ordered. When the mother is discharged from hospital she should be given instructions for a supplementary formula so that she may be free from the baby occasionally. This gives the baby an opportunity of adjusting to a formula which will be required if the mother is ill. FORMULA FEEDING

At 8 hours of age the baby is offered 1½ to 2 oz. of 5 % glucose water. At 12 hours of age a milk feeding may be given. The formula may be made from evaporated or powdered milk or from one of the completely prepared mixtures. A normal baby will thrive on any of these formulae. Processed milk is preferable to fresh cow's milk for the new-born baby, because it is partially digested during processing. The prepared formulae are convenient but more expensive and it is impossible to vary the sugar content of these preparations other than by dilution. With all artificial feedings the mother must be instructed in regard to proper sterilization. (See also p. 22, "Formula Feeding.") EVAPORATED MILK FORMULA

To 1 oz. of evaporated whole milk per pound is added 2 oz. of water per pound. To the total mixture is added 1 oz. of carbohydrate. The baby is given ½ to 1 oz. with gradual increases until he is taking 3 to 5 oz. every 4 hours, 6 feedings a day. The 2 A.M. feeding is discontinued in most cases when the baby is discharged from the nursery. If partly skimmed evaporated milk is used, to 10 oz. of milk is added 15 oz. of water, plus 1 oz. of carbohydrate. Many physicians prefer to use a partly skimmed evaporated milk rather than the evaporated whole milk. In some babies the reduced fat content lessens vomiting and colic.

18

THE NEW-BORN .

PREPARED FORMULA

For the convenience of the mother and because there is less likelihood of contamination during preparation, prepared formulae have a place in infant feeding. Before suggesting any prepared formula the physician should read the description of the ingredients of the mixture carefully. Some companies add vitamins and iron. The wisdom of these various additions may be questioned, but as long as the physician is cognizant of the contents and has some knowledge of general requirements in vitamins and minerals, he can ensure that excessive intake of such additives is avoided. WHOLE MILK FORMULA

Such a formula is not often used for the new-born infant. The simplest calculation is based on the baby's weight. The day's volume is calculated by adding 2 oz. of whole milk per pound to 1 oz. of water per pound, mixing and adding 1 oz. of carbohydrate to this mixture. The baby is started with ½ to 1 oz. with gradual increases until he is taking 3 to 5 oz. every 4 hours, 6 feedings a day. The 2 A.M. feeding is discontinued in most cases when the baby is discharged from the nursery. (See also p. 22, "Formula Feeding.") VITAMIN REQUIREMENTS

From the age of 2 weeks the infant should receive daily: vitamin A, 2,500 units; vitamin C, 35-50 mg.; vitamin D, 400-800 units. These are given in drop form unless they have been added to the milk formula. CIRCUMCISION

A discussion will be found under "Procedures."

CARE OF THE FULL-TERM BABY

19

BREAST ENGORGEMENT

The breast engorgement which develops within 10 days after birth in some new-born infants, either male or female, is due to a passive transfer of the lactating hormone from the mother. Occasionally a clear milky discharge appears from the nipple. No treatment is required; milking the nipple is contra-indicated since it predisposes the infant to infection and the development of a breast abcess. EPITHELIAL PEARLS

These are pin-head sized, linearly arranged, glistening, discrete, whitish, elevated lesions, usually in the region of the apex of the vault along the long axis of the hard palate. These small mucous cysts disappear spontaneously. They should not be mistaken for thrush. No treatment is required. INSTRUCTIONS FOR THE MOTHER

While in hospital the mother should receive instruction regarding feeding, dressing, bathing, and weighing the baby. The more practical the demonstration classes are, the more confidence the mother will develop in her ability to give her baby adequate care at home. DISCHARGE FROM HOSPITAL

On the day the baby is to be discharged from hospital the physician should re-examine him. Any doubts or questionable abnormal findings of the initial examination should be re-assessed. Specific observations should be made to exclude infection ( thrush, impetigo, conjunctivitis, and omphalitis). Any degree of jaundice should be noted. The physician should check the record of weight, intake of feedings, and stool. Any abnormal findings should be recorded accurately, and he should then sign the baby's chart.

20

THE NEW-BORN

SUMMARY

A clear airway, early recognition and treatment of respiratory distress, a safe nursery, and adequate feeding are the main requirements in the first week of life. TERMINOLOGY Apgar Rating Dr. Virginia Apgar devised a method of evaluating the newborn infant that reflects the function of respiratory, cardiac, and central nervous systems which may be designated arithmetically as a ratio of 10. After performing any necessary resuscitative measures, the evaluation of the Apgar rating should be recorded. All babies with a score of 7 or less should be observed carefully until stabilized. 1 Erb's Palsy Dr. Wilhelm Erb was a German physician who described the syndrome known as Erb's Palsy. Occasionally a birth injury occurs to the fifth and sixth cervical nerves on one side. The infant loses power to abduct and externally rotate the arm as well as to supinate the forearm. The affected limb is adducted and internally rotated at the shoulder with the forearm in pronation. On occasion the phrenic nerve roots may be damaged, resulting in paralysis of the diaphragm on the same side.

Moro Refiex Dr. Moro was a professor of paediatrics at the University of Heidelberg in 1918 when he described the reflex which bears his name. Absence of this reflex indicates brain damage. The reflex may be elicited by three methods: ( 1 ) slapping the table near the baby's head; (2) lifting the baby's head and letting it drop back suddenly; (3) pulling the shoulders of the baby off the table by holding the baby's hands, and then suddenly letting the baby's shoulders fall back. 1 Virginia Apgar, "A proposal of a new method of evaluation of the new born infant," Curr. Res. Anesth. 32 (1953), 260-67.

CARE OF THE FULL-TERM BABY

21

The reflex consists of a quick bilateral movement of arms upwards and laterally away from the body, with fingers extended and separated. Then there is a gradual grasping return of the arms. The reflex largely disappears at about 12 weeks of age in the full-term and a little later in the premature infant. An asymmetrical response is found in babies with Erb's Palsy or a fracture of the clavicle or arm. Babies with delayed mental development or cerebral palsy often show a persistence of this reflex beyond the 3-month period.

3. Formula Feeding

the caloric requirement of the normal full-term baby is about 110 cals./kg. (50 cals./lb.) of body weight per day, whereas a premature baby requires 130 to 150 cals. /kg. ( 60-70 cals. /lb.). Preparation of feeding of the full-term baby has been outlined in the previous chapter and feeding of the premature will be dealt with in the chapter on "Care of the Premature Infant." Fluid requirement of the normal infant is 3 oz. per lb. per day up to a maximum of 40 oz. per day. DURING THE FIRST MONTH

Caloric Value of Milk per ounce Whole evaporated (undiluted) ( Borden's, Farmer's Wife #1, Carnation) Partly skimmed evaporated (undiluted) (Farmer's Wife #2, Nutrilac, Morning Milk) Skimmed evaporated ( undiluted) (Farmer's Wife #3)

* * *

Whole milk (undiluted) Skimmed milk (undiluted)

Vitamin Content

40

32

One 15 oz. can of milk contains 600 units of vitamin D

29 20 11

* *

*

Formulae made from all these milks usually require

FORMULA FEEDING

23

carbohydrate added to each day's feeding: 1 oz. for babies under 12 lb. and 1¼ oz. for babies over 12 lb. Caloric Value of Carbohydrate per Ounce

Cane sugar Com syrup Dextri-maltose Barley flour

120 140 108 100

cals. cals. cals. cals.

(2 (2 (4 (4

tbl.) tbl.) tbl.) tbl.)

Typical Formula for a 10 lb. (4.5 kg.) Baby

Evaporated whole milk Water Com syrup

10 oz. 20 oz. 2 tbl.

400 cals. 140 cals. 540 cals.

The formula should be boiled for 3 minutes or terminally sterilized. The baby is offered 6 oz. every 4 hours for 5 feedings per day. Additional vitamins A, D, and C should be added after two weeks of age. (See p. 18, "Care of the Full-Term Baby.") PREPARED FORMULAE

These mixtures, sold in liquid or powdered form, contain the required carbohydrate and are prepared by adding boiled water. They are safe, convenient but more expensive than the whole or evaporated milk formulae. Many have a full complement of vitamins, are easy to transport and are, in general, nutritionally adequate. Most full strength feedings contain 20 cals. per oz. Many brands on a weight-volume basis simulate breast milk, i.e., fat 3.5 per cent, protein 1.5 per cent and carbohydrate 6.5 per cent. Many companies plan to add ascorbic acid to all evaporated milks in the near future.

24

THE NEW-BORN

When the prepared formula which contains adequate vitamins is discontinued, supplemental prophylactic vitamins should be given to the baby daily. A.

PREPARATIONS WHICH SIMULATE BREAST MILK

1. Enfalac. Full strength feeding is made by adding 1 measure (powder) to 2 oz. of water or 1 can (liquid) to 1 can of water. This reconstituted milk contains 1,500 units of vitamin A, 400 units of vitamin D, and 50 mg. ascorbic acid per 30 oz. in addition to nine other vitamins. 2. Enfalac with iron. This is the same as Enfalac but contains in addition 8 mg. of elemental iron per 30 oz. 3. S.M.A.-S:26. Normal dilution is made by adding 1 measure (powder) to 1 oz. of water or one 15-oz. can (liquid) to one can of water. The feeding contains 2,500 units of vitamin A, 400 units of vitamin D, 50 mg. of ascorbic acid in addition to niacinamide 5 mg., riboflavin 1 mg., and thiamine 0.67 mg. per 32 oz. 4. Similac. Normal dilution is made by adding 1 measure (powder) to 2 oz. of water or one 15-oz. can (liquid) to one can of water. Thirty ounces contain 2,500 units of vitamin A, 400 units of vitamin D and 15 mg. of ascorbic acid.

B. PREPARATIONS CONTAINING LOWER FAT AND HIGHER PROTEIN (COMPARED TO BREAST MILK)

1. Olac. One measure (powder) to 2 oz. of water

contains 2,500 units of vitamin A, and 400 units of vitamin D per 30 oz. The added carbohydrate is dextri-maltose. 2. Lactogen. One measure to 2 oz. of water is the normal dilution. The added carbohydrate is lactose; no vitamins are added. 3. Pe/argon. This is a milk powder acidified with lactic acid; 1 measure to 2 oz. of water is the normal dilution. The added carbohydrates are lactose, sucrose, dextri-

FORMULA FEEDING

25

maltose, and starch. This feeding may cause lactic acid acidosis in the premature infant. C.

EVAPORATED MILK WITH

7 PER CENT SUCROSE ADDED Farmer's Wife Prepared is in two forms: (i) evaporated whole milk; (ii) evaporated 2 per cent milk. Normal dilution is 1 can (liquid) to 1 can of water. This feeding contains 600 units of Vitamin D and 7 5 mg. ascorbic acid per 30 oz. These prepared formulae, in normal dilution, contain about 20 calories per oz.; no carbohydrate should be added since all contain about 6.5 per cent sugar. D.

POWDERED MILK

1. Dryco. This is a powdered partially skimmed milk, with no added carbohydrate. Normal dilution of 1 tbl. of powder to 2 oz. of water contains 15 cals. per oz. Thirty oz. contain 3,000 units of vitamin A and 480 units of vitamin D. Carbohydrate is usually added. 2. Klim. This is a powdered whole milk, with no added carbohydrate. Normal dilution of 1 tbl. of powder to 2 oz. of water contains 20 cals. per oz. and 480 units of vitamin D per 30 oz. Carbohydrate is usually added. 3. Starlac. This is a powdered skimmed milk. Normal dilution is 1 tbl. (powder) to 2 oz. of water. This contains 11 cals. per oz. There are no added vitamins. Carbohydrate is usually added. E. HYPOALLERGIC FEEDINGS

These feedings are used for infants who are allergic to milk, or babies who, following a gastro-enteritis, have a temporary intolerance to cow's milk, and in the treatment of galactosaemia. None of these "milk substitute formulae" require added carbohydrate since all contain, in the normal dilution, about 7 to 8 per cent, weight by volume, of carbohydrate.

26

THE NEW-BORN

1. Mull-Soy. When normally diluted, 1 can (liquid) to

1 can of water contains 1,200 units of vitamin A, 280 units of vitamin D, 18 mg. of ascorbic acid plus five other vitamins. Mull-Soy powder ( 1 measure to 2 oz. of water) does not contain added vitamins. The added carbohydrates in both forms are dextrose, sucrose and dextrins-maltosedextrose. 2. Sobee. It is in powder or liquid form. The normal dilution is 1 tbl. (powder) to 2 oz. of water; 1 can (liquid) to 1 can of water. The added carbohydrates are dextrimaltose and sucrose. Thirty oz. contain 2,500 units of vitamin A and 400 units of vitamin D. Both Mull-Soy and Sobee are derived from soy bean and contain 20 cals. per oz. in normal dilution. 3. Nutramigen. This is a protein hydrolysate of casein, to which has been added dextri-maltose, arrowroot, starch and com oil. Normal dilution is 1 measure (powder) to 2 oz. of water (20 cals. per oz.). Thirty oz. of normal dilution contain 2,500 units of vitamin A and 400 units of vitamin D. Nutramigen as well as being a hypoallergic feeding is also useful for infants with cystic fibrosis of pancreas who are not thriving on the usual milk feedings and supplementary pancreatic enzymes. SPECIAL FORMULAE

A. POWDERED PROTEIN MILK (Borden's) To whole milk is added a culture of streptococcus lacticus bacillus. After inbubation, part of the whey is drained from the curd. The remaining whey and curd are mixed and then made into a powder by evaporating the water. Removing part of the whey lowers the carbohydrate value and thereby increases the protein-carbohydrate ratio. This milk is most useful in the treatment of the non-specific diarrhoeas of infancy. Full strength feeding of 15 tbl. to 30 oz. of boiled cooled

FORMULA FEEDING

27

water contains 12 cals. per oz. No carbohydrate is added usually, but occasionally a 1½ strength mixture is used to increase caloric intake ( 1½ strength: e.g., 22 tbl. to 30 oz. of water). This high solute concentration should not be used for the premature or dehydrated infant. B. LACTIC Acm MILK (Borden's) Lactic acid milk powder is made from 2 per cent milk cultured with streptococcus lacticus as is powdered protein milk except that no whey is drained from the incubated mixture. (Liquid lactic acid milk is available also in many areas.) This milk is useful for the baby who may require a partially digested formula during a period of convalescence from a diarrhoea or in whom the weight gain has been less than satisfactory. Normal dilution of 1 tbl. to 2 oz. of water contains 15 cals. per oz. without addition of carbohydrate. One oz. of carbohydrate is added usually to the day's feeding. Neither powdered protein milk nor lactic acid milk can be boiled or terminally sterilized since these processes cause coagulation. These formulae are prepared by mixing the powder into boiled cooled water. The milk may be heated prior to feeding by placing the "bottled" feeding in warm water. C. L0FENALAC This balanced formula which is low in phenylalanine is a specially processed casein hydrolysate with vegetable fat, dextri-maltose, minerals, and vitamins added. Sufficient supplementary vitamins are included in the normal dilution except for vitamin C. Normal dilution is 1 measure to 2 oz. of water, and supplies 20 cals. per oz. This milk is essential in treating phenylketonuria where there is a congenital inability to metabolize adequately the essential aminoacid phenylalanine found in all natural

28

THE NEW-BORN

protein foods. Early diagnosis and the proper treatment can prevent or arrest continuing mental deficiency. This milk should be used only in cases of phenylketonuria. D. Lo-So This milk is prepared from skimmed milk from which approximately 95 per cent of the sodium has been removed. Normal dilution of 1 tbl. of powder to 2 oz. of water contains 11 cals. per oz. This milk is a useful adjunct in the treatment of congestive heart failure in infancy where, in addition to digitalis and diuretics, a restricted sodium intake is necessary.

E.

LoNALAC

It is powdered whole milk low in sodium. Normal dilution of 1 tbl. to 2 oz. of water contains 20 cals. per oz. SUMMARY

There are about a hundred different types of milk available in Canada and the United States that have some application in infant feeding. The physician must know the fat, protein and carbohydrate content of the milk he is prescribing. If adequate vitamins have been added there is no need for further supplementation. In general, it is best to use a few preparations extensively and develop a good clinical appraisal of their merits. Keeping abreast of current knowledge of infant feeding and switching from brand to brand as new products appear are not synonymous.

4. Care of the Premature Infant

PREMATURE BIRTH AND ITS ASSOCIATED COMPLICATIONS

are the main causes of mortality and morbidity in the neonatal period. Idiopathic respiratory distress, intracranial haemorrhage, increased susceptibility to infection, fluid and electrolyte disturbance, a tendency to aspirate and anaemia, are the main complications in the neonatal period of the premature baby. Some survivors will show defects of sight (rentrolental fibroplasia or cataracts), loss of hearing, cerebral palsy, or mental retardation. Since the gestational age and prognosis, immediate and ultimate, are directly related, all efforts should be made to prolong gestation to as near term as possible.

DEFINITION AND

PROGNOSIS

Babies under 2.5 kg. (5½ lb.) are usually classified as premature regardless of gestational age. Approximately 1O per cent of all babies born in Canada are premature. A few infants who have a normal or prolonged gestation but are within these weight limits, owing to placental insufficiency, present a different clinical appearance than the usual premature baby. Gestationally these babies may be premature, mature, or post-mature. It is true, also, that some babies who are 2.5 kg. or slightly less may appear to be full

30

THE NEW-BORN

term judging by their breathing pattern, sucking reflexes, skin texture, and activity. These infants often follow a familial pattern. FOETAL GROWTH

Although much information is lacking concerning average weights in relation to gestation, Gruenwald1 and Hendricks2 have made contributions in this field. TABLE II AVERAGE WEIGHTS FOR STATED PERIODS OF GESTATION

Gestational age in weeks

Weight in grams

24 26 28 30 32 34 36 38 40 42 44

665 850 1,165 1,475 1,875 2,350 2,725 3,000 3,250 3,400 3,500

Weights markedly above the expected for gestation commonly occur in infants of diabetic mothers. Infants below expected weight have intrauterine growth retardation due to placental dysfunction or other causes. MORTALITY

The figures below show the mortality rate of prematures in the Hospital for Sick Children, Toronto, 1959-1963. Infants weighing less than 1000 gm. had a mortality rate of 9 5 per cent 1001-1500 gm. 50 per cent 1 P. Gruenwald, "Chronic distress and placental insufficiency," Biol. Neonate. 5 (1963), 215-65. 2C. H. Hendricks, "Patterns of foetal and placental growth: The second half of normal pregnancy," Obst. and Gyn. 24:3 (1964), 357-65.

CARE OF THE PREMATURE INFANT

31

1501-2000 gm. 17 per cent 2001-2500 gm. 5.5 per cent Since these babies usually were admitted with some complication as well as prematurity, the mortality rates are higher than those of most new-born nurseries. MORBIDITY

In a study by Knobloch et al. the over-all incidence of serious neurological abnormalities in a large group of prematures was 8.2 per cent compared with 1.6 per cent (control). If the birth weight was less than 1501 gm. the incidence was 50.9 per cent. 3

CARE FOLLOWING DELIVERY PREPARATION

When a premature birth is expected, the delivery room staff should notify the nursery and an incubator is heated to a temperature of 33° C. (91.4° F.) and taken to the case room. The chamber is flooded with oxygen. After resuscitation the baby is placed in the incubator and brought to the nursery. If the baby appears to be in good condition, he is weighed by means of an overhead scale. Unless the baby has respiratory difficulty or is cyanotic no further extra oxygen is given routinely. If the baby is cyanotic, however, a concentration of oxygen sufficient to clear the cyanosis should be given, directly by funnel if necessary. In general, prolonged administration of concentrations of oxygen over 40 per cent should be avoided. When supplemental oxygen is required continuously, the concentration should be checked every three hours by means 3H. Knobloch, R. Rider, P. Harper, B. Pasamanick, "Neuropsychiatric sequelae of prematurity," J.A.M.A. 161 (1956), 581-85.

32

THE NEW-BORN

of an analyser such as the Beckman apparatus. As long as the baby is receiving supplemental oxygen the relative humidity should be maintained at 60 per cent; higher concentrations are unnecessary. VITAMIN K

The premature baby should be given 1 mg. of vitamin K1 oxide intramuscularly regardless of any administration of vitamin K to the mother prior to delivery. BATHING AND WEIGHING

When the baby's condition permits, a hexachlorophene bath is given within the incubator in the first two hours. Excessive handling of the baby should be avoided. A convenient time to weigh the baby daily is before or after bathing. The premature usually loses 6.5-8.5 per cent of his birth weight during his first 5 days. His weight may remain stationary for 2 to 4 weeks ( the smaller the premature the longer the time) . Once he begins to gain, he usually gains 7-9 oz. per week for the first month. STIMULATION OF THE PREMATURE

Many prematures have a markedly irregular breathing pattern characterized by respirations which are periodically fast ( over 60 per minute) or slow ( under 20 per minute) . At times there are prolonged apnoeic spells. Until normal breathing patterns are established a nurse should be in constant attendance to stimulate the baby to breath when apnoea occurs. Often flicking the soles of the feet or rubbing the back will suffice to initiate breathing, although occasionally more vigorous methods are required. ( See p. 162, "Resuscitation.") Even if the breathing pattern seems satisfactory, it is wise for the first 2 days to turn the baby from side to side

CARE OF THE PREMATURE INFANT

33

every 2 hours stimulating him to cry for a short period to ensure a good air exchange and a more complete expansion of lungs. ANTIBIOTICS

If the baby seems well and the amniotic fluid and the mother appear healthy, no antibiotic treatment is indicated for the baby delivered of a mother with early rupture of the membranes. However, if the fluid appears infected, if the mother is ill, or if the baby is distressed, antibiotic therapy should be given. Aqueous penicillin G 15,000 units ( 10 mg.) per kg. body weight combined with streptomycin sulphate 10 mg. per kg. body weight is administered intramuscularly every 12 hours for 6 days. Chloramphenicol may be given instead of the penicillin-streptomycin mixture if the proper dosage is used. ( See p. 181, "Dosages of Drugs.")

FEEDING The variation in premature infants of comparable weights in their toleration of feeding makes it necessary that the type, frequency, and amount of feeding be individualized as well as the method of administration. Furthermore, daily adjustments are necessary especially in the very premature infant. Most of the difficulties result from attempts to increase the volume too quickly. If such a problem develops, reversion to the schedule suggested for a less mature baby often is helpful. THE LARGE PREMATURE (1.8 kg.-4 lb. or over)

The infant is given nothing by mouth for 12 hours. If the breathing pattern is normal and the baby is sucking well, 15

34

THE NEW-BORN

ml. (½ oz.) of 10% glucose solution is started at 12 hours of age. The baby is fed by bottle every 3 hours. The feeding, if tolerated, is increased by 5 ml. every feeding for the next four feedings. At 24 hours of age, a feeding of % breast milk, ¼ protein milk, or a prepared formula may be given every 3 hours, 8 times a day starting with 30 ml. ( 1 oz.) and increasing by 5 ml. per feeding. If the baby tolerates this, the increase may be continued until he is receiving a total of 200 ml./kg. (3 oz./lb.) per day. In a 1.8 kg. (4 lb.) infant this would be 45 ml. ( 1½ oz.) every 3 hours, 8 feedings per day. When the baby weighs 2.3 kg. (5 lb.) the number of feedings may be reduced to every 3 hours, 7 feedings per day. At 2.8 kg. ( 6 lb.) feeding at 4-hour intervals is usually adequate. If the baby is not sucking well, or if the weight gain is inadequate, the feeding should be given by gavage. THE SMALL PREMATURE (1.1-1.8 kg. or 2½--4 lb.)

These babies require constant nursing care because of respiratory difficulties and regurgitation. In general, it is best to give nothing orally for 24 hours. If the breathing pattern is satisfactory, at this time, a 10% glucose solution is given, starting with 5 ml. and increasing by 2-4 ml. per feeding, until the baby is receiving 15 ml. ( ½ oz.) . At 48 hours of age a milk feeding is started, giving 15 ml. ( ½ oz.) every 3 hours, 8 feedings per day. Each feeding is increased by 5 ml. daily until the infant is receiving 200 ml./kg. (3 oz./lb.) per day. These babies do not suck well, and require feeding by gavage ( # 8 French soft rubber gavage tube) or an indwelling polyethylene catheter ( # 5 French). If the baby is fed by gavage through his mouth, the length of the tube passed is equivalent to the distance from the umbilicus to the baby's chin. If an indwelling polyethylene catheter is used, it is passed through the nose a distance equivalent

CARE OF THE PREMATURE INFANT

3S

to that from nose to ear to xiphoid. This catheter should be changed every five days. Often by the time the infant weighs 1. 7 kg. ( 3 ½ lb.) he is sucking sufficiently to warrant a trial of feeding by bottle. It is best to start with one bottle per day, and gradually, over a period of 10 days, to wean the baby to feeding by bottle completely. THE VERY SMALL PREMATURE (under 1.1 kg.-2½ lb.)

These babies frequently have respiratory difficulties and/ or intracranial damage which may make feeding very difficult. Infants with respiratory distress require intravenous fluids for maintenance of hydration and prevention of hypoglycaemia. (See p. 88, "Respiratory Distress.") Usually nothing is given orally for 24 to 36 hours, then a #3 ½ French polyethylene indwelling gastric catheter is passed through the nose into the stomach. Starting with 2-3 ml. of 10% glucose, dripped slowly into the catheter, the volume is increased by 1-2 ml. every other feeding as tolerated. The baby usually is fed every 2 hours. At 72 hours of age, a milk feeding is given starting with 10-12 ml. and increasing by 1-2 ml. per feeding per day, as tolerated, every 2 hours until the baby is receiving 200 ml./kg. (90 ml./lb.) per day. Frequently it is necessary to reduce the volume of feeding. The physician depends on the observations of an experienced nurse for the final adjustment of each feeding. When the baby weighs 1.1 kg. (2½ lb.) a 3-hour feeding schedule is adequate. VITAMINS

A concentrated multiple vitamin supplement containing approximately 2,500 I.U. vitamin A, 600 I.U. units of vitamin D, and 50 mg. of ascorbic acid should be given daily starting at 2 weeks of age.

36

THE NEW-BORN

GENERAL OBSERVATIONS

Aspiration of feeding is a constant hazard of the premature. This can be minimized by careful calculation of the volume of the feeding and by the careful technique of a nurse especially trained in this field. An interstitial infusion of % 5 % glucose ½ normal saline or Ringer's lactate solution ( 20 ml. /kg.) may be indicated if difficulty is encountered in maintaining hydration or if the weight is stationary. Occasionally the indwelling polyethylene gastric catheter is not tolerated. A gastrostomy ( under local anaesthesia) may be indicated for feeding during a critical period. THE

INCUBATOR

USE

Each premature baby should be placed in an incubator at birth. Of the numerous designs available, the newer incubators, Isolette (Air-Shields) and Armstrong, have proven satisfactory for temperature, air and humidity controls, administration of oxygen, and observation and handling of the baby. The small premature baby is kept in the incubator until he weighs 2 kg. ( 4½ lb.). The larger premature (2 kg. (4½ lb.) or over), may be moved to a bassinet after 2 days if breathing, temperature, and activity are normal. The baby usually can be discharged from hospital when he weighs 2.5 kg. (5½ lb.). TEMPERATURE AND HUMIDITY

The baby's colonic temperature should be maintained between 37-37.5° C. (98.6-99.5° F.). The small premature ( 1 kg.) usually requires an incubator temperature between 33.3-34.4° C. (92-94° F.), the larger premature (2 kg.) 31-32.2° C. (88-90° F.).

CARE OF THE PREMATURE INFANT

37

Some neonatologists suggest that if the baby's temperature is subnormal, there is an increase in caloric and oxygen

requirements. Initially the humidity may be 70 per cent for a few hours, then it is gradually reduced to a relative humidity of 60 per cent. A higher humidity may be used to reduce heat loss in a very small infant whose temperature cannot be maintained at physiological limits otherwise. Excessively high humidity for prolonged periods of time enhances bacterial and fungal growth. ROTATION AND CARE

Once a week the premature baby should be moved to a freshly cleaned incubator. The older models of incubators have more inaccessible areas than the new models and cleaning is therefore more difficult. Even the newer apparatus requires considerable time for meticulous cleaning. In the past a few epidemic infections such as pseudomonas aeruginosa have been traced to "infected" incubators. If a premature infant develops an infection, it is wise to make cultures from swabs applied to several areas within the incubator. A satisfactory procedure is to wash the incubator with hexachlorophene and warm water followed by rinsing with a mild iodine solution such as Wescodyne (West Chemical Products). This solution, diluted 3 oz. to 4 gal. of water, is non-staining, non-irritating, non-toxic, and is active against bacteria, fungi, and viruses.

ANAEMIA

COMPLICATIONS

Since the premature baby is born with an iron deficiency, haemoglobin estimations should be done every 2 weeks. If the level is 7 gm. %or less the baby should be transfused

38

THE NEW-BORN

with 22 ml./kg. (10 ml./lb.) of body weight using semipacked cells. Occasionally a premature baby, responding poorly to treatment for an infection, requires a transfusion even when his haemoglobin is at a higher level. At 6 weeks of age oral iron should be started using 0.6 ml. of Fer-In-Sol or Mol-iron drops twice a day. Utilization of exogenous iron before this time is minimal. With growth it may be necessary to increase this dosage to three times a day depending on the toleration and need of the baby. A full-term normal infant has a haemoglobin at birth of approximately 18 gm. per 100 ml. of blood which falls to about 11 gm. at three months of age and gradually increases to 12 by one year. Iron therapy should be continued in the premature until he approximates the haemoglobin concentration of his full-term counterpart. INFECTION

Premature babies are more prone to infection than fullterm babies. Careful attention to technique must be maintained in the premature nursery at all times by personnel who are "infection conscious" and "infection free." The reaction of a premature baby to infection often has few visible signs, making early diagnosis difficult. He should be suspected of having an infection if he deviates in any way from a previously satisfactory course. (See p. 68, "The Ill Baby.") THRUSH

Premature babies are prone to develop oral thrush. Prophylactic administration orally of 100,000 units of Mycostatin suspension on the first, third, and fifth days reduces the incidence markedly. If the baby develops thrush he should be given 100,000 units of Mycostatin 3 times a day by mouth for 5 days.

CARE OF THE PREMATURE INFANT

39

Occasionally oral monoliasis unresponsive to Mycostatin responds to the topical application of 1% aqueous gentian violet or acrifiavine, after removing the plaques with a 1% sodium bicarbonate solution. PLACENTAL INSUFFICIBNCY

Placental insufficiency is the term used for the malnourished new-born. This condition may be associated with premature, mature, or post-mature infants. Such infants have a birth weight which is less than expected for gestational age and are considered to have suffered intra-uterine growth retardation. In some cases the placenta is small and in others either gross or microscopic abnormalities are found, but frequently, the placenta appears normal. These babies have loose skin, and a loss of subcutaneous fat, are prone to hypoglycaemic convulsions, acidosis, and aspiration pneumonia. (Treatment of hypoglycaemia is discussed under "Twitching and Convulsions," acidosis under "Intravenous Therapy.") In addition to early feeding difficulties, a maturational lag may follow. Usher• has suggested that "foetal malnutrition" might be a better term to describe this group of babies. Whereas the premature baby of less than 33 weeks gestation has woolly hair, undescended testes, a soft pinna, and a breast nodule less than 3 mm. in diameter, the malnourished older counterpart of equal birth weight has silky hair, descended testes, a pinna with cartilaginous components and a breast nodule larger than 3 mm. Up to 36 weeks gestation the premature baby has a single transverse crease on the sole across the metatarsal arch. After 36 weeks, the dermal pattern is more complex. Examination of the sole should be done in the first 24 hours while the crease is discernible. After 20 hours the skin becomes dry, desquamates, and •Personal communication from Robert Usher.

40

THE NEW-BORN

the dermal pattern is obscure. These observations are useful in differentiating the premature from the infant with foetal malnutrition when the duration of pregnancy is uncertain. CONCLUSION

The majority of the larger premature infants do well but the immediate and long-term prognoses in some cases suggest that researchers must continue to study the prevention as well as treatment of prematurity. There are a considerable number of small premature babies who, with a very guarded prognosis at birth, have thrived without demonstrable sequelae to justify the time and effort which is required for these infants. Improvement in premature care has been assisted by laboratory investigations, but nothing can supplant the meticulous nursing care by specially trained personnel.

5. Rashes and Skin Infections

is covered with vernix caseosa which may be heavy or so thin that it is barely noticeable. Within a few hours this white, greasy covering becomes dry and "flaky"; it falls off and usually, by 6 hours, the skin becomes erythematous. This flushed appearance begins to fade at 24 hours, as the skin becomes dry and begins to desquamate, especially in the folds of the skin. After desquamation the skin is a pinkish-white and by 3 days of age a yellow tinge, due to physiological icterus, is frequently noticeable. A yellowish vernix caseosa is either caused by haemolytic disease with staining due to an elevated bilirubin or associated with anoxia with staining caused by meconium in the amniotic fluid. The skin of the post-mature baby is wrinkled, "parchment-like" and there is excessive desquamation. The nails are long, often yellow stained, and the umbilical cord has a greenish colour. The premature baby's skin is thin, and bruises easily. The baby has lanugo on the face and extremities and a prominent network of capillary venules. For the first few days the skin of the new-born may be oedematous on the dorsum of hands or feet or on the legs below the knees or in the pubic area, especially if the baby is premature. The skin of the new-born baby is less efficient in regulating body temperature, it has an increased endothelial permeAT BIRTH THE NORMAL BABY

42

THE NEW-BORN

ability predisposing to petechiae, and it is more susceptible to infection than that of the older infant. GENERAL PRINCIPLES IN THE CARE OF THE SKIN

Many rashes in the new-born infant are related to "overheating," "over-dressing," and applications of "baby oil." If the environmental temperature is above 22.2° C. (72° F.), a light cotton shirt, besides the diaper, is quite sufficient. Ideal room temperature is about 22.2° C. (72° F.). The well baby who is perspiring is over-dressed. A healthy new-born baby should be bathed with plain water with or without a super-fatted soap or hexachlorophene. Oily and greasy preparations used for cleansing or lubrication increase body heat and predispose to infection; however, plain mineral oil may be used for bathing if the skin is excessively dry. Perfumed baby oils and powders may irritate the skin and cause contact dermatitis. A plain talcum powder (hydrous magnesium silicate) is useful in keeping intertriginous areas dry. Boracic acid should never be used, since its absorption through macerated skin surfaces has been known to be fatal. HEXACHLOROPHENE REACTION

Seep. 9, "Care of the Full Term Baby." MILIA

These tiny pin-point whitish papules, which are retention cysts of pilosebaceous follicles, appear on the nose, chin, and cheeks. They are benign, require no treatment, and disappear spontaneously. ACNE NEONATORUM

This infantile type of acne which appears on the face, especially on the cheeks, possibly a result of hormonal transfer, is characterized by comedones, papules, and occa-

RASHES AND SKIN INFECTIONS

43

sionally pustules. Male infants are affected more often than female. The lesions improve when the area is cleansed with 3% hexachlorophene and water, but may continue for many weeks. Applications of oil or powder are not advisable. TOXIC ERYTHEMA

Scattered, discrete, pin-head sized, "flea-bite like," yellowish-white papules, surrounded by a zone of erythema, may appear in the diaper area, on the abdomen, thorax, and back about the second day. The lesions usually disappear spontaneously early in the second week. Toxic erythema is benign and needs no special treatment other than keeping the involved areas cool and dry. The aetiology is unknown, but it may be caused by an ingested allergen. MILIARIA (Prickly Heat)

This eruption, appearing on the cheeks, neck, trunk, and buttocks, caused by a plugging of the ducts of the sweat glands, may consist of tiny clear blisters, yellowish papules, or an erythematous papulo-vesicular rash, depending on the depth of the obstruction. Excessive heat predisposes to the condition. Controlling room temperature and avoidance of "over-dressing" prevents or aids in the treatment of this eruption. Compressing the areas with a cool solution of soda bicarbonate ( 1 tablespoon to 1 pint of water) or witchhazel hastens its disappearance. INTERTRIGO

Skin folds of groin, gluteal region, axillae, and neck frequently become irritated ( erythematous), macerated and, at times, secondarily infected. Bathing with hexachlorophene, exposure to dry warm air, the use of drying agents such as witch-hazel and separation of the folds with cotton batting usually are sufficient to bring it under control. Another useful drying agent consists of Burrow's Solution,

44

THE NEW-BORN

zinc oxide, talcum, and glycerin added to lime water. (See p. 178, "Dosages of Drugs.") If the area becomes infected, applications of neomycinbacitracin cream are required. Resistant cases may be associated with seborrhoea or monilial infection. If monilia is present, nystatin ointment usually is effective. SEBORRHOEA

This usually is manifested by thick, greasy, yellowish crusts forming over the scalp (cradle-cap), and eyebrows with an erythema and crusting of the intertriginous areas. The reaction is caused by a dysfunction of the sebaceous glands. The most severe form, exfoliativa erythroderma ( Leiner's disease), is a generalized eruption, with desquamation of greasy scales and an erythema of the underlying skin. The crusts of the scalp should be removed after softening with mineral oil followed by cleansing with hexachlorophene. The generalized erythematous eruption responds to applications of 0.5% hydrocortisone cream; 1% Vioform may be added. If a secondary infection in the intertriginous areas develops intramuscular penicillin should be given pending the result of the cultures and neomycin-bacitracin cream should be applied. Sebizon ( a cream-type sodium sulphacetamide lotion) is useful for infection of the scalp. Occasionally a severe diarrhoea develops with a lowering of the serum proteins necessitating a blood transfusion. PERIANAL DERMATITIS

An erythema of the skin about the anus with small pinpoint ulcerated areas may develop between the third and seventh day, possibly resulting from the alkalinity of the stool in susceptible babies. Breast-fed babies are affected less often. Treatment includes cleansing the area with liquid

RASHES AND SKIN INFECTIONS

4S

petrolatum and exposing the area to the air 3 times a day. A protective ointment such as zinc oxide applied to the area with each change of diaper will prevent further irritation. SUBCUTANEOUS FAT NECROSIS

Local areas of subcutaneous fat necrosis may develop on the face or neck, usually as a result of obstetrical trauma, hypoxia, or excessive cooling. The skin overlying the indurated area may have a reddened or purplish discolouration. Usually the lesions regress within 6 to 8 weeks, but often there is a residual atrophy of the subcutaneous tissue and a "dipping in" of the overlying skin. No treatment is required. PARONYCIIlA

Paronychiae may occur in the new-born baby especially in the thumb or toe. Usually these are mild and require compressing with normal saline, followed by an application of rubbing alcohol three times a day. If the infection becomes more extensive, a culture should be made from blood and the local lesion. Pending result of the culture and sensitivities, intramuscular methicillin should be given. ( See p. 184, "Dosages of Drugs.") IMPETIGO

Impetigo may be pustular or bleb type or an exfoliating form ( dermatitis exfoliativa neonatorum-Ritter's disease) . Pustular impetigo is characterized by the appearance of a few papules, often in the groin area, and resembling the rash of toxic erythema. However, especially in the hot humid weather, these lesions may become enlarged with formation of blebs containing pus. If the baby is well, and the pustules are sparsely scattered, breaking the blisters with cotton batting wipes, bathing with hexachlorophene and applying a neomycin-bacitracin cream usually suffices. However, if

46

THE NEW-BORN

the lesions are widespread a culture should be made and the baby should be given intramuscular methicillin 3 times a day for 5 days to prevent development of local or systemic extension. (See p. 184, "Dosages of Drugs.") Impetigo characterized by the development of crusting pustular blebs requires intramuscular methicillin after breaking the blisters, making cultures of the pus. Bathing with hexachlorophene and the application of the antibiotic cream is also necessary. Babies with this condition should be observed carefully for localization of infection in meninges, lungs, bone, and genito-urinary system. Dermatitis exfoliativa neonatorum is a very contagious form of impetigo with widespread bullous lesions and is accompanied often by marked constitutional disturbances. After making cultures of pus and obtaining a sample of blood for culture and for estimation of electrolytes, an intravenous glucose-saline infusion is started, to which is added penicillin G and methicillin. Modification of antibiotic therapy may be indicated by sensitivity studies of the organism. ( See p. 80, "Intravenous Therapy" and "Dosages of Drugs.") If the baby is toxic or in shock, a small blood transfusion and/or intravenous hydrocortisone should be given. The baby should be cared for in an isolated area and the nurse should wear plastic disposable gloves, especially when breaking the bullous blisters and applying the neomycinbacitracin cream. EPIDERMOLYSIS BULLOSA

This congenital defect of skin, characterized by formation of recurrent bullae, must be differentiated from bullous impetigo. The lesions usually begin over areas subject to trauma such as the heels, sacrum, knees, or wrists. Rubbing the skin causes a bulla to form (Nikolsky's sign). New bullae appear in spite of adequate systemic antibiotic treat-

RASHES AND SKIN INFECTIONS

47

ment. The fluid in the lesions is sterile unless it is secondarily infected. When the bullae break, the denuded areas can be protected against bacterial infection by applications of 1 : 1000 aqueous solution of acriflavine. CONGENITAL SYPHILIS

An erythematous or macular rash appearing on buttocks, face, extremities, often on the palms and soles of the newborn baby, especially if associated with a purulent nasal discharge and hepato-splenomegaly, requires serologic tests of the parents and baby. This disease is now rare because of routine testing of pregnant women for syphilis. CONCLUSION

Many rashes in the new-born infant can be prevented by control of temperature, proper clothing, and adequate care of skin. Since most rashes are benign and self-limited, care should be taken not to prolong or complicate the condition by improper or excessive treatment. However, if the newborn infant develops an infection of skin, treatment should be prompt, specific, and vigorous.

6. Dysphagia, Vomiting, and Diarrhoea

DIFFICULTY IN SW ALLOWING IF THE NURSE observes that the new-born infant is choking with feeding, prompt and careful attention is needed. In many cases, an alert nurse may detect a problem prior to the first feeding because of the infant's inability to swallow normal mucous secretions, which drool from the mouth and require frequent suctioning. Aspiration of these secretions often causes respiratory distress. MANAGEMENT

1. If the infant has respiratory distress, the pharynx should be suctioned immediately and the infant placed in moist oxygen with head low. Occasionally endotracheal suction is required. 2. A radiograph of the chest should be taken and, if massive aspiration or localized collapse has occurred, bronchoscopic suction may be indicated. 3. A #5 French rubber catheter should be passed into the stomach to ensure oesophageal patency. Choanal atresia may be excluded as a possibility by passing a catheter through each nostril into the nasopharynx. MECHANICAL LESIONS

Swallowing difficulties may be mechanical in such conditions as micrognathia, cleft palate, choanal atresia, large

DYSPHAGIA, VOMITING, AND DIARRHOEA

49

tongue, or oesophageal stenosis with or without fistula. Lack of co-ordination of the swallowing mechanism is encountered in children with cerebral damage, pharyngo-oesophageal dysphagia, or familial dysautonomia. For details of management see p. 88, "Respiratory Distress." 1. Cleft Palate may be associated with a harelip, micrognathia, and glossoptosis (Pierre-Robin syndrome). In most cases the infant can be fed best with an ordinary nipple with a large hole. Suction apparatus should be available in case the baby chokes on mucus or the feeding. In the Pierre-Robin syndrome gavage feeding may be necessary initially. These infants have a serious problem because of respiratory obstruction; in some cases, the tongue may have to be sutured to the lower lip to keep it forward, occasionally a tracheotomy is required. 2. The presence of choanal atresia should be suspected if the infant chokes with feeding or has indrawing when breathing quietly, but breathes freely when crying with mouth open. Diagnosis is proven by the inability to pass a small catheter into the nasopharynx or to detect a passage of air from the nose by stethoscope. Adenoid enlargement of the new-born occasionally will produce a similar problem. These infants are difficult to manage and require the assistance of a paediatric otolaryngologist. 3. A large tongue usually is an isolated benign condition. In rare cases glossomegaly may be associated with lymphangioma, cretinism, glycogen storage disease, or Hurler's syndrome. 4. Oesophageal stenosis will have been detected by inability to pass a small catheter into the stomach. NEUROMUSCULAR PROBLEMS 1. Cerebral damage or ma/development is the commonest cause of swallowing difficulties in new-born infants.

50

THE NEW-BORN

Usually these infants are lethargic and suck poorly. This diagnosis may be suspected if there is a history of complicated pregnancy, delivery, or difficult resuscitation. Cerebral oedema is often associated with transient vomiting and listlessness. Cerebral damage associated with intracranial bleeding intensifies the baby's signs and often complicates his recovery. The baby may have opisthotonos, lethargy, convulsions, a full fontanelle, or a shrill cry. Gross blood in the spinal fluid or radiographic evidence of skull fracture will help confirm this diagnosis. Kemicterus usually is associated with difficult sucking and swallowing. Management of such infants is discussed under "The Ill Baby." 2. Pharyngo-oesophageal dysphagia. Very occasionally a new-born infant is unable to swallow but has no other evidence of neurological deficits and no mechanical obstruction. Sucking is more vigorous than in the infants with cerebral damage but food and secretions pool in the pharynx and there is a constant threat of aspiration. This condition is thought to be related to delayed maturation of the swallowing reflex. These infants may be fed by a gavage tube but usually require a gastrostomy. When the condition has stabilized, the mother may be able to care for the baby at home. Suction apparatus must be available at all times and respiratory infection should be treated promptly. These infants usually recover between 1 and 2 years of age.

VOMITING Vomiting occurs so commonly in the first week of life that its significance is frequently overlooked. Approximately 7 5 per cent of normal new-boms vomit to some degree but rarely does this continue beyond the first 3 days. In addition to this benign regurgitation, almost any abnormal condition of either enteral or parenteral aetiology may have vomiting as a prominent symptom.

DYSPHAGIA, VOMITING, AND DIARRHOEA

51

PATHOPHYSIOLOGY

The act of vomiting is a complex process which involves increased salivation, changes in respiration, contractions of the respiratory muscles and diaphragm, closing of the glottis, contraction of the pyloric canal with relaxation of the stomach, and antiperistalsis. The cardiac sphincter is inhibited and the gastric contents are forced into the dilated oesophagus by contraction of the abdominal muscles at the same time as the diaphragm descends. Finally, the diaphragm relaxes and the expiratory muscles of the abdominal wall contract. Because of the closed glottis there is an increase in intrathoracic pressure which compresses and empties the oesophagus. There may be an associated contraction of the oesophagus and a wave of antiperistalsis. Vomiting is under control of the vomiting centre which is situated in the medulla near the respiratory centre. This area is stimulated by impulses coming not only from the gastrointestinal tract but from other organs such as the heart and vestibular apparatus. Certain toxins and drugs make the vomiting centre more sensitive so that the normal afferent stimuli are sufficient to produce vomiting. Increased intracranial pressure and partial asphyxia may stimulate the vomiting centre. For these reasons, it is apparent that a wide variety of conditions will produce vomiting in the new-born period. The real problem is therefore a matter of differentiating the significant from the insignificant and of identifying the various causes of the former so that appropriate treatment may be instituted. CLINICAL ASSESSMENT OF VOMITING

In assessing the symptoms of vomiting, the most valuable ally is an experienced nurse. Her observations can help the physician decide if the vomiting is important. The following points should then be considered carefully:

52

THE NEW-BORN

I. HISTORY

1.

FAMILY HISTORY

This is rarely helpful but occasionally provides an important and immediate answer. Familial conditions which may appear with vomiting in the neonatal period include milk allergy, congenital adrenal hyperplasia, cystic fibrosis (meconium ileus), phenylketonuria, and galactosaemia.

2.

PREGNANCY AND DELIVERY

Infections in the mother during pregnancy such as influenza, hepatitis, pneumonia, Coxsackie, herpes simplex, or chicken-pox may produce serious illness owing to transfer of the infection to the new-born. Toxaemia in the mother often causes a toxic, vomiting infant. The details of the delivery may indicate possible intracranial injury or asphyxia. Premature rupture of the membranes may be an aetiological factor in infection of the infant. 3.

DETAILS OF VOMITING

(a) Age of onset. Non-consequential vomiting, caused by gastric irritation, occurs early. Of the organic lesions, vomiting is earliest in oesophageal atresia with or without fistula. These infants regurgitate mucus from birth and there is choking and immediate regurgitation often associated with respiratory distress or with the first feeding. Upper gastro-intestinal obstruction, gastric perforation, and intracranial lesions ( e.g. haemorrhage) may produce vomiting in the first day of life. ( b) Characteristics of the vomitus. The vomitus usually consists of the feeding offered but there may be variable amounts of mucus. This frequently occurs in benign conditions as well as those associated with organic disease. Yellow or light green vomitus may occur in many conditions but the presence of dark green vomitus indicates

DYSPHAGIA, VOMITING, AND DIARRHOEA

53

obstruction as does the vomiting of meconium or faecal material. The presence of blood in the vomitus may indicate only that the baby has been feeding from a cracked nipple or swallowed some blood at the time of delivery. The benign vomiting of normal new-borns due to gastric irritation may be slightly blood streaked. Persistent blood in the vomitus may suggest oesophageal ulceration in association with a hiatus hernia. Gastric ulceration more frequently produces melena. Haematemesis, after the second day of life, may indicate haemorrhagic disease in the new-born. (c) The nature of vomiting. Regurgitation often is associated with overfeeding, hiatus hernia, or gastro-oesophageal incompetence (chalasia). Forceful vomiting is more suggestive of pyloric or small bowel obstruction. (d) The amount of vomitus. The nurse is helpful in deciding the amount of the feeding which is being retained. Small amounts of regurgitation are of less significance. Change in weight of the baby will also assist in deciding the amount of the feeding which is being retained. ( e) Feeding technique. Improper feeding techniques, such as use of too large or too small a nipple, "burping" the baby too much or too little, and incorrect position of the baby at breast or bottle, causing excessive swallowing of air, may produce vomiting. 4.

OTHER SIGNS

(a) In the vomiting infant it is of utmost importance to

know whether meconium has been passed. Although small amounts of meconium may be passed in the first few hours in the infant with obstruction, the complete absence or diminishing stool is abnormal. The presence of bloody mucus in the stool may indicate a strangulated bowel, intussusception, or an acute enterocolitis. ( b) It is helpful to know about the baby's ability to suck and swallow the feeding. Swallowing may be difficult if

S4

THE NEW-BORN

there are mechanical lesions affecting the oesophagus. In intracranial lesions control of these functions often is poor. ( c) The appetite should be noted. It decreases in conditions such as infection or intracranial lesions; however, it is often good in partial mechanical obstruction. ( d) Fever or hypothermia may indicate an infection; tachycardia or hyperpnoea may indicate a respiratory or cardiac problem. ( e) Twitching or convulsions frequently indicate intracranial problems; however, they may indicate a metabolic upset. (f) Irritability or listlessness may also indicate infection or intracranial problems. II. PHYSICAL EXAMINATION

Although a complete physical examination is imperative, certain findings should be stressed. 1. An estimate should be made of the baby's general status in regard to nutrition, colour, tissue turgor, and general activity. 2. The fontanelle should be palpated carefully for evidence of increased intracranial pressure. 3. Abdominal distention may be absent in the presence of upper gastro-intestinal tract obstruction. 4. The abdomen should be palpated carefully for the presence of masses or enlarged organs. Pyloric stenosis very rarely produces vomiting in the first week of life although a small nodule may be palpable in the pyloric area in some normal infants. It is important to palpate for liver enlargement since cardiac failure may often be associated with vomiting in conjunction with some respiratory distress. 5. It is essential to listen for bowel sounds. Absence of peristalsis may indicate intraperitoneal infection or a generalized illness with intestinal ileus.

DYSPHAGIA, VOMITING, AND DIARRHOEA

55

6. There should be an examination for inguinal hernia as strangulation may occur in the new-born period. 7. A rectal examination is necessary. It can be embarrassing to find on the second day that an infant has an imperforate anus and yet the temperatures have been recorded at regular intervals. It is important to be gentle when carrying out this procedure, since otherwise there may be tearing of the anal mucous membrane. 8. If the infant is having any difficulty in swallowing, or is choking, or has excess mucus, it is useful to establish oesophageal patency by passing a rubber catheter into the stomach. At the same time this is done, excess mucus if present may be aspirated. 9. Careful auscultation of the chest for bowel sounds is necessary since a diaphragmatic hernia may be present with vomiting rather than respiratory distress. III. RADIOLOGICAL STUDIES

1. Radiographs of the abdomen in three views (A.P., lateral, and upright), are the most useful procedures and should be carried out as soon as possible in all cases of suspected intestinal obstruction. The distribution of air in the gastro-intestinal tract provides much information. Complete obstruction of the upper gastro-intestinal tract can be diagnosed by the absence of air in bowel distal to the obstruction. Free air in the peritoneal cavity is an important sign. It indicates a perforation of the gastro-intestinal tract which may be caused by acute ulceration of the stomach, or an obstruction of the large bowel. Calcification in the peritoneal cavity is evidence of prenatal peritonitis. The condition of meconium ileus can be frequently diagnosed on a radiogram of the abdomen. The tenacious meconium in this condition produces small bubbles of air which give the bowel a rather foamy appearance.

56

THE NEW-BORN

2. With complete or incomplete obstruction in the region of the second part of the duodenum, a barium enema is sometimes helpful in establishing the diagnosis of partial malrotation of the colon. 3. An upper gastro-intestinal series using contrast media is seldom used in the first week. In suspected oesophageal atresia with or without tracheo-oesophageal fistula, lipiodal usually is used as contrast media and this is removed at the end of the examination. 4. If regurgitation persists suggestive of an oesophageal hiatus hernia or gastro-oesophageal incompetence an upper gastro-intestinal series using barium as the contrast medium is indicated. IV. LABORATORY TESTS

1. A urinalysis always is important in the vomiting newborn as in other age groups. Renal infection and anomalies usually are associated with vomiting. 2. A white blood count and smear may be useful. Although the white blood count is normally high in the new-born infant, the presence of toxic granulation suggests a need to search for infection. 3. A blood culture should be made if infection is suspected. 4. Sweat chloride estimation is useful in meconium ileus; however, the estimation in the new-born period is not always reliable. 5. Blood urea nitrogen and serum levels of carbon dioxide content, pH, sodium, potassium, chloride, as well as calcium and phosphorus may be useful in cases associated with dehydration or other evidence of electrolyte disturbance. 6. In the icteric infant, laboratory studies, should be carried out to differentiate the aetiological factors. (See p. 110, "Jaundice.")

DYSPHAGIA, VOMITING, AND DIARRHOEA

S1

V. MANAGEMENT OF VOMITING

1. VOMITING IN THE NEW-BORN WHO IS OTHERWISE WELL (a) The usual cause of this benign condition is con-

sidered to be gastric irritation because of swallowed amniotic fluid. There may be other factors involved, relating to the neurological system or a metabolic upset. Regardless of aetiology, the management is simple and consists of gentle gastric lavage, using 3-5 ml. of 2 % soda bicarbonate solution prior to the feeding. This procedure usually does not need to be continued over more than 1 or 2 days. ( b) The infant who is overfed frequently regurgitates a small amount at the end of the feeding. This infant thrives in spite of the symptom but some reduction of the feeding should be made. • ( c) The baby who is underfed may regurgitate also. These infants tend to swallow a great deal of air and have pylorospasm. They may have constipation or pass small, frequent, starvation-type stools. The treatment consists in increasing the feeding. ( d) If the baby continues to regurgitate some of the feeding, it is often helpful to keep him in a more upright position by use of a "hiatus hernia sling. "t Since there is frequently a degree of pylorospasm in these infants, a soluble antispasmodic tablet containing phenobarbital and atropine may be given ten minutes prior to feeding. (See p. 179, "Dosages of Drugs.") A thickened feeding made by adding 1 tbl. of barley flour to every 10 oz. of formula and cooking the mixture in a double-boiler for 30 minutes may be required. Feeding technique should be carefully discussed with the nurse in charge. The size of the nipple and the hole in the nipple should be adjusted as necessary to prevent excess air being swallowed with the feeding. ( e) Some new-born infants, and especially prematures,

58

THE NEW-BORN

tolerate fats poorly. In these infants a partly skimmed evaporated milk feeding is helpful. (/) Infants with allergy to cow's milk may develop vomiting in the new-born nursery. Frequently there is the triad of colic, vomiting, and loose stools. The stools may be streaked with blood. If there is a family history associated with these symptoms, the diagnosis is almost certain. However, in some cases the diagnosis will only be established by a trial of a non-milk feeding, such as Mull-soy or Sobee. Breast milk feeding of all infants would obviate many of these problems. 2. VOMITING IN ASSOCIATION WITH MORE SERIOUS DISEASE

If the clinical evaluation and initial investigation reveal or suggest serious illness, the baby will require either immediate treatment or further urgent investigation. In any case, where an obstructive lesion of the gastro-intestinal tract is discovered or strongly suspected, the baby should be transferred as soon as possible to a centre equipped to deal with this problem, and should be examined by a surgeon skilful in the management of such conditions. In most cases of serious vomiting the baby will require intensive nursing care as well as correction and maintenance of fluid, electrolytes, and acid-base balance by an intravenous infusion (see p. 80, "Intravenous Therapy"). (a) Oesophageal atresia. Infants with isolated oesophageal atresia or with a fistula from the upper segment of the oesophagus into the trachea should be kept in the "head low" position. The infant should not be fed by mouth; an intravenous should be started in order to supply the maintenance fluid and electrolyte requirements (see p. 80, "Intravenous Therapy"). Gentle suction should be carried out to keep the pharynx clear of mucus. In the more com-

DYSPHAGIA, VOMmNG, AND DIARRHOEA

S9

mon type with a fistula from the lower segment into the trachea, the infant should be kept in the "head elevated" position to reduce the amount of gastric juice regurgitated into the bronchial tree. ( b) Bowel obstruction. In obstructive lesions beyond the stomach, a gastric tube should be passed and the stomach contents aspirated by continuous suction. An intravenous glucose-saline infusion should be started and blood made available for transfusion (see "Intravenous Therapy") . In most cases of obstruction due to atresia, volvulus, or other malformations of the bowel, the urgency of immediate operation is obvious. The presence of free air in the abdominal cavity indicates a perforated viscus. This may occur in the stomach or through strangulated necrotic bowel. In some cases of Hirschsprung's disease, perforation occurs through the friable bowel wall. Infants who gradually develop signs of large bowel obstruction over the first 2 or 3 days of life, and who have not passed any meconium, may be suspected of having the "meconium plug syndrome." These infants have vomiting which becomes bile stained as well as abdominal distention and visible peristalsis. The obstruction is relieved by the passage of a long, firm mass of meconium which is dark green in colour except at the distal end which is pale yellow, dry, and bulbous. The plug may be passed after a rectal thermometer or examining finger has been inserted into the rectum. If this condition is suspected, the meconium plug may be passed after the careful installation of an enema of 15 to 20 cc. made from one part of glycerin to 6 parts of normal saline. Radiographic examination of the colon, using thin barium or lipiodol, will outline the narrow distal colon which must be differentiated from Hirschsprung's disease. In some cases the meconium plug will be passed with the expulsion of the contrast media.

60

THE NEW-BORN

Care must be taken to differentiate infants with this syndrome and those with meconium ileus due to cystic fibrosis. With meconium ileus, a surgeon is required to open the abdomen and remove the sticky, tenacious material from the bowel, much as toothpaste is squeezed out of a tube. Replacement pancreatic enzyme then is instituted to prevent the recurrence of the condition. These infants require continued treatment for their underlying condition of cystic fibrosis. Infants with Hirschsprung's disease often present with bowel obstruction in the new-born period. The obstruction may be relieved without surgical intervention, and in such cases it is necessary to do a careful follow-up study with a barium enema and at times a rectal biopsy to confirm or disprove this diagnosis. (c) Congenital pyloric stenosis. As noted previously, pyloric stenosis rarely causes vomiting in the new-born nursery. The vomiting usually begins at about 3 weeks of age and initially involves only 2 or 3 feedings each day. The episodes then become more frequent until every feeding is vomited in a projectile manner. The baby is usually hungry and takes another feeding immediately after vomiting. There is no bile in the vomitus but the material may be the colour of coffee grounds because of superficial gastric bleeding from severe vomiting. The baby's weight becomes stationary and then a weight loss follows. Constipation usually occurs but there may be a pseudo-diarrhoea. Jaundice occurs in rare instances. On general physical examination the baby may appear normal but with persistent vomiting he develops a pale greyish colour, loss of tissue turgor with sunken eyes and depressed fontanelle due to dehydration. The respirations become shallow and slow because of the alkalosis. It is advisable when this condition is suspected to exa-

DYSPHAGIA, VOMITING, AND DIARRHOEA

61

mine the abdomen while the infant is being fed a bottle of sugar solution. This will demonstrate the gastric peristaltic waves passing from left to right across the epigastrium, frequently followed by projectile vomiting. The olive-shaped pyloric tumour is usually palpated more easily in the right upper quadrant after the baby has vomited. An upper gastrointestinal radiograph using barium may demonstrate the characteristic "string sign" in the pyloric area. It should be noted, however, that radiographic studies may be misleading and the diagnosis should be confirmed by palpation of the pyloric tumour. Biochemical determinations of serum will show a raised pH and carbon dioxide content. The serum chloride and potassium levels are usually low, although low serum potassium may not be apparent when severe dehydration is present. The treatment of pyloric stenosis is surgical (Ramstedt pyloromyotomy) and the baby should be prepared for operation as quickly as possible. An intravenous infusion of 5% glucose in normal saline (20 ml./kg.) should be started, followed by % 5 % glucose and ½ normal saline. Fluid replacement should be adequate but over-hydration must be avoided. Potassium should be added to the intravenous as soon as the baby has voided (see p. 80, "Intravenous Therapy"). The infant will usually be ready for operation in 24 to 48 hours. Four hours postoperatively the infant is fed 15 ml. ( ½ oz.) of breast milk, Olac, or other formula. The feeding is offered every 4 hours and each feeding is increased by 5 ml. If the baby vomits, the same amount as the previous feeding is given. Increases are continued until the baby is receiving the full caloric requirement. ( d) Oesophageal hiatus hernia. In about 50 per cent of cases of hiatus hernia appearing in infancy the symptoms

62

THE NEW-BORN

begin in the first week of life. The vomiting, however, is of the regurgitation type and is seldom severe enough to suggest a serious problem at this t~e. It is frequently the development of associated signs and symptoms of failure to thrive, repeated aspiration, haematemesis, dysphagia, or anaemia which prompt the physician to carry out further investigation. The diagnosis will be confirmed in most cases by radiographic studies of the region using barium as a contrast medium. Special techniques are used to demonstrate oesophageal reflux and in some cases it is only possible to demonstrate gastro-oesophageal incompetency. Ulceration of the lower oesophagus may produce secondary shortening in untreated cases. If the diagnosis is doubtful, an oesophagoscopic examination will reveal the lesion and also outline the extent of the ulceration present. Treatment of this condition requires keeping the infant in a semi-upright position by the use of a hiatus hernia sling. The feeding is thickened by the addition of precooked cereal or preferably by the use of 1 tbl. barley flour to each 10 oz. of formula, cooked in a double-boiler for 30 minutes. A large nipple hole is required for this type of feeding. When ulceration of the oesophagus is present, antacids such as Amphojel are given between feedings, and anticholinergic drugs such as Monodral or Pro-Banthine are used to reduce the acidity of regurgitated gastric contents. (See "Dosages of Drugs.") In a few cases the infant fails to improve with medical management and surgical intervention is required. ( e) Gastroenteritis. The management of this condition is described under "Diarrhoea," in this chapter. (f) Other conditions. The treatment of the numerous serious parenteral conditions associated with vomiting depends on a prompt and accurate diagnosis. In addition to

DYSPHAGIA, VOMITING, AND DIARRHOEA

63

treating the underlying condition, parenteral fluid and electrolyte therapy will frequently be required ( see "Intravenous Therapy").

DIARRHOEA Epidemics of gastroenteritis in a new-born nursery can have a high morbidity and mortality. A diarrhoea developing in a neonate should therefore be considered as infectious until proven otherwise. BENIGN DIARRHOEA ( usually readily correctible)

The new-born may develop loose, frequent stools for a variety of reasons. It is usually possible to separate those infants with severe illness which requires intensive treatment from those with mild attacks due to physiological variations or minor feeding disturbances. An infant with a minor upset is not ill and there is no dehydration, fever or loss of weight. He continues to feed well. Mild diarrhoea has a number of explanations: 1. Transitional stools may be loose and frequent. Breastfed infants may have a loose stool with each feeding. 2. Babies who are overfed may have bulky, frequent stools. Underfed infants may have frequent small stools. 3. Some new-borns tolerate carbohydrates poorly, others have loose stools on a low fat formula. Specific carbohydrate enzymal deficiency is rare and requires special tests. 4. Allergy to cow's milk often produces diarrhoea with colic and vomiting. In some cases, the stool is streaked with blood. The infant usually does not look seriously ill in spite of the triad of signs. The feeding should be changed to a soy bean type formula. Infants on soy bean milk often have bulky loose stools.

64

THE NEW-BORN

SERIOUS DIARRHOEA

Diarrhoea of the severe type has a similar clinical picture regardless of aetiology since most of the signs are produced by loss of fluids and electrolytes with associated acidosis. The young infant is not able to compensate for large losses of fluid caused by diarrhoea and vomiting. Loss of fluid into the bowel may be sufficient to produce shock before a diarrhoeal stool has been passed. Usually the stools become loose and a bright yellow, then become green, watery and may be streaked with blood. The baby develops a grayish cyanosis with sunken eyes and fontanelle. Tissue turgor is poor and the mucous membranes become dry. The infant is listless and has a weak cry. The temperature may be raised, normal, or subnormal. The abdomen may be distended. In hypertonic dehydration the baby is often irritable and the skin is doughy. GASTROENTERITIS

Gastroenteritis can develop in one baby in the new-born nursery but more frequently occurs as an epidemic. Causative organisms in such epidemics often are not isolated but are considered viral in origin. Infection may be caused by pathogenic strains of E. coli, salmonella, or shigella organisms. Staphylococci, pseudomonas aeruginosa, aerobactor aerogenes, and monilia may be cultured from the stool following antibiotic therapy and are usually considered opportunists. In a debilitated infant these organisms can cause a gastroenteritis. Other causes of diarrhoea and vomiting may be confused with gastroenteritis: 1. Milk allergy, if severe. 2. Congenital adrenal hyperplasia with salt-wasting. ( See "Congenital Defects").

DYSPHAGIA, VOMITING, AND DIARRHOEA

6S

3. Galactosaemia. 4. Hirschsprung's disease ( congenital megacolon). The diarrhoea is spurious. The abdomen is distended, stools are small and may contain mucus and blood. A barium enema usually reveals the aganglionic segment. A surgical consultation for biopsy and definite treatment is indicated. MANAGEMENT

1. The infant affected should be isolated and, if more than one case occurs, the nursery should be closed to further admissions. A source of infection (carrier, feeding etc.) should be sought. 2. A stool culture should be made. 3. In mild cases where there is no dehydration, 10% sugar solution in small quantities should be given orally frequently. If an adequate quantity is retained and diarrhoea improves, intravenous fluids will not likely be required. Occasionally an interstitial infusion of % 5 % glucose, ½ normal saline (5 ml./kg.) is useful. 4. If there are signs of dehydration, blood should be taken for pH, carbon dioxide content, sodium, chloride, and potassium estimations. A glucose-saline intravenous infusion should be started immediately. Moreover in cases where the infant continues to vomit clear fluids, even though no gross signs of dehydration are present, intravenous fluids are indicated to prevent the complications of a developing dehydration. The earliest sign of dehydration is a loss of weight. If facilities for intravenous therapy are unavailable, the infant should be transferred before he becomes grossly dehydrated. Intravenous fluids are required initially to restore the volume of circulating fluid necessary for renal function. Usually 20 to 30 ml./kg. body weight of% to 5% glucose, ½ normal saline given over 2 hours, is satisfactory. If the

66

THE NEW-BORN

infant shows continuing signs of shock, plasma or whole blood (5-10 ml./kg. body weight) should be given. Restoration of the fluid deficit should be accomplished over the following 24 to 48 hours. The usual volume given is about 150 ml./kg. body weight/day. However, continuing losses and the general clinical response of the baby, which must be continually under surveillance, can influence the rate. The baby should be weighed daily and intake and output of fluids charted as accurately as possible. (See p. 80, "Intravenous Therapy.") 5. Acidosis may require intravenous bicarbonate. If a hypertonic dehydration (sodium over 155 meq/1.) is observed, the intravenous infusion of % to 5 % glucose, ½ normal saline should be given slowly. Potassium often is required but should not be given until renal function is established. ( See pp. 80, 181, "Intravenous Therapy" and "Dosages of Drugs.") 6. If the baby appears acutely ill and toxic, blood for culture should be taken and intravenous chloramphenicol given. 7. Convulsions caused by tetany or water intoxication may develop during treatment. The rate of the intravenous infusion should then be slowed and blood taken for calcium and sodium estimations. Ten ml. of 10% calcium gluconate should be added to the pedatrol containing 40 ml. of % 5 % glucose ½ normal saline. A fall in serum sodium concentration may suggest water intoxication requiring treatment with 3 % sodium chloride solution ( 15 ml. /kg. body weight). 8. If the baby is oliguric, an estimation of the blood urea nitrogen should be made. Failure of urinary output after adequate hydration indicates a renal complication. The rate of the intravenous infusion should be reduced, potassium should be discontinued, and an expert opinion should be obtained.

DYSPHAGIA, VOMITING, AND DIARRHOEA

67

9. After hydration is restored, the rate of intravenous infusion is slowed gradually as fluids orally are tolerated, and then the intravenous infusion is discontinued. After 24 hours of 10% sugar solution, a half-strength milk feeding may be started. SUMMARY

Although difficulty in swallowing, vomiting or diarrhoea may at times be benign and self-limiting, the rapidity with which the clinical state of the new-born infant can deteriorate necessitates prompt investigation of the sign at its first appearance. TERMINOLOGY

Hiatus Hernia Sling A semi-upright position reduces regurgitation in some infants who "spit up" feedings, as well as in those with a demonstrable hiatus hernia. The "positioning" of the infant is accomplished by raising the head of the mattress frame to a 70° angle, and suspending the baby with a diaper folded beneath the buttocks and around the trunk. The restraint then is "pinned" to the mattress.

7. The Ill Baby

to see a baby who appears seriously ill, but, after careful examination, has no obvious systemic localization of disease. Such a baby may refuse his feeding or take it poorly, the temperature may be elevated, normal or subnormal, and there is usually a slight increase in his respiratory rate although respiratory distress may not be evident. The baby usually is listless, pale, irritable, and there is a loss of the normal lustre of the cornea. Often this baby is in shock (grey, listless, and hypotensive). The physician immediately recognizes a serious illness without being able to define it precisely. However, the aetiology is often apparent within 24 hours if the investigation is adequate. A PHYSICIAN MAY OCCASIONALLY BE CALLED

AETIOLOGY

A. The cause of this clinical picture usually is infection and often the aetiological agent is a gram negative organism or a pathogenic staphylococcus. Since such an illness frequently runs a fulminating course, an infant with these symptoms should be considered to have an infection until such a diagnosis can be disproved. There are, however, other causes of this clinical picture: B. Intracranial damage C. Blood loss D. Metabolic disturbance

THE ILL BABY

69

E. Cardiac failure F. Unsuspected poisoning G. Neonatal obstruction of bowel MANAGEMENT UNTIL THE DISEASE IS MANIFEST

1. A review of the details of the pregnancy, labour, and delivery of the mother, in addition to a complete physical examination of the baby, is essential. 2. Blood should be taken for making a culture and for estimations of pH, carbon dioxide content, sodium, chloride, potassium, calcium, sugar, and urea nitrogen. An intravenous glucose-saline infusion with chloramphenicol should be started immediately. (See pp. 80, 181, "Intravenous Therapy" and "Dosages of Drugs.") 3. The infant should receive intensive care in an isolated unit. He should be placed in an incubator with a relative humidity of 60 per cent. Supplemental oxygen should be administered if he is hyperpnoeic or cyanotic. Ten per cent glucose solution may be offered orally but if this is not tolerated, all fluid must be given intravenously. 4. If blood loss has occurred or if the baby is shocked and not in cardiac failure, a blood transfusion is indicated: 22 ml./kg. (10 ml./lb.) body weight of fresh whole compatible blood in conjunction with intravenous hydrocortisone ( 10-20 mg. every 6 hours). 5. If cardiac failure is suspected (hyperpnoea, tachycardia, and an enlarging liver), an electrocardiogram and radiographs for heart size should be taken and the baby given his initial dose of digoxin. (See p. 177, "Dosages of Drugs.") 6. When the baby has responded to treatment for shock (improvement in colour, activity, cry, pulse, and blood pressure) a lumbar puncture should be done to exclude meningitis, since meningeal signs in the new-born are minimal. An

70

THE NEW-BORN

atraumatic tap may reveal frank subarachnoid bleeding suggestive of a birth trauma. 7. A complete blood count and urinalysis should be done in addition to obtaining urine for culture, collected as aseptically as possible using a plastic bag collector. The white blood count is normally elevated in a new-born infant, but the presence of toxic granulations on a blood smear confirms suspicion of infection. An increase in nucleated red blood cells above the normal of 10 per 100 white blood cells suggests haemolysis. A deficit of haemoglobin indicates haemolysis or blood loss. Although 30 mg. % of protein often is found in the urine of a new-born infant, microscopic examination of a centrifuged specimen showing numerous pus cells suggests a urinary tract infection. 8. At this stage a re-appraisal of the history and a reexamination of the patient is essential noting especially the colour, ocular fundi, fontanelle, elasticity of skin, air exchange, heart sounds, and size of liver and spleen. 9. Radio graphs of skull, chest, and abdomen may be indicated to exclude intracranial calcification, staphylococcal pneumonia, or signs of bowel obstruction. 10. Subdural taps are indicated if the fontanelle is full or if the baby fails to respond to treatment. A.INFECTION

In the first few days a hitherto well new-born infant may become acutely ill with an infection transmitted from the mother. Coxsackie infection, herpes simplex, cytomegalic inclusion disease, toxoplasmosis, and congenital syphilis are examples. Omphalitis, impetigo, or upper respiratory tract infection may spread to the blood stream producing meningitis, urinary tract infection, staphylococcal pneumonia, osteomyelitis, or a generalized septicaemia.

THE ILL BABY

1.

71

0MPHALITIS

The cord is often swollen and moist, and has a foul odour, with redness of the surrounding skin. However these signs may be minimal. Usually the baby is lethargic, feverish, and slightly jaundiced. Chloramphenicol is continued for 10 days unless the sensitivity test of blood or umbilical cultures indicates a change. Some cases of portal hypertension, which present at a much later date, have their origin in an unrecognized umbilical infection in the new-born period. 2. IMPETIGO

See p. 41, "Rashes and Skin Infections." 3.

MENINGITIS

Neonatal meningitis is usually caused by coliform bacteria. If lumbar puncture discloses purulent cerebrospinal fluid, chloramphenicol, sulphisoxazole, and penicillin are given intravenously until the result of the culture and the sensitivities of the causative organism are known. Drugs required for specific organisms are as follows ( see also p. 181, "Dosages of Drugs"): E.coli Pseudomonas aeruginosa Staphylococcus Pneumococcus or streptococcus Meningococcus Haemophilus influenza

Chloramphenicol, sulphisoxazole, and ampicillin if sensitive Polymyxin B intramuscularly and intrathecally Methicillin intramuscularly and intrathecally Penicillin Penicillin and sulphisoxazole Chloramphenicol and/or ampicillin

72

THE NEW-BORN

Many of these babies are in shock requiring intravenous hydrocortisone. Digmdn should be given if cardiac failure develops. If the baby is anaemic or in shock and not in cardiac failure, a small transfusion 12 ml./kg. (5 ml./lb.) of fresh compatible whole blood is indicated. 4.

URINARY TRACT INFECTION

If pus cells are found in the urine of an acutely ill infant, chloramphenicol is continued unless the sensitivities of the organism indicate a change of drug. A satisfactory specimen for culturing can be obtained after washing the external genitalia with phisohex and sterile water and using the attachable plastic collector. The freshly voided specimen must be examined and cultured immediately. If a urinary tract obstruction is suspected, the baby should be catheterized, a specimen of urine obtained for culture, and a cystogram done. Cases of obstruction may be associated with an elevation of blood urea nitrogen, potassium, phosphorus, and an acidosis indicative of impairment of renal function. A urological consultation should be obtained since the obstruction must be relieved in addition to the correction of the metabolic upset by intravenous fluids. An intravenous pyelogram should always be performed to complete the investigation as soon as the general condition improves.

5.

STAPHYLOCOCCAL PNEUMONIA

If there is a history of staphylococcal infection in the mother or impetigo in the infant, associated with tachypnoea, cyanosis, scattered rales, and a distended abdomen in a toxic baby, the diagnosis is likely to be staphylococcal pneumonia. In early cases, signs referable to chest may be minimal or absent. Radiographs of chest will suggest

THE ILL BABY

73

the diagnosis in unsuspected cases. Classical radiographic changes include infiltration, atelectasis, and characteristic cystic areas. There is frequently a pleural effusion (purulent) and/or pneumothorax. Because a sudden rupture of a sub-parietal abcess causing an acute tension pneumothorax is always a possible risk, it is necessary to have a "thoracentesis tray" available at all times. In such an emergency, requiring prompt release of the air that is under tension, there may not be time to confirm the diagnosis by radiograph. Methicillin and penicillin G are given with an intravenous infusion. If an effusion or pneumothorax is present, aspiration and instillation of methicillin, with closed chest drainage using a rubber catheter, should be instituted at once. If the baby is toxic and in shock, intravenous hydrocortisone and/or a small transfusion is indicated. If cardiac failure develops the baby should be digitalized. 6.

OSTE0MYELITIS

This condition should be suspected if there is swelling, redness, and tenderness located at the end of a long bone such as the upper end of tibia or the lower end of radius. Often an arthritis, especially of the knee, manifests as an extension of the disease process. At times this diagnosis is missed because of the paucity of signs, careless examination, or failure to consider the possibility of the diagnosis. If this condition is suspected, it is essential to take blood for culture before starting antibiotic therapy. Since the causative organism may be a resistant pyogenic staphylococcus, methicillin and penicillin G are given, for a minimum of 3 weeks, and later modified if necessary when results of cultures and sensitivities are available. An orthopaedic consultation is required since aspiration of pus or traction or "splinting" may be required.

74

7.

THE NEW-BORN SEPTICAEMIA

Suspicion of a septicaemia is intensified by the appearance of jaundice, petechiae, and splenomegaly. The diagnosis is confirmed by a positive blood culture. Chloramphenicol is continued for 10 days modified by the result of the blood culture, sensitivity of the causative organism, and the response of the patient. The baby should be observed closely for localization of infection in meninges, urinary tract, lungs, or bone. B. INTRACRANIAL DAMAGE

Occasionally an ill baby has a history of a traumatic delivery. The baby usually is depressed, has a weak or high-pitched cry, the fontanelle may feel tense, the Moro reflex is absent or depressed. The cerebrospinal fluid may be grossly blood-tinged if there has been subarachnoid bleeding. The baby should be given 1 mg. of vitamin K1 oxide intramuscularly. Radiographs to exclude a skull fracture and subdural taps to exclude a subdural haematoma are necessary. Another type of intracranial damage is caused by prolonged anoxia due to a protracted difficult delivery, occasionally associated with a premature separation of placenta or interference with the circulation of the cord. These babies are depressed, inactive, and suck poorly. Prognosis should be guarded since some of these infants have residual brain damage. Expert nursing care is required for all these babies. Since they feed poorly, gavage feeding is often necessary until the sucking reflex becomes normal. Pharyngeal suction, oxygen, and intramuscular paraldehyde and sodium phenobarbital should be available at all times since these babies are prone to take convulsions. ( See p. 127, "Convulsions and Twitching.") Since hydrocephalus occasionally develops with the intra-

THE ILL BABY

75

cranial damage, the circumference of the head should be measured weekly. The hydrocephalus usually is self-limiting, but a neurosurgical consultation is required if a fracture or subdural haematoma is present or if the head circumference is increasing at an abnormal rate. The long-term prognosis of an infant who has sustained intracranial damage should be guarded. However, many of these infants who have had a stormy course in the neonatal period have survived without demonstrable sequelae. An electro-encephalogram taken after clinical improvement usually is more useful in a consideration of the eventual prognosis than as an aid in diagnosis if taken during the acute phase. C. BLOOD LOSS

The baby may lose blood prior to birth, having bled into the mother's circulation, or into a twin, or into the placenta. Traumatic bleeding into liver, spleen, or retro-peritoneal tissues may occur during delivery. Rarely does a baby sustain an adrenal haemorrhage. Post-partum bleeding may occur from gastro-intestinal tract or from a circumcision because of a clotting defect. If marked blood loss has occurred the baby requires an immediate transfusion to correct anaemia and hypovolaemia. If an adrenal haemorrhage is suspected, intravenous hydrocortisone should be given also. After taking a sample of blood for coagulation studies, fresh whole compatible blood should be given in a sufficient volume to replace the estimate blood loss. D. METABOLIC DISTURBANCE

A rare but important cause of the_ ill baby syndrome is the infant with salt-losing adrenal insufficiency. The infant loses weight, becomes dehydrated, and feverish. The diaper is always wet. Associated diarrhoea and vomiting

76

THE NEW-BORN

may mislead the physician into diagnosing a gastro-enteritis. (Seep. 149, "Congenital Defects.") Renal insufficiency associated with an elevated blood urea nitrogen requires intravenous fluids in addition to renal investigation. Renal obstruction, if present, must be relieved. A high solute feeding such as double-strength protein, skimmed milk, or lactic acid milk, made by artificial addition of lactic acid, rather than by culture, have produced metabolic disturbances in some babies, especially prematures. These are corrected by using a lower protein feeding after correcting the metabolic upset by intravenous fluids. Infants with disturbances of amino-acid metabolism may present a difficult diagnostic problem, which can be solved by amino-acid chromotography. E. CARDIAC FAILURE

These babies usually have a pallor and slightly increased respiratory rate. There may be no significant cardiac murmur and enlargement of the liver may be questionable when the baby is first examined. Differentiation between cardiac and respiratory illness may be difficult. Examination will disclose absence of femoral pulsation if aortic atresia or coarctation is present. If cardiac failure is suspected the infant should be placed in an incubator, given oxygen and an initial dose of digitalis and immediately transferred to a centre where definitive cardiological investigation can be done. (See p. 177, "Dosages of Drugs.") F. UNSUSPECTED POISONING 1. BoRACIC Acm

The danger of mistaking boracic acid solution for sugar solution should preclude its use in the new-born nursery. Inadvertent ingestion and application to open skin areas

THE ILL BABY

77

result in vomiting, diarrhoea, and dehydration. There usually is an erythematous rash, especially of the palms and soles, and the mucous membrane of the mouth becomes congested. Severe shock may cause convulsions, renal shutdown, and even death. If the ingestion has been recent, gastric lavage followed by intravenous glucose fluids is recommended. Occasionally a replacement transfusion or peritoneal dialysis is required if there is renal involvement. 2. SODIUM CHLORIDE (SALT)

The mistaking of salt for sugar in the preparation of formula has resulted in a hypematraemic and hyperchloraemic dehydration with acidosis. Death has been reported in some cases. Salt should not be stored in the "formula room" in the hospital. If this preventable accident occurs, a peritoneal dialysis should be done. 3. PHENOL

This substance should never be used. Phenol applied to the umbilical stump has resulted in local corrosion of skin, methemaglobinaemia, vomiting, and jaundice. Shock and renal failure develop and death can occur within 24 hours from respiratory failure. Prompt replacement transfusions have saved lives. 4.

ANTIBIOTIC OVERDOSAGE

Chloramphenicol is excreted after conjugation with glucuronate in the liver in the same manner as bilirubin. Because of the immaturity of the enzyme systems, especially in the premature, this drug must be given in smaller doses than in older children. Toxic accumulation results from excessive dosage and produces the "gray syndrome" with a cyanotic pallor, lethargy, abdominal distention, ileus, and death.

78

THE NEW-BORN

A toxic manifestation of novabiocin is a yellowish discolouration of skin. Tetracycline may be deposited in the bones and teeth. The toxic effects and the optimal dosage of many drugs used for the new-born infant are unknown. It is the duty of the physician to be aware of the current knowledge of the drugs he is using, especially for the neonatal period. 5. NARCOTIC WITHDRAWAL

Seep. 137, "Maternal Medications and the Infant." G. NEONATAL OBSTRUCTION OF BOWEL

Occasionally the new-born with a neonatal obstruction such as duodenal obstruction, strangulation by volvulus or perforation of stomach or bowel has the clinical appearance of the "ill baby." The abdomen may, or may not, be distended and bowel sounds may be absent. Radiographs of the abdomen in the antero-posterior, lateral, and upright positions may disclose air in the peritoneal cavity indicating a perforation; calcification suggestive of pre-natal peritonitis; or a grossly distended stomach with absence of air in small bowel diagnostic of a high bowel obstruction. A surgical consultation should be obtained immediately in order that an operation may be performed before the infant's general condition deteriorates. In many instances surgery is successful if the diagnosis is made soon after birth. CONCLUSION

The "ill baby" is an acute medical emergency requiring vigorous yet explicit treatment. The outcome often depends on the ability of the physician to make an early diagnosis

THE ILL BABY

79

for which specific treatment is available, and on the expert care of a specially trained nursing staff. The gravity of the possible illness and the uncertainty of the prognosis make it necessary for the physician to be sure the parents understand the baby's general condition and are kept informed of major changes in it.

8. Intravenous Therapy

of fluids, electrolytes, and hydrogen ion concentration ( acid base balance) when healthy and when adequate water and nutrients are ingested. Deficits may occur as a result of excessive losses through vomiting, diarrhoea, sweating, polyuria, or inadequate intake. Imbalances may occur also through use of a feeding with a relatively high load of electrolytes or by renal failure. It is not essential for a physician to know all the complexities of cellular metabolism, but he must recognize the indications for intravenous fluids and have a working knowledge of the selection of the infusion and the rate of its administration. Dehydration, a shift in the hydrogen ion concentration ( acid base balance), and gross imbalances of electrolytes are indications for specific intravenous therapy. However, before the marked deviations occur which make such indications obvious, the physician may forestall a deterioration in the baby by starting the intravenous infusion early. This is true especially with dehydration. THE BODY REGULATES NORMAL BALANCES

DEHYDRATION

The usual signs of dehydration are listlessness, dryness of the mucous membranes of the mouth, oliguria, a lustreless appearance of the cornea, fever, and a loss of elasticity of skin. As the condition progresses, the fontanelle and

INTRAVENOUS THERAPY

81

eyeballs become sunken and the baby develops the typical gray colour characteristic of hypovolaemic shock associated with isotonic or hypotonic dehydration. In cases where water loss is proportionately greater than the loss of electrolytes, especially sodium, a hypertonic (hypematraemic) dehydration develops which is characterized by hyper-irritability with a "doughy" rather than an inelastic feel to the skin. Convulsions may occur. A baby who is moderately dehydrated has lost up to 5 per cent of his weight, if severely dehydrated about 10 per cent. If the loss is less than 5 per cent usually a trial of 10% sugar solution, by mouth, offering 1-1 ½ oz. by bottle every hour is sufficient. If the loss is greater than 5 per cent, and especially if the early signs of dehydration appear, intravenous fluids are required. FLUID REQUIREMENTS

The fluid requirements in a dehydrated baby are: 1. Water deficit i.e., the estimated loss: if mild 5 per cent, if severe 10 per cent or more. 2. Replacement of continuing abnormal loss through vomiting, diarrhoea, excessive urinary output, and sweating. This often has to be estimated from the observations of the nurse. 3. Maintenance requirements. TABLE III MAINTENANCE REQUIREMENTS PER

(after 48 hours of age)

Infant Premature

24 Houas

Water

Sodium

Potassium

110 cc. per kg. (50 cc. per lb.) 130 cc. per kg. ( 60 cc. per lb.)

0.9 meq. per kg. 1.75 meq. per kg.

0.9 meq. per kg. 1.75 meq. per kg.

NOTE. For the first 2-3 days of life, the maintenance requirement of water is 60-80 ml. per kg. per 24 hours.

82

THE NEW-BORN

TYPE OF FLUID AND RATE OF ADMINISTRATION

There are many solutions suitable for intravenous administration, but exact knowledge of the uses of a few simple mixtures is more desirable than a passing acquaintance with the complex, especially if laboratory facilities are limited. Experience has shown in most cases that, if provided with an adequate hypotonic saline-glucose solution, the body will correct imbalances if renal function is normal. However, "polyelectrolyte" solutions which, in addition to glucose and salt, contain a maintenance concentration of potassium, phosphate, and magnesium, are more physiological. They are useful when prolonged intravenous therapy is necessary. A satisfactory solution in the usual case of dehydration is % 5% glucose, ½ normal saline, which provides 5 meq. of sodium and 3.3 gm. of glucose per 100 ml. If the dehydration is 5 per cent or less, the rate may be 10 ml. /kg. ( 5 ml./lb.) per hour for 2 hours, with the remainder given over the remaining 22 hours. If the deficit is 10 per cent, the rate should be increased to 20 ml. /kg. ( 10 ml. /lb.) per hour for 2 hours and the remainder given over the following 22 hours. When renal function is established, potassium should be added to the infusion as indicated. Repeated observations and assessment of the baby's hydration should be made. If facilities for accurate weighing are available this procedure should be repeated every three hours until there is clinical improvement. If signs of dehydration persist the rate should be increased; if oedema appears the rate of administration should be reduced. The use of the Pedatrolt and Micro-Dript facilitates giving intravenous fluids to the neonate, especially the premature, where physiological variation between dehydration and oedema is limited.

INTRAVENOUS THERAPY

83

Example If a baby weighs 3.5 kg. (7 lb.) and is mildly dehydrated (5 per cent loss) his requirements are: 1. Deficit: 5% of 3.5 kg.= 175 ml. 2. Maintenance: 110 X 3.5 = 385 ml.

Total for the first 24 hours is 560 ml. approximately, provided no continuing losses occur. In the first 2 hours (10 X 3.5 X 2) = 70 ml. are given at 35 ml. per hour, and the remainder (560-70) = 490 ml. are given at a rate of ( 490 /22) approximately 22 ml. per hour for the remaining 22 hours. If continuing losses occur, the rate is increased periodically based on the estimated loss. NOTE. If intravenous fluids are required in the first 48 hours in a new-born, a solution made by mixing equal parts 10% glucose in distilled water and % 5% glucose, ½ normal saline is more advantageous than the latter solution alone. The increased glucose content protects against hypoglycaemia, hyperkalaemia, and metabolic acidosis. The decreased salt concentration reduces the renal load. The special solution used in the treatment of idiopathic respiratory distress are delineated under "Respiratory Distress." ACID BASE IMBALANCE 1. METABOLIC ACIDOSIS

Acidosis is the commonest metabolic imbalance in the ill baby. The usual sign of hyperpnoea, common in the older acidotic child, is frequently absent. The baby appears ill and lethargic, with periods of apnoea. Suspicion of a metabolic acidosis should be high in all acutely ill or dehydrated babies. If laboratory facilities are available, a blood sample

84

THE NEW-BORN

should be drawn for estimation of pH, carbon dioxide content, and electrolytes ( sodium, chloride, potassium, and calcium) and then the % 5% glucose, ½ normal saline infusion should be started. If the pH is 7 .3 or higher and the carbon dioxide content is 13 mM. /litre or above, adequate intravenous % 5 % glucose, ½ normal saline will correct the imbalance if renal function is normal. However, if the pH is below 7.3 and the carbon dioxide content is below 13 mM./litre, intravenous bicarbonate should be given. It is safest to correct the bicarbonate deficit by increments of 5 mM. /litre. Since 1.16 ml. /kg. /body weight of 5 % sodium bicarbonate solution will increase the carbon dioxide content by 1 mM. /litre, to make a 5 mM. correction it is necessary in a 3.5 kg. baby to give (1.16 X 3.5 X 5) = 20 ml. of 5% sodium bicarbonate. The bicarbonate solution should be mixed with an equal volume of % 5 % glucose, ½ normal saline. If the volume of fluid used to give the calculated amount of bicarbonate is excessive in relation to the baby's fluid requirements, a more concentrated bicarbonate solution may be used ( 5 % ) . After a 5 mM. /litre correction the patient and electrolytes are re-evaluated and more bicarbonate is given as required. If no laboratory facilities are available, an intravenous infusion of % 5 % glucose, ½ normal saline should be started, and the baby transferred to a centre equipped to make these estimations. 2.

RESPIRATORY ACIDOSIS

In the normal neonate the pH is 7.35-7.45. Occasionally an infant with poor respiratory exchange develops a respiratory acidosis with a pH below 7.35, but with an increased carbon dioxide content ( above 25 mM. per

INTRAVENOUS THERAPY

85

litre) . Since the problem is inadequate ventilation, consideration should be given to the methods of assisted respiration. Sodium bicarbonate should not be given, unless there is a significant concomitant metabolic acidosis. 3.

RESPIRATORY ALKALOSIS

Encephalitis or salicylate intoxication may cause the baby to over-breathe with a pH above 7.45 and with a decreased carbon dioxide content. Treatment should be directed to correction of the underlying disease that is causing stimulation of the respiratory centre. Tetany may occur. If it does develop, intravenous calcium gluconate is required. (See p. 127, "Twitching and Convulsions.")

4.

METABOLIC ALKALOSIS

Vomiting from pyloric obstruction or excessive administration of bicarbonate may be associated with an elevated pH ( over 7.45) and an elevated carbon dioxide content (over 25 mM./litre). (Seep. 60, congenital pyloric stenosis.)

IMBALANCES OF ELECTROLYTES 1.

HYPOKALAEMIA

A baby receiving intravenous fluids over a prolonged period should have a maintenance potassium requirement added to these fluids, after the serum level is estimated. Potassium should not be given until renal function is established. If the infant is tolerating oral fluids, a minor potassium deficit may be corrected by the administration of fluids high in concentration of potassium ( apple or orange juice), or by potassium chloride solution ( 7.5 meq. of K per 5 ml.).

86

THE NEW-BORN

A gross deficit of potassium may produce abdominal distention, ileus, decreased or absent reflexes, tachycardia, muffled heart sounds, and flat T waves on the electrocardiogram. Severe gastro-enteritis, prolonged vomiting of pyloric stenosis, and continuing gastric suction often produce marked hypokalaemia requiring intravenous potassium. With a serum level below 3.5 meq./litre a maximum replacement of 3 meq./kg./24 hours in a solution of no greater concentration than 40 meq./litre at a rate no greater than 0.5 meq./kg./hour is recommended. 2. HYPONATRAEMIA

The salt-losing type of adrenogenital syndrome is the commonest cause of severe hyponatraemia in the first month of life. If untreated, the illness may progress to coma, convulsions and death. (See p. 149, "Congenital Defects.") If the serum sodium is below normal, the deficit is obtained by subtracting this value from the normal. Calculation of the required replacement consists of taking the sodium deficit, multiplying by 0.6 (the diffusion constant), and multiplying again by the weight (kg.). In normal infants the sodium level is about 140 meq./litre. In a 7 kg. infant, if the sodium value is 120 meq., the deficit is ( 140120) = 20 mg. and the salt replacement equals (20 X 0.6 X 7) = 84 meq. Three per cent saline contains 513 meq. of sodium/litre. The volume of this solution to replace the sodium deficit is (84/513 X 1000) = 164 ml. (In the case of the salt-losing adrenogenital syndrome other treatment is required as well; see p. 150, "Congenital Defects.") 3. liYPERNATRAEMIA

In this condition fluid loss is proportionately greater than the deficit of sodium. The sodium level in the serum usually is over 155 meq./litre. Hypematraemic dehydration must be corrected slowly. The hypotonic solution of% 5% glu-

INTRAVENOUS THERAPY

87

cose, ½ normal saline has proven adequate. If an associated acidosis or potassium deficit is present, this condition should be treated also. Glucose in distilled water should never be used in this situation.

4.

HYPOCALCAEMIA

A baby with tetany and a positive Trousseau's sign associated with a decreased calcium level ( normal 10 mg. % ) or without signs and calcium level below 8 mg. % should be given intravenous calcium gluconate. (See p. 127, "Twitching and Convulsions.")

CONCLUSION Of all the advances in therapy for the infant, the knowledge of fluid balance, the development of intravenous solutions, and the techniques of their administration rate high in the list of important discoveries. TERMINOLOGY

Micro-Drip This small metal dropper, incorporated in the intravenous fluid "line," delivers a very small drop (60 drops/ml.). A more accurate rate can be maintained by this apparatus when giving intravenous fluids to the new-born or premature infant than by the conventional dropper (10 drops/ml.). Pedatrol This is a plastic "sausage-shaped" bag which contains 50 ml. of fluid when filled. It is divided into five connected compartments of 10 ml. each. In intravenous therapy of infants this apparatus facilitates measuring the volume of fluid given, as well as acting as a safety mechanism against "flooding" the baby.

9. Respiratory Distress

in the new-born nursery is respiratory distress. Obstruction of the upper respiratory tract causes choking, indrawing, and excessive pharyngeal mucus (drooling); conditions affecting the lower respiratory tract usually cause tachypnoea, grunting, dyspnoea, and cyanosis. Yet while indrawing is a usual sign of obstruction of the upper respiratory tract, it is often an accompaniment of disease of the lower respiratory tract in the new-born, and especially in the premature infant because of the reduced compliance (stiffness) of the lung and the elasticity of the thoracic wall. THE COMMONEST AND MOST DIFFICULT PROBLEM

PHYSIOLOGY

The full-term normal infant takes his first breath spontaneously within a few seconds after birth, and this is usually accompanied by a short period of crying. Millar1 divides infants into three groups: 1. Infants who breathe at approximately 40 respirations per minute throughout the first 4 days after birth and show no signs of fluctuation. 2. Infants with respiratory rates which are high-60 per minute or more during the first hour-and subsequently decrease to normal within a few hours after birth. lfferbert C. Millar, Franklin C. Behrle, Fred W. Samuel, and Richards D. Bliss, "Studies of respiratory insufficiency in newborn infants," Paediatrics, August 15, 1956.

RESPIRATORY DISTRESS

89

3. Infants with a mean respiratory rate which is 40 per minute at birth but which, during the next 36 hours, increases to 60 per minute or more. Group 1 babies saturate the blood 90 per cent or more with oxygen and reach a normal acid-base balance within 1 or 2 hours after birth. Group 2 babies oxygenate their blood and reach acid base balance as quickly as those of Group 1, but have a lower tidal volume and slower expansion of lungs and thus a higher respiratory rate. Group 3 babies demand special observation since about half develop cyanosis or respiratory distress. One-quarter of these die within the first week, and in these low tidal volume persists. In general, infants with respiratory difficulties who survive beyond 48 hours and develop a normal breathing pattern do well. However, a small percentage may suddenly die after a few days of appearing well. Furthermore, a few even with terminal illness do not show an increase in respiratory rate. Apnoeic spells are a poor prognostic sign. AETIOLOGY

Respiratory distress may be caused by: A. Obstructive lesions of the upper respiratory tract B. Expanding lesions of lung or mediastinum C. Intrinsic lesions of lung D. Congenital heart disease with failure E. Intracranial damage F. Metabolic disturbances G. Generalized infection These will be dealt with individually below. MANAGEMENT

1. The airway should be cleared of mucus, the chin should be pulled forward in order to straighten the air passage. Oxygen should be given if the baby is cyanotic up to 100 per cent concentration if necessary. When supplemental

90

THE NEW-BORN

oxygen is required over several hours, periodic attempts should be made to reduce the concentration as the baby's colour improves. If continuous oxygen is required, an estimation every 3 hours of the concentration in the incubator should be made and recorded. Prolonged periods at an unnecessarily high concentration of oxygen predispose the baby to retrolental fibroplasia resulting in varying degrees of permanent visual impairment. 2. A rapid and complete examination should be made, noting especially the colour, type of breathing (indrawing, chin tugging), respiratory rate, amount of oral mucus, expansion of thorax, air exchange, adventitious pulmonary sounds, localization of heart, valvular sounds, and the size of the liver. An accurate assessment of heart size can be made only by radiograph. 3. A #5 French stiff rubber catheter should be passed through each nostril into the nasopharynx to exclude the possibility of choanal atresia, then through the mouth into the stomach to rule out the possibility of a tracheo-oesophageal fistula. Before removing the tube any mucus in the stomach may be aspirated. 4. If the baby has severe upper respiratory obstruction ( e.g., choanal atresia, abductor paralysis of vocal cords), which causes difficulty in inspiration and which is not relieved by pulling the chin forward, the infant requires immediate intubation. An otolaryngologist should be consulted immediately since a tracheotomy may be required in addition. 5. Radiographs of chest ( antero-posterior and lateral) should be taken in all cases since many causes of distress elude the most careful clinical examination. A pressure pneumothorax requires immediate thoracentesis. If the pneumothorax is persistent, closed chest drainage may be required. Diaphragmatic hernia urgently requires surgical

RESPIRATORY DISTRESS

91

repair. While awaiting operation the infant should be nursed in a semi-upright position and a gastric tube should be passed into the stomach for continuous suction. NOTE. Radiographs will establish a diagnosis of diaphragmatic hernia, pneumothorax, pneumomediastinum, expanding lesion of mediastinum or lung, atelectasis pneumonia, idiopathic respiratory distress syndrome, Wilson-Mikity syndrome, or cardiomegaly. 6. If the cause of distress is not established at this stage, skull films, subdural taps, and a lumbar puncture may be indicated. 7. It may be necessary to obtain blood for estimation of pH, carbon dioxide content, sugar, and calcium in order to exclude a metabolic acidosis, hypoglycaemia and hypocalcaemia as causes; if there is suspicion of infection, a blood culture should be made. 8. During the acute phase all babies should receive constant nursing supervision in an intensive care unit with facilities for administration of oxygen, resuscitation, and biochemical control. A. OBSTRUCTIVE LESIONS OF THE UPPER RESPIRATORY TRACT

Choanal atresia, often associated with marked distress, and tracheo-oesophageal fistula with excessive oral mucus and choking, are readily diagnosed by catheter. Congenital stridor, caused by laryngomalacia or congenital defects of the larynx, such as a haemangioma, cord paralysis, and tracheal stenosis, usually require direct laryngoscopic examination for confirmation of the diagnosis after radiograms of the chest have been obtained. A surgical consultation is important since most cases require immediate surgery. Intubation may be required while the infant is awaiting tracheotomy.

92

THE NEW-BORN

B. EXPANDING LESIONS OF LUNG OR MEDIASTINUM

A surgical consultation is indicated in any expanding lesion of thorax or any defect that may be causing tracheal compression. Conditions such as diaphragmatic hernia, large pneumothorax or pneumomediastinum, enlarging emphysematous, or cystic areas within the lung or a large mediastinal mass causing tracheal compression can be diagnosed by radiographs of the chest and require surgical correction. C. INTRINSIC LESIONS OF LUNG

1. ATELECTASIS

This can be diagnosed on radiographs by areas of collapse. If limited, the condition can usually be alleviated by incubator care with oxygen concentration up to 100 per cent if necessary to improve or clear the cyanosis and 60 per cent humidity, the baby being turned from side to side and stimulated to cry. Because repeated pharyngeal suction is often required, prophylactic intramuscular penicillin is recommended. (See p. 183, "Dosages of Drugs.") If the atelectasis is massive, bronchoscopic suction is occasionally indicated. 2.

ASPIRATION PNEUMONIA

Pneumonia due to aspiration of amniotic fluid may be confused with congenital pneumonia. Davies2 has shown by pathological studies that congenital pneumonia is very rare. Aspiration most often occurs in the mature or post-mature baby with a complicated natal history. This time of occurrence is an aid in differentiating it from the idiopathic respiratory distress syndrome, which usually is associated with prematurity. 2Pamela A. Davies and W. Ahern, "Congenital Pneumonia," Arch. Dis. Child. 37 (December, 1962), S98-602.

RESPIRATORY DISTRESS

93

While most of these infants show respiratory distress at birth about one-fifth appear well for 18-24 hours. Rales are usually present and scattered densities are seen on radiographs. Both aspiration and neonatal pneumonia require an intramuscular antibiotic such as penicillin in addition to the usual care for respiratory distress. Staphylococcal pneumonia rarely occurs during the first week of age. ( See p. 68, "Ths Ill Baby.") 3. IDIOPATHIC RESPIRATORY DISTRESS SYNDROME (Hyaline Membrane disease) Usually the baby who develops respiratory difficulty in the first 24 hours associated with prematurity, delivery by Caesarean section, or maternal diabetes, will have idiopathic respiratory distress syndrome. 3 Many theories have been postulated as to the cause of this entity. Prematurity and changes in the pulmonary vasculature are of primary importance. The absence of fibrinolysin and of the surface tension lowering substance, surfactant, evident in infants who have succumbed, suggests defective or delayed formation of these substances. Cardiac failure and electrolyte disturbances are secondary effects. Aspiration, as a cause, has been disproven. The aetiology still remains obscure. The syndrome is characterized by tachypnoea from birth, and within a few hours the development of dyspnoea, grunting, intercostal and subcostal retraction, "chin-tug," and cyanosis. Clinical examination by stethoscope suggests a reduced air exchange but pulmonary studies have shown the minute volumes are higher than normal with a greatly reduced blood-gas exchange. Rales may be heard and if present in the first 24 hours often indicate a poor prognosis. SJ. B. J. McKendry, "Hyaline membrane diseases of lung in the newborn," Quart. Rev. Paediat. 16 (October-December, 1961), 4.

94

THE NEW-BORN

Patho-physiological Changes A combination of alveolar hypoventilation, a right to left shunting of blood in lungs and heart, reduced pulmonary blood flow, and cardiac output results in a reduced bloodgas exchange which causes hypoxia. This, in tum, leads to hypoxaemia with depression of cerebral function. Because of the hypoxaemia, lactic acid, and other organic acid metabolites are produced which cause a metabolic acidosis. The inefficient gas exchange also results in retention of carbon dioxide which further reduces the blood pH and produces a respiratory acidosis. Cellular catabolism causes an increase in serum potassium, which, if severe, may interfere with cardiac function. In infants who recover, the dyspnoea may increase for two days then gradually lessen with an improving bloodgas exchange, and the baby becomes pink, active, and hungry. In the moderate cases, metabolic and respiratory acidosis with a hyperkalaemia and dehydration occur. In fatal cases the dyspnoea, retraction, and cyanosis steadily increase with a worsening of the acid base imbalance, an increasing hyperkalaemia, and cardiac failure. These babies usually die in a state of asphyxia and exhaustion between 12 and 72 hours of age. Apnoeic spells are common in these babies. Radiological Findings Radiologically there is a generalized fine granular pattern and/or a ground glass appearance of unexpanded lung which, at times, gives the picture of an airbronchogram. Rarely there may be an accompanying pneumothorax or pneumomediastinum. Treatment Treatment includes placing the baby in an incubator in an oxygen concentration sufficient to relieve cyanosis up to

RESPIRATORY DISTRESS

95

100 per cent if necessary, and with a relative humidity of 60 per cent. The infant should have special nursing care. The special nurse should turn the baby from side to side every hour and gently remove excessive pharyngeal mucus when necessary by careful aspiration. Excessive aspiration of pharyngeal mucus, which interferes markedly with the blood-gas exchange, is dangerous, especially in the premature. If the baby is only slightly distressed and his colour is fairly good, a 15 % sugar solution may be started at 12 hours as the oxygen concentration is gradually reduced to normal. In the moderately affected infant it is best to give nothing by mouth for 24 hours. After blood is taken for an estimation of pH and carbon dioxide content, an intravenous infusion of 10% glucose in distilled water should be started and run at a rate adequate for weight and state of hydration. The maintenance requirement of intravenous fluid is 80 ml./ kg./24 hours, for 48 hours, then 110-130 ml./kg./24 hours depending on the maturity of the infant. (Seep. 81, "Intravenous Therapy.") If the pH (hydrogen ion concentration) is: 7 .20-7 .25 to every 100cc. of 10% glucose in distilled water add 0.42 grams ( 5 meq.) of soda bicarbonate 7.10-7.20 add 0.83 grams (10 meq.) 7.00-7.01 add 1.25 grams (15 meq.) less than 7.00 add 2.08 grams (25 meq.) If a rapid correction is indicated ( severe acidosis) a 5 % solution of soda bicarbonate may be used. The correction of the metabolic acidosis requires repeated estimations of pH and carbon dioxide content, the number being determined by the clinical and biochemical response of the baby. Usher4 4Robert Usher, "The respiratory distress syndrome of prematurity," Paediat. Clinics N.A. 8 (2) :525.

96

THE NEW-BORN

reported a substantial decrease in mortality principally by the administration of intravenous bicarbonate solutions. If hyperkalaemia (6 meq. or more) develops, one unit of Toronto insulin is added to every three grams of glucose in the solution. To prevent secondary bacterial infection intramuscular chloramphenicol is given. ( See p. 181, "Dosages of Drugs.") If laboratory facilities are available, more extensive determinations may be done in addition to blood pH. Determinations of the carbon dioxide content, electrolytes, and blood sugar are indicated in the more severe cases, since a baby with secondary imbalances may survive if they are corrected who otherwise would not. By plotting the pH and carbon dioxide content values on established graphs, such as the Hospital for Sick Children (Toronto) adaptation of the Davenport diagram (Fig. 1), the partial pressure of carbon dioxide and the buffer base or plasma bicarbonate values may be obtained, and these values will elucidate the mixed picture of metabolic and respiratory acidosis. In the very severe form of the disease the baby often deteriorates in spite of an adequate correction of the metabolic acidosis. The use of assisted ventilation by a respirator such as the "Bird" has saved a few of these babies. Diminishing air exchange, increasing cyanosia, decreasing level of consciousness, and increasing respiratory effort are the main clinical features to be assessed in order to determine when assisted respiration is required. If blood studies show that the partial pressure of the carbon dioxide is over 7 5 mm.Hg. (venous) or if the partial pressure of oxygen is less than 40 mm.Hg. (arterial), while the baby is breathing pure oxygen, assisted ventilation is required. If intubation is needed for more than 24 hours a tracheotomy is necessary. Assisted ventilation appears to help a few severely affected infants who weigh more than 1800 gm. and who have survived at least 24 hours. To date about 5 per cent

•t~-,

40

Buller Ba■ ep m.eq./1.

➔ c

35

1m

tlffl±fill!I

'a

:. · +-~ ·-I•··"/

] 25

i

j

i:0

u

Q.

0"'

u

{+}'t:£H

il,11:j:!~'ljlftfil

{1ft

·-1_-iJl'!'lt

_;::t:

I

35

+"M

Wif:f{;HI

~

: RESPIRATORY - ACIDOSIS

:::::.

40

_i-q

;t

, 30---iH

METABOLIC ALKALOSIS

P CO2 mm. Hg.

:t:U i;;

Ii

--nij1t .

·•±;.ti:!

;:;;;_;'

~,,q~FHl-i~

fi ¼Hf:!th i1JJ, .,

=-~· ;i~

d1;1 ::iW

f-j

'f ti:

" ·· tk1-1s 1

RESPIRATORY ALKALOSIS

s 0

:E

ACIDOSIS

[H+] nano-eq. 398 pH

6-4

316 251 6·5 6·6

200 160

6'7

6·8

125

6·9

100 BO 7·0 7·1

62

7·2

so 7·3

40 7·4

31

7·5

10

s 0

-n+1

ffi METABOLIC

25

7-6

20 7·7

Fm. 1. The Hospital for Sick Children Neonatal Research Unit

16 7·8

12

7-9

.tr

ii

,..,

Q.

~i

~1.H

' +\11,/'

nit,

25 20

X\t. ,, ..b - +•--.-·_ .

~+!

10

t.

j:J:c

20 15

30

0

u

:i:

98

THE NEW-BORN

of these infants at the Hospital for Sick Children, Toronto, who by previous experience would have succumbed, have been saved by this treatment. 5 If apnoea develops, the treatment is to aspirate the airway quickly with mouth suction, put a small oxygen mask over the infant's mouth and nose (not compressed tightly) with oxygen flowing at 5 litres per minute, and flick the feet. If there is no respiratory response, the physician inserts an infant plastic airway, applies intermittent pressure, no greater than 30 millimetres of mercury. If there is still no response the doctor intubates the baby and gives mouth to mouth respiration or uses the "Bird" respirator until the baby is breathing spontaneously and regularly. 4. PULMONARY DYSMATURITY

Wilson and Mikity6 in 1960 described a type of respiratory distress occurring in premature babies usually under 1600 gm., which they called pulmonary dysmaturity. Radiographs of lungs show a characteristic "bubble-like" pattern of cystic areas surrounded by thickened septum. These babies have cyanosis from birth or develop it within 2 weeks and often are "oxygen dependent" for several months. About 50 per cent survive and improve gradually after 6 or 8 months. D. CONGENITAL HEART DISEASE

Respiratory distress, especially hyperpnoea, with tachycardia and an enlarging liver, with or without cyanosis, is indicative of cardiac failure. If the radiographs of the chest reveal a large heart, the diagnosis is more likely. 11 M. Delivoria-Papadopoulos, H. Levison, P. R. Swyer, "Metabolic and physiological effects of assisted ventilation in severe idiopathic respiratory distress syndrome,'' Amer. Soc. Paediat. Res., Abstracts, June, 1964. 6M. G. Wilson and V. G. Mikity, "New form of respiratory distress in premature infants," Amer. J. Dis. Child. 99 (1960), 484-99.

RESPIRATORY DISTRESS

99

The baby should be given supplemental oxygen and an initial dose of digoxin and transferred to a paediatric cardiac unit. (Seep. 177, "Dosages of Drugs.") Many defects which formerly were fatal to the new-born baby, now are amenable to newer techniques of cardiovascular surgery. E. INTRACRANIAL DAMAGE

Subarachnoid haemorrhage, subdural haematoma, or neonatal meningitis occasionally present as respiratory distress. Radiographs of skull, lumbar puncture, and subdural taps establish the diagnosis. (For management see p. 68, "The Ill Baby.") F. METABOLIC DISTURBANCES

Occasionally the baby with hypoglycaemia or hypocalcaemia has respiratory distress rather than convulsions. The diagnosis is established by estimating the levels of sugar or calcium in the blood. (For management seep. 127, "Twitching and Convulsions.") G. GENERALIZED INFECTION

Occasionally a baby has respiratory distress associated with a generalized infection such as a septicaemia. A lethargic or febrile infant, a history of maternal illness, the presence of an omphalitis or impetigo are sufficient to warrant taking blood for making a culture. (For management see p. 68, "The Ill Baby.") CONCLUSION

Respiratory distress in the new-born baby is a sign for immediate and definitive investigation. In many instances the prognosis depends on the early determination of the exact aetiology for which specific treatment is available.

10. Cyanosis

has a haemoglobin of 18-20 gm. % at birth, with an arterial oxygen saturation of 95-98 % . If arterial saturation falls to about 80%, cyanosis will appear. Since at least 5 gm. % of reduced haemoglobin in the skin capillaries is necessary to produce cyanosis, an anaemic baby (for example, 8 gm. % ) will require a much greater desaturation to produce this sign. Some healthy new-born infants show a peripheral cyanosis when undressed in a cool atmosphere. This clears when the baby is dressed and warm. As the baby gets older this tendency disappears. A few babies, especially prematures, show the "harlequin phenomenon," that is, the dependent half of the body becomes cyanotic for a short period. The duskiness which is a manifestation of an immaturity of the vasomotor centre clears when the baby is repositioned. THE NORMAL NEW-BORN INFANT

CLINICAL CAUSES

These are enumerated here, and will be discussed below: A. Respiratory disease B. Cardiac disease 1. Congenital heart disease (a) Cyanosis-with right to left shunting of unoxygenated blood ( b) Cardiac failure 2. Miscellaneous causes

CYANOSIS

101

C. Central nervous system dysfunction D. Shock E. Tetany and convulsions F. Methaemoglobinaemia It is difficult to differentiate, at times, between respiratory and cardiovascular disease causing cyanosis. Both may be associated with hyperpnoea, tachycardia, and cardiac enlargement. In general, if the heart is large, if there is a distinct cardiac murmur, if the lung fields are relatively clear, and if cyanosis is not relieved by a high concentration of oxygen, the cause is likely cardiovascular. Crying usually causes a deepening of cyanosis in the cyanotic baby with congenital heart disease. Similarly, if rales are present, if lung expansion is minimal, if a localized or generalized atelectasis is present and if cyanosis is relieved partially, or totally, in increasing concentrations of oxygen, the cause usually is pulmonary. Occasionally the problem is solved only with the passage of time, especially when the results of investigation are equivocal. A. RESPIRATORY DISEASE

Infants with a congenital obstruction of the upper respiratory tract such as choanal atresia, grossly enlarged adenoids, vocal cord paralysis, or tracheal stenosis usually have episodes of cyanosis with some degree of indrawing. Atelectasis, idiopathic respiratory distress, pneumothorax, diaphragmatic hernia, aspiration from a tracheo-oesophageal fistula, pneumomediastinum, lobar emphysema, neonatal pneumonia, or a paralysed diaphragm as a result of a birth injury, may cause cyanosis. In general, babies with pulmonary disease have hyperpnoea, dyspnoea, cough, or some finding on physical and/or radiological examination of the lungs. Fluoroscopy of chest will give evidence of the dynamic activity of lungs and

102

THE NEW-BORN

diaphragm. (For diagnosis and treatment see p. 88, "Respiratory Distress.") B. CARDIAC DISEASE

1.

CONGENITAL HEART DISEASE

(a) WITH CYANOSIS

Of approximately thirty-five congenital cardiac defects which cause cyanosis, four are most common: (i) transposition of the great vessels (ii) tetralogy of Fallot (iii) pulmonary valve atresia with intact ventricular septum (iv) tricuspid atresia (i) Transposition of the Great Vessels

This defect is characterized by a deep cyanosis appearing at, or soon after, birth and an enlarging egg-shaped heart with a narrow base. The heart sounds and electrocardiographic findings are variable. Cardiac enlargement is present in about 20 per cent of the cases. This is the commonest cause of cardiac cyanosis in the first week of life. (ii) Tetralogy of Fallot

Thirty per cent of the babies with this syndrome are cyanotic soon after birth. A harsh systolic murmur heard best along the left sternal border, a normal-sized "creur sabot," and a right ventricular loading pattern in the electrocardiogram suggest this condition. The combination of infundibular pulmonary stenosis and a ventricular septa! defect explain the condition. Cardiac failure never occurs. (iii) Pulmonary Valve Atresia (with intact ventricular

septum) Severe cyanosis is present at or soon after birth. Cardiac failure occurs, the heart is large and there is reduced lung

CYANOSIS

103

vascularity. A continuous ductal murmur occasionally is heard and an electrocardiogram shows left ventricular hypertrophy. (iv) Tricuspid Atresia

A cyanotic infant with a normal-sized heart, who has reduced pulmonary vascularity, a straight border of the right side of the heart on radiograph, a left axis deviation, a left ventricular and a right atrial hypertrophy on the electrocardiogram, likely has tricuspid atresia. In 50 per cent of the cases a harsh systolic murmur is heard along the left sternal border. Cardiac failure rarely occurs. (b) WITH CARDIAC FAILURE

There are certain cardiac conditions where cyanosis, if present, is overshadowed by cardiac failure. Tachypnoea, tachycardia, and an enlarging firm liver indicate failure. Although numerous cardiovascular defects may cause failure and slight cyanosis, the most common in the newborn period are: ( i) coarctation of the aorta (ii) large patent ductus arteriosus (iii) aortic atresia (iv) arterio-venous aneurysm Differentiation of these lesions from pulmonary disease, which may produce cardiac enlargement, is difficult. (i) Coarctation of the Aorta

This condition is associated with an absent or diminished femoral pulse. The coarctation usually is preductal (infantile type) and is often associated with a ventricular septal defect. The heart is large with signs of failure, the lungs are plethoric and a systolic murmur may be heard

104

THE NEW-BORN

posteriorly. The electrocardiogram shows right ventricular hypertrophy. (ii) Large Patent Ductus Arteriosus

This defect is characterized by bounding peripheral pulses due to a wide pulse pressure. There is a systolic murmur, loudest over the second and third left parasternal intercostal spaces, which is transmitted posteriorly. The heart is large with signs of failure and the pulmonary vascularity is increased. The electrocardiogram shows a left ventricular loading pattern. (iii) Aortic A tresia

This lesion is associated with very weak brachia! and femoral pulses, fulminating congestive failure, and a very large heart at birth. The murmur is variable and electrocardiogram shows marked right ventricular hypertrophy. (iv) Arterio-venous Aneurysm This condition should be suspected in the new-born if failure is present and no cardiac lesion is demonstrable. Auscultation may disclose a bruit over the skull, lungs, or liver. 2.

MISCELLANEOUS CAUSES

Viral myocarditis such as a Coxsackie-B infection or glycogen storage disease of the heart may cause failure and cyanosis within the first week of life. Endocardial fibroelastosis or an aberrant left coronary artery is a rare cause of cardiac failure within the first month. Treatment Since most cardiovascular defects are amenable to corrective or palliative surgery, all patients should be transferred to a paediatric cardiac unit once the diagnosis is

CYANOSIS

10S

suspected. Treatment, prior to and during transfer, should be initiated as follows: ( 1) Oxygen. The infant with cardiac failure should be given digitalis and kept in an oxygen concentration sufficient to improve or clear the cyanosis. Babies with a marked right to left shunting of unoxygenated blood will not clear in a 100 per cent oxygen concentration. (2) Digoxin. The position of the edge of the liver should be marked on the skin of the abdominal wall. If the heart is responding to treatment the liver edge should recede gradually. The total digitalizing dose of digoxin is 0.06 mg./kg. body weight. One half of this amount is given intramuscularly, stat, one quarter is given in 6-8 hours, and the remaining quarter is given after a further 12-hour interval. Since there is no antidote for an overdose of digitalis the exact calculation should be re-checked carefully before administration. If possible an electrocardiogram should be taken prior to the third dose to ascertain any early toxic effects of the drug. The maintenance dose is ¼ to ¼ of the total digitalization dose and is given daily in two equally divided doses. (3) Diuretics. If transfer is delayed and if the baby is not improving rapidly on digoxin, diuretics may be given, using a mercurial compound (such as Mercuhydrin) or chlorothiazide. (Seep. 179, "Dosages of Drugs.") ( 4) Electrolyte correction. Many babies with cyanosis due to a cardiac defect have a concomitant acidosis which, if corrected, may help to tide the baby over a critical period. If a blood pH and carbon dioxide content are obtained, these values when plotted on an adaptation of the Davenport Chart will delineate the extent of a metabolic and/or respiratory acidosis. (See p. 80, "Intravenous Therapy.") (5) Cardiac surgery. Paediatric cardiac surgery can now save many babies who formerly succumbed in the neonatal period even when given adequate digitalis. Conditions which

106

THE NEW-BORN

may require immediate surgery in spite of adequate medical care include: (i) preductal coarctation of aorta (excision and anastomosis) (ii) pulmonary atresia with severe stenosis ( valvulotomy) (iii) severe tetralogy with anoxic spells (Blalock operation) (iv) tricuspid atresia (Blalock operation or Potts' procedure) ( v) transposition of great vessels ( creation of an atrial septal defect) (vi) large patent ductus arteriosus with intractable failure (closure) (vii) very large ventricular septal defect with severe failure (banding of pulmonary artery) (viii) an arterio-venous aneurysm with failure such as internal mammary artery or vein of Galen (resection) The Potts' proceduret is not performed ordinarily at this age if a second operation is contemplated. The Glenn proceduret is not usually done before 6 months of age. (6) General Nursing Care. Until they are stabilized, all cyanotic babies, who are prone to apnoeic spells, should be under constant nursing care. Occasionally intravenous fluids are required when oral intake is insufficient. However, care must be taken not to overload the circulation which is already operating under a mechanical defect. (Seep. 80, "Intravenous Therapy.") C. CENTRAL NERVOUS SYSTEM DYSFUNCTION

A birth injury due to a traumatic delivery, anoxia due to placental dysfunction, or cord obstruction often result in a cyanotic baby. The cyanosis is a manifestation of damage to the respiratory centre and is usually accompanied by apnoea, Cheyne-Stokes breathing and occasionally a

CYANOSIS

107

convulsion. Rarely a baby will sustain a spinal cord injury during birth which results in cyanosis. In general, babies with central nervous system dysfunction are very listless with a weak or high-pitched cry and unco-ordinated eye movements. Occasionally there is intracranial bleeding manifested by fresh or xanthochromic fluid in the cerebro-spinal fluid or a subdural haematoma which can be diagnosed by subdural taps. Treatment usually is supportive except in the case of a subdural haematoma, where removal of the fluid by subdural aspiration is required. In some cases neurosurgical removal of the membrane is necessary.

D.SHOCK Fulminating infections may cause a baby to become listless, pale, cyanotic, hypotensive, and to go into shock. Sepsis should be suspected in cases where there is a history of maternal illness or a traumatic delivery associated with an infection in the umbilicus. (For treatment see p. 68, "The Ill Baby.") Occasionally internal bleeding, either intracranial or intra-abdominal from a ruptured liver or spleen, results in shock and a greyish pallor. Rarely the baby bleeds into the mother's circulation or the baby loses excessive blood via the cord during or shortly after birth. Occasionally bilateral adrenal haemorrhage makes a baby listless, pale, and shocked, with the development of peripheral cyanosis. If the baby is hypovolaemic, owing either to internal or to external bleeding, a transfusion is essential. If an adrenal haemorrhage is suspected, intravenous hydrocortisone should be given. If the haemorrhage is extensive the baby usually dies. Very rarely a baby is born with adrenal insufficiency manifested by cyanosis, shock, and an electrolyte upset. (For diagnosis and treatment see p. 68, "The Ill Baby.")

108

THE NEW-BORN

E. TETANY AND CONVULSIONS

Transient cyanosis may be associated with tetany or a convulsion. (For diagnosis and treatment see p. 127, "Twitching and Convulsions.") F. METHAEMOGLOBINAEMIA

Methaemoglobinaemia is produced continuously within the red blood cells of a normal baby by the oxidation of haemoglobin. This methaemoglobin differs from oxyhaemoglobin in that it cannot yield its oxygen to the tissues. Normally the methaemoglobin is reduced back to haemoglobin almost as rapidly as it is formed by an enzyme system, which maintains the level at a small fraction of the total haemoglobin concentration. At birth the level of methaemoglobin may be 0.43 grams % and falls progressively with age to about 0.10 grams % at 1 year. In congenital methaemoglobinaemia there is a deficiency of coenzyme 1 ( disphosphopyridine nucleotide). The affected infant is born with an ashen or dusky violet cyanosis which is unrelieved in 100 per cent oxygen. The blood is chocolate brown in colour. The diagnosis may be proven by spectroscopic examination or by chemical estimation of the methaemoglobin content. 1 Emergency treatment consists of intravenous methylene blue 1 % solution (0.2 ml./kg.) given over 5 minutes. This must be followed by oral ascorbic acid 200-500 mg. daily in divided doses. Certain chemicals may produce an acquired form of methaemoglobinaemia. The most important in the new-born are nitrites found in well water in certain districts. Formulae made with this water produce cyanosis which is frequently 1 0. Armstrong, J. B. J. McKendry, and K. W. Siemon, "Methemoglobinemia in infancy: Two case reports, one of the congenital and one of the acquired variety of methemoglobinemia." CMAJ 79 (Sept. 1, 1958), 295-392.

CYANOSIS

109

misdiagnosed as cyanosis due to congenital heart disease. The cyanosis may be corrected by the administration of methylene blue and its recurrence prevented by using a nitrite-free water. 2 (See p. 179, "Dosages of Drugs.") CONCLUSION

Cyanosis in the new-born infant is a critical disturbance usually involving respiratory, cardiovascular, or central nervous system. Advances in treatment of idiopathic respiratory distress (hyaline membrane disease), new cardiovascular techniques applied to the neonate with fulminating congenital cardiac defects, and improvement in supportive care of the infant with intracranial damage have improved the prognosis of the cyanotic baby. TERMINOLOGY CARDIOVASCULAR OPERATIONS

Blalock operation. This operation is an anastomosis of the subclavian artery to the pulmonary artery, always performed on the side opposite to the aortic arch. Glenn procedure. This procedure is an anastomosis of the distal end of the divided right pulmonary artery to the side of the superior vena cava. Potts' procedure. This procedure is an anastomosis of the pulmonary artery to the aorta. 2 H. Medovy et al., "Cyanosis in infants in rural areas (well-water methaemoglobinaemia)," CMAJ 56 (May 1947), 505.

11. Jaundice

should be considered a paediatric emergency until proven otherwise. JAUNDICE IN THE NEW-BORN

PHYSIOLOGY

Bilirubin, a degradation product of haemoglobin, is present normally in the serum in a concentration of 1 mg. % or less. Levels of 5 mg. % or more produce jaundice. Jaundice before the third day, especially on the first day, is likely due to iso-immunization or infection. Icterus, increasing or appearing after the tenth day, is likely due to biliary obstruction. Overlapping these time intervals are some of the rarer causes of jaundice, for example, virus infections, such as herpes simplex or neonatal hepatitis, and the hereditary defects of the red blood cells. Serum bilirubin is in two forms: the non-conjugated or indirect-acting, and the conjugated or direct-acting portion. Only the non-conjugated is neurotropic. Conjugation of the indirect component requires glucuronyl transferase and uridine disphosphate glucuronic acid enzymes of the liver. These enzyme systems, immature at birth, especially in the premature, are taxed to capacity to form direct-acting bilirubin from physiological post-partum breakdown of the red blood cells. If, in addition, a haemolytic process occurs, the bilirubin concentration rises precipitously. Kemicterus, or staining of the brain substance, especially basal ganglia,

JAUNDICE

111

occurs if the concentration of the u.nconjugated bilirubin reaches critical levels. CLASSIFICATION

A. B. C. D. E.

Physiological jaundice Haemolytic disease Infection Biliary obstruction Hyperbilirubinaemia due to other causes

MANAGEMENT OF THE JAUNDICED NEW-BORN

All mothers should have a determination of Rhesus grouping performed early in pregnancy. If it is Rhesus negative, the blood should be tested for antibodiest at 28 weeks and again at 36 weeks and, if these are present, the titre of concentration should be obtained. 1. If the baby is Rhesus positive and the mother Rhesus negative, a direct Coombs' testt should be done. If it is positive the baby likely has Rhesus incompatibility. 2. If the direct Coombs' test is negative and the blood group of the baby and mother indicate a possible ABO incompatibility (e.g., the mother 0, baby A) an indirect Coombs' testt should be done. If it is positive, the baby has ABO incompatibility. Usually the haemolysin testt on the mother's blood is also positive. 3. If an unknown incompatibility is suspected because of a positive Coombs' test in the baby but there are no demonstrable Rh antibodies in the mother, the antibody can be indentified by testing the mother's serum against a panel of cells of known antigens. 4. The level of indirect ( unconjugated) bilirubin must be estimated and repeated as necessary. A replacement transfusion should be performed, when indicated, to prevent the concentration of bilirubin from reaching a critical level which might cause kernicterus.

112

THE NEW-BORN

5. With incompatibilities excluded and if infection is suspected, a blood culture should be made ancl the baby started on intravenous chloramphenicol. ( See p. 181, "Dosages of Drugs.") 6. Biliary obstruction, congenital spherocytosis, and enzymal defects of the red blood cells require special tests. Medications given to the mother occasionally cause jaundice in the new-born infant. 7. A group of patients, which is becoming gradually smaller through continued research, those with idiopathic hyperbilirubinaemia, still exists and they may require replacement transfusion. A. PHYSIOLOGICAL JAUNDICE

Physiological jaundice in the full term begins about the third day, reaches a peak on the fifth day and subsides gradually by the eighth day. It is likely due to a temporary deficiency of the conjugating enzymes of liver and increased blood destruction. In the premature infant jaundice begins later, may become more intense and last longer. The unconjugated bilirubin level in the serum rarely increases above 12 mg. % in physiological jaundice. B. HAEMOLYTIC DISEASE

1. RHESUS INCOMPATIBILITY Investigative Background

If the red blood cells of a Rhesus monkey are injected into a rabbit, an antibody is produced in the rabbit's serum. This serum will agglutinate the red blood cells of 85 per cent of our general population (Rh positive people) . The 15 per cent lacking this factor are called Rh negative. In 12 per cent of all marriages, an Rh negative woman marries an Rh positive man; theoretically, foetal iso-immunization might occur in about 1 in 10 babies. However, because of heterozygosity in the father ( 4 7 per cent), of

JAUNDICE

113

placental integrity, and because the first-born usually does not develop the disease, only about 1 in 150 babies is affected. The Rh antigens were originally considered to be three linked pairs of genes, represented as Cc, Dd, Ee. If the father is homozygous for C he is designated as CC, if heterozygous Cc. Rh negativity is usually designated as cde/cde but C or E may be present. Since Dis responsible for most cases of Rh incompatibility, the term Rh positive is used to designate a person whose red blood cells include D or a variant thereof. The D positive cells used in testing are CDe/CDe, CwDe/cDE, cDE/cDE. Sensitization in an Rh negative baby is rare but understandable since c, C, e, and E may also act as antigens. Furthermore, new factors have been discovered outside the original six-gene Rh concept, for example: cw, nu, Ex, etc. Other blood group families which also may cause haemolytic disease include Kell, Kidd, and Duffy antigens. Antepartum Recognition Most cases of Rh incompatibility can be diagnosed antepartum. Saline and albumin antibodies develop in the mother when iso-immunization occurs. The saline antibodies which do not cross the placenta are of no significance. Albumin antibodies cross the placenta; studies of these anti-bodies usually are done on the mothers at 28 and 36 weeks of gestation. Titres beginning at 1 : 8 and increasing to 1 : 16 or 1 : 64 are not unusual. If the titre of antibody level is estimated by the indirect Coombs' test (ICT), the levels will be higher than by the albumin method. The prognostic value of the antibody rise is limited in regard to estimation of severity of disease in the foetus. Rarely are antibodies present with the first baby unless the mother has had a previous transfusion or miscarriage. A concomitant ABO incompatibility tends to lessen the Rh sensitization but not the dangers of hyperbilirubinaemia.

114

THE NEW-BORN

Severe Cases Where a mother has a history of sensitization associated with repeated abortions, stillbirth, or hydrops, referral early in pregnancy to a "Rhesus Centre" is indicated. These centres are able to determine the probable zygosity of the father, and the degree of sensitization of the baby by bilirubin determination ( chemical or optical density testing) of the fluid obtained by serial amniocentesis. An adequate decision can then be made as to the need for an intrauterine transfusion and the best time to induce labour, based on the previous maternal history and on adequate knowledge of the compromise required between the problem of haemolysis and the complications of prematurity. Clinical Evaluation At delivery the infant may appear perfectly normal or show various degrees of pallor, lethargy, or oedema. The vernix caseosa and cord may be yellow, but the baby is not. Liver and spleen may be of normal size or show varying degrees of hepatosplenomegaly. Within a few hours the baby usually develops jaundice, which may increase markedly. Occasionally petechiae develop, usually indicative of thrombocytopenia and capillary fragility. If the foetus has been sensitized early, the baby may have a pallor, indicative of severe anaemia, with little sign of jaundice. Severe sensitization may produce a pale oedematous baby with impending heart failure. Laboratory Findings The baby's red blood cells are Rh positive, the mother's Rh negative, and the direct Coombs' test is positive. The haemoglobin varies from normal to less than 10 gm. % . At birth the cord bilirubin may be slightly elevated or over 4 mg. % . The peripheral blood smear may show normoblasts well in excess of a normal of 10 per 100 white blood

JAUNDICE

115

cells. Reticulocytes may rise from the normal of 6 per cent up to 40 per cent or 50 per cent. With the hourly increase in the baby's age, clinical signs become more pronounced and the bilirubin level may rise rapidly to a level of 40 or 50 mg. per cent. Treatment Most babies affected with haemolytic disease due to Rh sensitization will require replacement of their blood with suitably matched Rh negative blood to avoid kernicterus. This complication, which may occur in the full term on the third or fourth day, and in the premature on the sixth or seventh, is characterized by hyperirritability, poor sucking reflex, muscle twitching, and a high-pitched cry. Recovery may take place or progressive brain damage with convulsions and opisthotonos may develop. The bilirubin level is the best laboratory guide as to whether a blood replacement is necessary. If the cord bilirubin (serum) is over 4 mg. % at birth, or if the serum level is over 8 mg. at 7 hours, 10 mg. at 12 hours, 15 mg. at 24 hours, or 20 mg. at 48 hours, a replacement usually is indicated ( see Fig. 2) .1 In 50 per cent of these cases two or more replacements are necessary. Serial estimations ( sometimes every 2-3 hours) of bilirubin should be done until the level begins to fall. No one test is an absolute guide. There are situations where a replacement should be done below these levels, as well as a few instances where a replacement may not be necessary at these levels. Prematurity is a case in point; here, because of liver enzyme immaturity, replacement is done at lower levels of bilirubin. Haemoglobin levels are helpful. Normally at birth the level should be 20 gm. % , gradually falling to 12 gm. % lFred H. Allen, Jr. and Louis K. Diamond, Erythroblastosis Fetalis (Boston, 1958), p. S1.

116

THE NEW-BORN ___ ,.......

20

,,,.,. i..----

(/) CD

_ Zone of obligatory 2 15 exchange transfusion 3

_,,(

I/ ~

0 0

~

5

,,v I ~,,;:.'

/

•'!',.'ll-

V

,.......

_,,. ,,,.,.

~

i---

,.......

,,,.. i..----

,,;:.~~ ./

,,/

/

/

V

I'll

1,0~

✓

"O

/l

~

/

I

/

v

A

,,,.,. ,,,.,.

~

V

/

Zone in which exchange transfusion not Indicated on basis of bilirubin alone though it may btl Indicated on other grounds

... I-

0

3 6

9

12

15 18 ,21 24 27 30 33 36 39 42 45 48

AGE hr.

FIG. 2. The Relation between the Levels of Bilirubin and Age which Indicate the Need for a Replacement Transfusion

at 4 months of age. A ·cord blood level of 14 or less is an indication for replacement. Moreover, low levels may be associated with impending cardiac failure. When the umbilical vein is used in the replacement, the auricular pressure may be measured readily. A pressure of 10 cm. or more of water is indicative of heart failure. Ideally the pressure should be maintained between 5 and 7 cm. of water. The technique of exchange transfusion and blood required are described on p. 164. The procedure is not

JAUNDICE

117

without danger, as the mortality is 1 per cent even in the most experienced hands. However, untreated affected infants run a 10-15 per cent chance of developing kemicterus. Weekly or bi-monthly haemoglobin estimations should be performed on these babies after discharge from hospital. Up to 2 months of age a blood transfusion with compatible Rh negative blood is required if the haemoglobin falls to 8 gm. % or below. Oral iron is ineffectual and unnecessary as long as haemolysis persists. 2. ABO

INCOMPATIBILITY

In the general population 45 per cent of people are Group 0, 41 per cent group A, 10 per cent group Band 4 per cent group AB. A group O mother's serum contains anti-A and anti-B naturally occurring antibodies, which do not traverse the placental barrier and therefore do not cause foetal damage. Some mothers also produce hyperimmune antibodies, which likewise cross into foetal circulation. These cause haemolysis. If the antibody is of the hyper-immune A type and enters foetal circulation of a group A baby, haemolysis occurs. Since prior sensitization is not required, a first-born child, in contrast to the situation with Rh incompatibility, may be affected. Usually the mother is group O and the baby group A; occasionally the mother is group O and the baby group B; very rarely the mother is group A and the infant B, or the mother group B and the infant group A. Unlike the possibilities with Rh incompatibility there are no diagnostic tests prior to delivery that give evidence of foetal sensitization. As with Rh incompatibility not all babies are affected. Approximately 1 in 4 of all pregnancies are hetero-specific, but only 1 in 15 babies are affected by the incompatibility. At birth the infant may appear normal or may exhibit mild jaundice within 24 hours. The jaundice seldom

118

THE NEW-BORN

becomes intense and hepatosplenomegaly is not usual. However, rarely, the condition may be indistinguishable clinically from a severe affected Rhesus incompatibility. The indirect Coombs' test and enzyme tests ( e.g. papain and trypsin) are positive; the direct Coombs' test is usually negative or weakly positive. Haemolysins against the infant's cells may be demonstrated in the mother's serum. Spherocytes are more plentiful than in Rh incompatibility; nucleated red blood cells are not as numerous as in Rhesus incompatibility. Serial bilirubin estimations are necessary. The indications for replacement transfusions, using group O neutralized blood, are the same as in Rhesus sensitization. C. INFECTION AS A CAUSE

If Rhesus and ABO incompatibility have been excluded as causes, infection is the next consideration. First a blood culture should be made and a Mazzini testt performed for congenital lues. Several urine specimens should be examined. If pus cells are present, a catheter specimen should be cultured. The cord stump should be carefully examined, and a culture made if indicated. In the severely ill infant a lumbar puncture should be done to rule out meningitis. Petechiae, mild jaundice, lethargy, hepatosplenomegaly, pyrexia, and thrombocytopenia suggest sepsis. The antibiotic of choice is chloramphenicol, given intravenously with appropriate parenteral fluid, until the sensitivities of the isolated organism are available. (See p. 181, "Dosages of Drugs.") If bacteriological studies give negative results, a urine sample should be examined for cytomegalic inclusions, as well as blood taken for complement fixation and Sabin dye tests for toxoplasmosis. Radiographs may reveal intracranial calcifications which are characteristic of either infection. Occasionally infants develop jaundice concomitant with

JAUNDICE

119

a severe diarrhoea. These babies usually die. Post mortem examination discloses a hepatitis, the result of an ascending infection. Herpes simplex transmitted from the mother requires viral transmission and complement fixation studies. To date there are no specific laboratory tests to confirm the clinical diagnosis of neonatal hepatitis, which may be caused by the virus of infectious hepatitis or of serum hepatitis. However, occasionally, a Coxsackie-B or other virus may be isolated. D. BILIARY OBSTRUCTION

Jaundice increasing or continuing after the tenth day is likely a result of some form of biliary obstruction. The infant may be born with a congenital obliteration of some portion of the biliary duct system which may be extrahepatic or more commonly intrahepatic. Congenital absence of all intrahepatic ducts is very rare. The parenchymal liver cells may show gradations of damage from bile staining to advanced biliary cirrhosis, depending on the extent of the obstructive defect and the age of the patient. A few extrahepatic defects, for example, choledochal cyst, atretic common duct, stenotic hepatic duct, and a gall bladder which drains into the duodenum but is not connected to the common bile duct, are correctible by surgery. If biliary obstruction is suspected, the infant should have operative cholangiography and liver biopsy, in order to ascertain the nature of the defect. If the correction is accomplished, continuing irreversible cirrhosis will be prevented. However, to complicate the problem, neonatal hepatitis, in the obstructive phase, because of the inflammatory swelling of the cords, is often indistinguishable from biliary atresia. Surgery in hepatitis is not necessary and may be harmful. In atresia and hepatitis, jaundice may be present at, or shortly after, birth. For a period both conditions stimulate icterus neonatorum. However, if jaundice continues or

120

THE NEW-BORN

increases after 10 days, other diagnoses must be considered and further investigations pursued. Usually the baby with neonatal hepatitis appears ill, whereas the infant with atresia appears rather well, apart from the jaundice. To compound the confusion, the stool of the baby with atresia may be coloured at times, owing to the sloughing of the heavily bilirubin-laden intestinal epithelial cells, but usually has very little bile. Jaundice may also vary in intensity. The serum bilirubin level becomes elevated in both conditions and a large proportion is of the direct type. Urobilin levelst in the urine, urine bilirubin concentrations, serum bilirubin levels, and urobilinogen and bile estimations in stool vary in both atresia and hepatitis. Liver function studies are abnormal in both, but the deviations are not differentially specified. However, daily examination of stool for bile pigments and urine for bile and urobilin may suggest that obstruction is incomplete. This information, correlated with the clinical status and repeated liver function tests, especially van den Bergh, t alkaline phosphatase and serum glutamic oxalic transaminase (S.G.O.T.), may indicate that neonatal hepatitis is the likely diagnosis. A rose bengal dye test showing incomplete obstruction (i.e. more than 10 per cent dye excretion in stool) would justify postponement of any operation. In many cases, differentiation is not possible without operative cholangiography and liver biopsy. In any event the investigation should be completed and a decision regarding operation should be made within two weeks. E. HYPERBILIRUBINAEMIA DUE TO OTHER CAUSES

Any infant who develops serum bilirubin above 12 mg. % in the first week of life may be said to have hyperbilirubinaemia. It is most important to note that, regardless of the aetiology of the hyperbilirubinaemia, these infants require serial bilirubin estimations since kernicterus is a

JAUNDICE

121

hazard and replacement transfusion may be required. A correlation of the level of the unconjugated bilirubin with the age of the patient is the best guide as to the necessity of this procedure. 1.

UNUSUAL Iso-IMMUNIZATION DUE TO MINOR

BLOOD FACTORS

These factors may cause severe jaundice. Their presence or absence can best be determined by performing a Coombs' test on the baby's cells after incubating them in the mother's serum, providing that ABO compatibility exists between mother and baby. The specific antibody, if present, is identified by testing the mother's serum against a panel of cells of known antigens. 2.

CONGENITAL SPHEROCYTOSIS

Fragility tests should be done if there is a family history of congenital haemolytic anaemia, or if there is a preponderance of spherocytes in the red blood cells of the peripheral smear. These tests may be difficult to interpret in the infant for the first three months. In a suspected case where there is no family history of the disease, fragility tests on the members of the family are necessary. ABO incompatibility must be excluded. A replacement transfusion is required if the indirect bilirubin rises to a critical level. 3.

EN-ZYMAL DEFECTS OF LIVER

(a) The full-term new-born infant is deficient in the enzyme glucuronyl transferase necessary for normal conjugation and excretion of bilirubin. The premature infant has a still greater deficiency of enzyme and a greater delay in its development after birth, which may result in hyperbilirubinaemia. Factors related to birth, such as anoxia and excess vitamin K may also delay the development of this

122

THE NEW-BORN

enzyme system. Anoxia with prematurity will result in a combined defect. ( b) There is a familial condition in which there is a delay in the development of the glucuronyl transferase enzyme system. In these cases the mothers may have several infants affected with a rather marked degree of "physiological jaundice." Ordinarily these infants do not develop kernicterus unless an additional factor is present, such as prematurity, anoxia or drug administration. ( c) In Crigler-N ajjir syndrome the glucuronyl transferase enzyme system does not develop. Such infants have a severe form of jaundice with high levels of indirect bilirubin. Frequently the hyperbilirubinaemia reaches a level which will produce kernicterus even beyond the first few weeks of life. The prognosis in this condition is poor. 2 Replacement transfusions often are required. ( d) In Gilbert's disease there is also a rise in indirect bilirubin but this is slight. The condition tends to be benign. It is thought that there is a reduction of glucuronyl transferase enzyme although this has not been definitely proven. ( e) In the Dubin-Johnson syndrome both direct and indirect bilirubin levels increase. There is conjugation of the indirect bilirubin within the liver cells; however, the enzyme which is responsible for excretion of conjugated bilirubin from the cell is defective. These patients remain permanently jaundiced. 4.

DRUGS

Certain drugs which are given to the mother immediately prior to birth or to the infant in the first few days of life may produce hyperbilirubinaemia. An excessive amount of vita21. F. Crigler and V. A. Najjir, "Congenital familial non-hemolytic jaundice with kemicterus," Paediatrics 10 (1952) 169-80.

JAUNDICE

123

min K increases haemolysis leading to greater increased production of indirect bilirubin within the first few days. One mg. of vitamin K1 oxide is sufficient for prothrombin formation and will not cause jaundice in the premature or full-term new-born. Sulphonamides also tend to produce a rise in the indirect bilirubin and should not be used in the neonate. There is a danger also in the long-acting sulphonamide preparations given to the mother immediately prior to delivery. The sulphonamides are especially hazardous since these interfere with the serum binding of bilirubin, and kernicterus may develop at a lower level of bilirubin than in other cases. Chloramphenicol competes with bilirubin for conjugation and reduces the amounts of available glucuronyl transferase. However, infants on excess amounts of this drug usually have difficulty because of chloramphenicol excess ( the gray syndrome) rather than jaundice.

5.

RED CELL ENZYMAL DEFECTS

Infants who have an inherited deficiency of glucose-6phosphate dehydrogenase or pyruvate kinase may show hyperbilirubinaemia of severe degree in the new-born period to require a replacement transfusion. These infants are frequently of Chinese, Mediterranean, or Negro parentage. Such infants are very sensitive to drugs which, associated with the enzyme deficiency, produce severe haemolysis. These drugs include vitamin K, sulphonamides, and salicylates. The diagnosis may be suspected by finding Heinz bodies in the blood smear. The methaemoglobin reduction test is required to establish the diagnosis.

6.

GALACTOSAEMIA

This may present with jaundice in the new-born period. It is related to hepatocellular damage with increase in both bilirubin components. The urine is "Clinitest" positive and

124

THE NEW-BORN

"Urostix" negative since the latter is a specific test for glucose. When this test is done it is important that the infant be on a milk feeding. If the test is positive, milk must be eliminated and a substitute feeding given, such as Nutramigen. 7.

BREAST MILK

In some mothers there is a steroid (pregnane-3alpha 20 beta-diol), excreted in breast milk which inhibits glucuronyl transferase. These infants usually develop a mild form of jaundice which clears when they are taken off the breast. This is one of the few contra-indications to breast feeding. 8.

RARE CAUSES

A few infants with prolonged physiological jaundice have been proven to have cretinism or cystic fibrosis. Hypertrophic pyloric stenosis developing near the end of the neonatal period rarely may have an associated jaundice. 9. IDIOPATHIC CAUSES

There still exists a group of patients in whom no known cause has yet been found for the hyperbilirubinaemia. The number, however, decreases with increasing knowledge. In all cases where the unconjugated level of bilirubin is rising toward critical levels, the indications for replacement transfusion remain the same. CONCLUSION

Jaundice may be indicative of several conditions amenable to specific treatment, the prognosis in each is dependent on the time required for appropriate therapy to be instituted. Every jaundiced infant, in addition to a most thorough clinical appraisal requires corroborative laboratory investigation.

JAUNDICE

125

TERMINOLOGY

Antibodies (Rh) Two types of antibodies may be demonstrated in the serum of the mother about to deliver a Rhesus sensitized infant. The antibodies found in saline solution do not cross the placental border, and hence are of no clinical importance. The blocking antibodies, which are demonstrable in albumin or colloidal suspensions, cross into foetal circulation, causing haemolysis. Direct Coombs' Test This test detects the presence of antibody on the red blood cells. Washed infant's cells are added to anti-human globulin, centrifuged, and examined for agglutination. If agglutination occurs, the test is positive. Haemolysin Test Haemolysins are present in the serum of a mother delivered of a baby with ABO incompatibility, and may be identified long after the papain and indirect Coombs' tests become negative. If haemolysis occurs when the baby's cells are incubated in the mother's serum, the haemolysin test is positive. Indirect Coombs' Test This detects the presence of circulating antibodies in the serum. Adult A1 or B cells ( depending on the infant's group) are incubated in the baby's serum, washed and then suspended in anti-human globulin. If agglutination occurs, the test is positive. This test usually remains positive longer than the papain test, becoming negative at the end of the third day. Mazzini Test This is a serological test for congenital lues and may be done in 25 minutes. Although hypersensitive, the test requires much less time than the more involved tests done for syphilis. Papain Test (an enzymal test) The baby's serum plus papain, plus adult A 1 or B cells ( depending on the baby's group) are incubated for 15 minutes and examined. If agglutination takes place, the test is positive. The papain test may be completed in 20 minutes, whereas the

126

THE NEW-BORN

indirect Coombs' test takes 1½ hours. However, occasionally the test is negative on the cord blood and tends to become negative earlier than the indirect Coombs' test. Rose Bengal Test Rose bengal dye (1 microcurie of 1131 labelled) is injected intravenously. Stool collection (free from urine) is done for 72 hours. Radioactivity of collected stool is estimated by Well's scintillation technique. In biliary atresia below 5 per cent of the dye is excreted in the stool. In neonatal hepatitis excretion is above 11 per cent. Urobilin and bilirubin Bilirubin is produced by the reticulo-endothelial breakdown of haemoglobin into porphyrin, iron, and globulin. From porphyrin, unconjugated bilirubin is formed and, after conjugation in the liver by glucuronyl transferase, is excreted into the gut. A portion is transformed by bacterial action into stercobilinogen. Some of the stercobilinogen is oxidized to stercobilin and excreted in the stool, and a small portion is re-absorbed into circulation as urobilinogen, and excreted by the kidney as urobilin. The rest of the stercobilinogen is re-absorbed as urobilinogen, and excreted by the liver into the gut. Van den Bergh Test This is a measure of the total (unconjugated and conjugated) bilirubin levels. The unconjugated portion is called the indirect bilirubin, and the conjugated is called the direct. The total normal level is less than 1 mg. % with a conjugated (direct) portion less than 0.5 mg. % . When jaundice, in Rhesus incompatibility, remains marked, in spite of a replacement transfusion, an inspissated bile syndrome is the likely diagnosis if a high conjugated level is obtained.

12. Twitching and Convulsions

A convulsion in a new-born requires immediate treatment to prevent brain damage and subsequent investigation of the aetiology to prevent if possible its recurrence. Normal infants will frequently exhibit tremulous movements of the chin or an extremity. This benign condition may, at times, be confused with twitching, which is a sign of the nervous system irritability that accompanies organic disease. Isolated myoclonic jerks also may occur in a normal new-born; if these recur frequently and persist, careful neurological investigation is indicated. PATHOPHYSIOLOGY

Although the brain is proportionately large at birth, it is structurally and functionally immature. Cortical activity, as shown by the electroencephalogram, is poorly developed. The immaturity of the central nervous system, however, appears to protect the new-born against certain noxious influences. Th6refore, a new-born can withstand levels of hypoglycaemia and anoxia which might produce convulsions or permanent brain damage in an older child. Idiopathic epilepsy and simple febrile seizures rarely appear in the neonatal period. Seizures which do occur at this time indicate a major abnormality affecting the function of the central nervous system. The most frequent cause of convulsions in the first week is intracranial damage (haemorrhage, oedema, or anoxia). Tetany related to low serum levels of ionized calcium and

128

THE NEW-BORN

hypoglycaemia are important metabolic causes of seizures. Infection of the central nervous system often is associated with convulsions and severe generalized infection also may produce convulsive episodes. These episodes may be caused by direct toxic effect on the brain or by upsets in water, electrolyte, or acid-base balance. Anoxia occurs in a number of serious diseases of the new-born and may be a causative factor. Congenital defects of the central nervous system may produce seizures. There are a number of rare metabolic defects including pyridoxine deficiency or dependency, maple syrup disease or hyperglycinemia. Phenylketonuria may be associated with seizures but these are seldom seen in the neonate. An infant with kernicterus may have convulsions or more commonly, twitching episodes. The infant with narcotic withdrawal may show twitching, and in some parts of the world neonatal tetanus occurs producing seizures. The possible categories for causes of a seizure will be described more fully below. Regardless of aetiology, a convulsion may be generalized or involve a localized area of the body. Immediate treatment is imperative since a prolonged convulsion predisposes to anoxia and cerebral oedema which, in tum, may lead to permanent neurological damage. CLASSIFICATION

A. Intracranial damage B. Tetany C. Hypoglycaemia D. Infection E. Electrolyte disturbances F. Recurrent seizures MANAGEMENT 1. The airway should be cleared by gentle suction and

adequate oxygen supplied by mask.

TWITCHING AND CONVULSIONS

129

2. Sodium phenobarbital 8 mg. (gr.1/s) or paraldehyde 0.2 ml./kg. body weight should be given intramuscularly. 3. Blood should be taken for sugar, calcium, and phosphorus determinations, then an intravenous cut-down started using a solution of equal parts of ¾ 5 % glucose---¼ normal saline and 10% glucose in distilled water. (See p. 80, "Intravenous Therapy.") 4. Five ml. of 10% calcium gluconate diluted to 2% with the intravenous solution in the Pedatrol should be given while awaiting the laboratory results. 5. If the convulsion persists in spite of this therapy, 3--4 ml. of 50% glucose should be given intravenously. Most convulsions in the neonate will be controlled by the measures outlined. 6. If the seizure continues longer than 15-20 minutes and if respirations are adequate and unobstructed, paraldehyde may be given intravenously as a 5 % solution ( 5 ml. of paraldehyde to every 95 ml. of the infusion). The mixture is run in slowly ( since cumulative effects of anti-convulsant drugs may cause serious respiratory depression) until the baby has received a maximum of 2 ml./kg./body weight of the 5 % solution. 7. If an anaesthetist is available, he can ensure an adequate air exchange is maintained by intubation and assisted respiration if necessary. If the seizure is not controlled by the paraldehyde infusion, a general anaesthetic should be given. 8. As soon as the seizure is controlled, further investigation should be made as quickly as possible. By this time, results of the estimations of calcium, phosphorus, and blood sugar should be available. The following procedures should be completed: (a) A rapid but comprehensive re-assessment of the infant should be done, noting particularly tension of fontanelles, pupil-size and response, haemorrhages in ocular

130

THE NEW-BORN

fundi, signs of skull trauma, muscle tone, level of consciousness, signs of infection ( which may be petechiae, omphalitis, or enlarged liver and spleen), cardiac and respiratory status. ( b) Lumbar puncture should be performed for evidence of subarachnoid bleeding or infection. (c) Radiographs of skull (for signs of fracture or calcification) and chest ( for signs of pneumonia) should be taken. ( d) Blood should be taken for making a culture and for estimating the serum pH, carbon dioxide content, sodium, potassium, and chloride values. ( e) Subdural taps are indicated if the fontanelle is tense or bulging. (/) An electroencephalogram may be useful in assessing brain damage. A. INTRACRANIAL DAMAGE

Trauma or anoxia occurring during labour and delivery are the usual causes of brain injury. Intracranial haemorrhage may occur from tearing of the small veins of the tentorium, the vein of Galen, or the pial veins near the superior longitudinal sinus which may produce a subdural haematoma. Premature infants are more susceptible to intracranial haemorrhage. Breech delivery increases the risk of intracranial damage. Anoxia during labour, delivery, or resuscitation has a varied aetiology. Inadequate oxygenation or over-sedation of the mother during delivery or undue delay in decision for a necessary Caesarian section are examples of preventable causes. Other conditions leading to an anoxic infant such as prolapse of the cord may be unavoidable. The infant may have cerebral oedema or multiple petechial haemorrhages in the brain. The anoxic infant is frequently inactive with a weak, high-pitched cry and has feeding difficulties.

TWITCHING AND CONVULSIONS

131

This may occur in infants with respiratory or cardiac problems or in those in shock. Anoxia may also occur as a complication of the seizure. Adequate oxygenation of the convulsing infant is important to prevent cerebral damage. Convulsions due to intracranial haemorrhage usually begin in the first 24 hours but may occur later with a subdural haematoma. When intracranial damage is suspected, the physician who has performed the delivery must conscientiously appraise the possible aetiological factors including: (i) prematurity (ii) sedatives and anaesthetic agents used on the mother (iii) associated medical illness in the mother, e.g. cardiac failure (iv) precipitate delivery or prolonged labour ( v) vitamin K administered to mother or infant (vi) abnormalities related to cord or placenta (vii) obstetrical problems such as breech or face presentation The physical signs, investigation and management of intracranial damage are outlined above in the chapter on "The Ill Baby." TETANY

Convulsions in the neonate may be due to a low level of ionized calcium. Temporary parathyroid insufficiency and/ or defective phosphorus excretion by the kidney have been considered the causes of this condition. The relationship of cerebral damage to hypocalcaemic tetany is difficult to assess. Infants who have come through prolonged labour frequently have increased levels of serum phosphorus which may be associated with decreased serum levels of ionized calcium. This is probably a secondary effect on renal or parathyroid function. The infant with hypocalcaemia may exhibit twitching and

132

THE NEW-BORN

strider as well as convulsions. Since Chvostek's sign may be elicited in normal infants, this is not a reliable test. Trousseau's sign may be elicited using a blood pressure cuff inflated above the arterial systolic pressure for 3 minutes. Carpal spasm indicates a positive test, but this also may be difficult to assess in the new-born period. Total serum calcium is decreased below normal values and serum inorganic phosphorus usually is raised. The infant has already received 5 ml. of 10% calcium gluconate as part of initial treatment and a further 5 ml. diluted to 2 % concentration should be given intravenously once the diagnosis is established. The intravenous administration of this dose should take at least 30 minutes. The dose of 10 ml. of 10% calcium gluconate diluted to 2% solution should be repeated every 4 hours as needed, depending on serum calcium values obtained. If bradycardia and vomiting occur, the intravenous calcium should be discontinued and the serum level re-estimated for possible hypercalcaemia. The infant should be given adequate fluids but over-hydration should be avoided. (See p. 80, "Intravenous Therapy.") When the infant has recovered from the acute episode, formula feeding is given with 15 ml. of Basaljel ( aluminum carbonate) added to the daily formula. Calcium gluconate syrup (Sandoz) 5 ml. (1 teaspoon) daily orally is used to maintain normal serum levels. If calcium levels cannot be maintained with this dosage, vitamin D2 may be necessary in a dose of 10,000 I.U. or more daily. Such cases should be referred to a centre equipped to perform metabolic studies since precise biochemical control is difficult and needs full and frequent investigation.

C.HYPOGLYCAEMIA An abnormally low blood sugar now is recognized as a more frequent cause of convulsions in the new-born.

TWITCHING AND CONVULSIONS

133

Although the normal neonate may have low blood sugar values, it is advisable to treat any infant who has a blood sugar below 30 mg. % associated with symptoms of pallor, twitching, or convulsions. Any infant with a blood sugar below 20 mg. % should have intravenous glucose therapy regardless of clinical status. Many causes of hypoglycaemia are known, but its exact aetiology in most cases seen in the first week is obscure. Infants of diabetic mothers usually have more serious trouble with respiratory distress, but the development of hypoglycaemia should also be suspected. Another group prone to hypoglycaemia are the infants with intrauterine growth retardation (see p. 39, "Care of the Premature Infant"). This is especially true if the mother has had toxaemia. The condition is thought to be due to depletion of glycogen stores. Management The infant with hypoglycaemia will already be receiving intravenous glucose-saline solution and possibly one dose of 50% glucose while awaiting results of laboratory investigation. This treatment should be continued once diagnosis is established by injecting 2-3 ml. doses of 50% glucose into the intravenous tubing often enough to keep the blood sugar at a safe level ( about 30 mg. % ) and the infant free of convulsions. Oral feeding is started while intravenous therapy is continued, at a decreasing rate, to prevent rebound hypoglycaemia. If normal blood sugar level cannot be maintained without intravenous therapy, the infant should be started on prednisone (2 mg./kg./day in four equally divided doses). In most cases the blood sugar will be stabilized in one week but other patients will require 5 or 6 weeks of therapy. Prednisone treatment should be discontinued gradually.

134

THE NEW-BORN

In those cases where steroids are required, and especially if these must be continued for more than a week, the infant should be transferred to a centre for intensive investigation for rare types of hypoglycaemia. These include: (a) Glycogen storage disease. This occasionally appears in first week. The liver is large. Convulsions are unlikely. ( b) Galactosaemia. Reducing substances are found in the urine when the infant is taking a milk feeding. True blood glucose level is low and no glucose is found in the urine. Fructosaemia may also cause hypoglycaemia. ( c) Adrenal insufficiency. In the neonate this usually causes more problems with electrolyte disturbances ( see p. 68, "The Ill Baby"). (d) Pituitary insufficiency. This rarely produces recognizable symptoms at this age. ( e) Idiopathic leucine-sensitive hypoglycaemia. This usually does not appear until after 1 month of age. (/) Islet-cell tumours. These are unknown in infants. Hyperplasia of islet cells has occurred in infants of diabetic mothers, and in erythroblastosis. It should be noted that prolonged convulsions may produce hypoglycaemia because of rapid depletion of glycogen stores in the new-born infant. The treatment outlined will prevent secondary hypoglycaemia from developing. Fifty per cent glucose is also useful since it helps to reduce cerebral oedema temporarily. E. ELECTROLYTE DISTURBANCES

Profound disturbances in body electrolytes may produce seizures. Administration of a feeding high in electrolytes such as protein or skimmed milk to a new-born with diarrhoea may produce hypernatraemia. This condition often is associated with convulsions in the recovery phase

TWITCHING AND CONVULSIONS

135

and there may be permanent brain damage. Water intoxication may also occur in the new-born, caused by giving an excess amount of glucose in distilled water intravenously to an infant who is depleted of salt. (Seep. 80, "Intravenous Therapy.") D.INFECTION

Bacterial meningitis and other infections of the central nervous system, such as toxoplasmosis and cytomegalic inclusion disease, are frequently associated with convulsions. Diagnosis of meningitis usually is confirmed by lumbar puncture. Treatment is described in the chapter on "The Ill Baby." Generalized infection may result in cerebral irritability including twitching and convulsions. With signs of infection and normal spinal fluid, a careful search should be made for other sites of infection. A blood culture, urinalysis, and radiograph of chest may be useful. F. RECURRENT SEIZURES

Recurrent convulsions which are not controlled by the suggested management require intensive neurological investigation to determine a cause. Pyridoxine deficiency or dependency is a rare metabolic disorder; 25 mg. of pyridoxine intramuscularly usually will control the seizures. A therapeutic trial could be given while awaiting the results of other investigations. An amino acid and sugar chromatogram of serum and urine may reveal other rare disorders such as maple syrup disease. Congenital defects of brain often are associated with other evidence of neurological deficits or congenital abnormalities. Pneumoencephalography may be necessary to discover the defect but is rarely justified at this age.

136

THE NEW-BORN

SUMMARY Twitching and convulsions in the new-born usually result from cerebral damage, metabolic disturbances, or infection. Prompt and precise treatment is required to prevent death, permanent neurological sequelae, or mental retardation. After the seizure is controlled, a careful investigation is required to find the cause since idiopathic and simple febrile seizures are almost unknown at this age.

13. Medications and the Mother

is the byproduct of potency of any drug administered to the body. The toxic effects of many drugs are minimal, transitory, and usually clinically unrecognizable. Moreover, the use of a drug even when the risk of serious toxicity is known may be warranted when this risk is weighed against the gravity of the illness. It has unfortunately become part of modem thinking that for every transitory disturbance or minor worry, science has a medicinal antidote. The medical profession acquiesces in this assumption when a physician, harassed by more urgent medical problems, finds it easier to prescribe than to explain. Since knowledge of the effects of drugs, especially their teratogenicity, is incomplete, medications, particularly for the pregnant woman, should be used only when there is a very specific indication for them. Medications affecting the infant may be divided into three groups: 1. Teratogenic drugs 2. Those causing signs of narcotic withdrawal 3. Those which create toxic effects by passive transfer TOXICITY, ALWAYS PRESENT AND UNDESIRABLE,

TERATOGENIC DRUGS

Thalidomide and busulphan (Myleran) administered to the mother may cause congenital abnormalities in the foetus. Quinine and amethopterin, which have been used by

138

THE NEW-BORN

women in attempts to produce abortions, have been associated with foetal malformations. Isolated reports also incriminate cortisone in high doses and hypoglycaemic agents. NARCOTIC WITHDRAWAL

A mother's addiction to morphine, or one of its derivatives, is accompanied often by drug withdrawal signs in the new-born infant. These manifestations are excessive restlessness, irritability, yawning, vomiting, diaphoresis, and in severe cases, convulsions and death. Treatment consists of giving the baby diminishing doses of Methadon (seep. 179, "Dosages of Drugs"), in addition to supportive and symptomatic care. TOXIC EFFECTS OF DRUGS BY PASSIVE TRANSFER

Oral progestins, anabolic agents, and occasionally stilbesterol may cause masculinization of the female infant when administered to the pregnant woman. Large doses of vitamin K and sulphonamides given to the mother near term may produce jaundice in the infant with a sufficiently high level of bilirubin to cause kemicterus. For indications that replacement transfusion is required to prevent this catastrophe see the chapter on "Jaundice." Phenothiazine drugs such as Largactil, Sparine, and Trilafon may produce jaundice by a mechanism of bile stasis. Reserpine may cause nasal congestion, intercostal retraction, lethargy and anorexia in the baby. Dilantin, salicylates, and Tridione may cause a thrombocytopenia with bleeding. Primaquine and nitrofurantoin may produce a haemolytic anaemia in a baby who has a glucose-6-phosphate dehydrogenase deficiency. Hexamethonium bromide has been reported associated with a paralytic ileus in the premature. Iodine compounds and propylthiourical may cause a goitre to develop in the

MEDICATIONS AND THE MOTHER

139

new-born baby. Although adrenal steroids administered to the mother usually have no serious effect on the baby, there is occasionally a suppression of the foetal adrenals with cortical cystic changes, which results in acute adrenal insufficiency. Tetracycline may be associated with growth retardation and deposition of the drug in bones and teeth, and gives permanent discolouration to the teeth. Terramycin may be deposited in the bones and teeth. Streptomycin administered to the mother may damage the eighth nerve of the infant. Chloramphenicol may cause bone marrow depression. Barbiturates, opiates, and peperidine (Demerol) may cause respiratory depression in the baby. Phenothiazine administered to the mother may increase the depressant effect of narcotics and barbiturates. In the management of labour and delivery a sustained adequate level of oxygen in the infant is most important. At no time is judgment, skill, and judicious use of drugs by the physician more important to the baby than at his delivery. CONCLUSION

Since our knowledge of the effects of maternal medication in the foetus is limited, drug therapy during pregnancy should be minimal and specifically indicated. A physician, aware of the now known toxic effects on the foetus of medications given to the mother, may, by alertness and continuing observation, recognize new associations which will add to medical knowledge in this recently expanding field.

14. Congenital Defects

of the aetiology of many congenital defects is increased by continual medical research, remedial measures for ·concomitant disabilities are being developed slowly but steadily. The hope of the future is complete prevention. Some abnormalities are determined environmentally, many have a genetic origin. AS OUR KNOWLEDGE

ENVIRONMENTAL INFLUENCES

Environmental influences include maternal nutrition, infection, and radiation as well as pre-natal and birth injury. Maternal-foetal incompatibility is outlined in the chapter on "Jaundice." The effects of medications given to the mother and their effects on the foetus are discussed also in the previous chapter. Nutrition Severe malnutrition and diets deficient in calcium, phosphorus, and vitamins A, B, C, and D, have contributed to foetal malformations. Infections Maternal illnesses during pregnancy which have been associated with foetal defects ( often mental retardation) include smallpox ( variola), chickenpox ( varicella), Asian influenza, cytomegalic inclusion-body disease, equine encephalitis, syphilis, typhoid fever, tuberculosis, toxoplasmosis, malaria, infectious hepatitis, and German measles (rubella).

CONGENITAL DEFECTS

141

Rubella ( German measles), contracted during the first trimester, especially during the first month, has been associated with an appreciable incidence of abnormalities ( deafness, cataracts, cardiac defects, microcephaly, and mental retardation). The disease may occur in the mother without a rash. Some authorities question in this connection the value of administration of gamma globulin, but until more evidence is available 20 ml. of human immune gamma globulin should be given intramuscularly to all pregnant women who have not had German measles previously if they are exposed to it in the first trimester of pregnancy. Infectious hepatitis may run a severe and prolonged course in adults. If a pregnant woman is exposed, she should receive 0.06 ml./kg./body weight of immune serum globulin intramuscularly. Radiation Since it is known that radiation can produce mutations in germ cells or damage a young foetus, radiography should be minimal and for very specific reasons, especially in the pelvic region during the early stages of pregnancy. Injury

Mechanical injury, anoxia due to severe maternal convulsions or mitral stenosis, and attempted abortions may adversely influence foetal development. Birth trauma and interference with circulation of the cord, especially where anoxia is severe and prolonged, cause intracranial damage with retardation. GENETIC CONSIDERATIONS

Genetics has contributed much to our understanding of congenital defects. The availability of chromosomal investigation provides the genetic counsellor with an expanding fund of knowledge that has increasingly practical applications.

142

THE NEW-BORN

Classification Congenital abnormalities may be divided into three broad groups. The first group includes those where the risk of recurrence is known and a predictable pattern can be estimated. Such diseases are cystic fibrosis of pancreas, muscular dystrophy, haemophilia, and phenylketonuria. The second group is heterogeneous, where a familial tendency exists but the pattern of inheritance is not known. Included in this group are harelip, cleft palate, diabetes, hydrocephalus, spina bifida with or without meningocoele, and many cases of mental retardation. The third group includes those diseases where a higher than normal frequency of recurrence exists but which do not conform to any established genetic pattern. Multiple congenital anomalies and tracheo-oesophageal fistula are examples. Environmental factors may be important. Risk of Recurrence In families where a child has been born with a defect the physician can give some information to the inquiring parent in regard to future children. In places where an experienced genetic counsellor is available it is usually more satisfactory to the patient and the physician for the counselling to be done by this expert. Fraser1 agrees with some authorities that a "risk rate," if considered alone and without reference to the exception, is false, but if it is given along with a statement of the concomitant inadequacy can serve as a useful guide. For example, if both parents appear clinically normal, the risk of a second child similarly afflicted with: (a) Anencephaly or meningocoele, is 5 per cent ( b) Hydrocephalus 1.6 per cent ( c) Cleft palate 2-5 per cent (d) Harelip 4-7 per cent 1F. C. Fraser, "Genetic counselling in some common paediatric diseases," Paediatric Clinics of North America (May, 1958), 475.

CONGENITAL DEFECTS

(e) Club foot

(/) Congenital dislocation of hip (g) Congenital heart disease (This varies considerably with type and family.) ( h) Cystic fibrosis of pancreas, and phenylketonuria ( mendelian recessive) With microcephaly, there is occasionally a mendelian recessive inheritance but it usually fits into the mixed group of diseases. (i) Idiopathic severe mental retardation (j) If a parent has idiopathic ( centrencephilic) epilepsy the risk of his child developing the same disability ( k) The incidence of diabetes mellitus in the general population is about With the risk of developing the disease before 80 years of age If both parents are non-diabetic the prospect of their having a second diabetic child is If one parent is diabetic the risk that they will produce a diabetic child is If both parents are diabetic the risk is nearly

CHROMOSOMAL

143

3 per cent 5 per cent 2 per cent 25 per cent

3 per cent

3 per cent 1 per cent 6 per cent 5 per cent

15 per cent 100 per cent

ABNORMALITIES

Background In the healthy human cell there are 46 chromosomes. In both the male and the female 22 pairs of chromosomes ( autosomes) are identical. Sex chromosomes differ; in the female there are two X chromosomes, in the male one X and one Y.

144

THE NEW-BORN

A specimen of skin, bone marrow, or blood can be cultured in vitro and special procedures used to stop cell division in the metaphase. The microphotograph of the chromosomes at this stage, arranged in a "paired order," is called a karyotype. The discovery by Barr2 of sex chromatin has been a further advance in genetics. This intranuclear body is present in more than 40 per cent of cells of the normal female but in less than 3 per cent of the cells of the male. Human autosomes are divided into 7 groups, characterized by length, constriction, and presence of satellites: Group A (numbers 1- 3) B (numbers 4- 5) C (numbers 6-12) D (numbers 13-15) E (numbers 16-18) F (numbers 19-20) G (numbers 21-22) Chromosomal defects which occur in the division of the cell result in structural changes and/or numerical variants. "Trisomy" is the name given to the occurrence of one extra autosome in a cell resulting in three instead of the normal pair. "Translocation" occurs when one or part of a chromosome becomes attached to another chromosome. Loss or deletion of a chromosome may occur. "Mosaicism" is the term used for a person having two or more cell types. Chromosomal studies reveal two or more karyotypes. CHROMOSOMAL SYNDROMES

(Mongolism) While there are other features, the characteristic appearance of the eyes typifies most mongoloid idiots. The eyes slant upward from the inner canthus, the palpebral fissure is small, and the epicanthic folds are prominent. The

DOWN'S SYNDROME

2 M. L. Barr, "Sex chromatin and phenotype in man,'' Science, 130 (1959), 679. ,

CONGENITAL DEFECTS

14S

iris of the blue-eyed mongoloid infant has Brushfield spots. t Often lens opacities and marked myopia are present. The head is small, brachycephalic with a fl.at occiput. A third fontanelle is often present. The bridge of the nose is fl.at; the mouth appears small; the thin lips are often "pursed" disclosing the tongue. There is usually a redundancy of skin at the back of the neck. The hands are broad. The little finger is short and curves toward the thumb. There is usually a Simian crease. t The muscles are hypotonic and the joint ligaments are lax. Often an umbilical hernia, congenital cardiac defect, and deficiency of cartilage in the pinnae are present. Duodenal atresia is present in a small percentage of cases. All patients with this syndrome are mentally retarded with an intelligence quotient of no more than 50. Mongolian idiots are prone to infections and often succumb to pneumonia. In a cardiac defect exists, the prognosis for longevity is worse. However, where formerly they often died in early childhood, antibiotics have prolonged their life expectancy. Mongolian idiots have a dermal pattern peculiar to the abnormality and this often aids in the diagnosis. The fine ridges on the palms and soles, which are composed of arches, whorls, and loops have a fairly constant variation in the normal person, and a different pattern in the mongoloid child. These patterns may be examined by a hand lens but usually the study is made of an inked imprint of hands and feet. The dermal pattern of a patient can be tabulated as a logarithmic expression which will assist in assessing the possibility of mongolism. Most paediatric teaching hospitals can perform dermatoglyphic testing and chromosomal analysis.

Chromosomal Studies In those cases where the clinical diagnosis is uncertain and the dermatoglyphic score is ambiguous, delineation of

146

THE NEW-BORN

the karyotype is essential. Most mongoloids have 4 7 chromosomes because of trisomy in autosome number 21. Occasionally a translocation occurs with a count of 46 chromosomes in a cell, but one chromosome is abnormal. Mosaicism occurs rarely. Incidence It has been estimated that a mother between 25 to 30 years of age has a 1 in 2,000 chance of producing a baby with Down's syndrome. If she produces one her risk of producing a second is 1 in 800. At age 45 a mother has a 1 in 40 chance of bearing such an affected infant; her risk of producing a second is 1 in 16. If a mother who has had an affected infant is young and anxious to have more children, a karyotype of the child should be obtained. If a translocation is discovered, the karyotypes of the mother and father should be investigated. The risk of having a second mongolian idiot is much greater if either parent, and especially the mother, has the translocation pattern. Information and Advice to Parents When a physician suspects this diagnosis, which should be at birth or within the first few days of life, a consultation is indicated. Parents, when first informed of the diagnosis, find it difficult to accept so grave a prognosis in an infant who appears normal to them. However, the initial mixed feelings of guilt, anger, and uncertainty will pass and the physician will be asked what should be done with the child. The doctor should point out the advantages and disadvantages of keeping the child in the home, and the advantages and disadvantages of institutional care. The age and emotional stability of the parents, the number and health of other children in the home, and the shortened life expectancy of a mongolian idiot, especially if congenital heart disease is present, are major considerations. After

CONGENITAL DEFECTS

147

careful consideration the parents should make the final decision. 0-TRISOMY SYNDROME

This syndrome, characterized by cleft lip and palate, microphthalmia, polydactylism, congenital cerebral, and cardiac defects, "rocker-bottom" feet and hypotonia is associated with a trisomy of group D ( 13-15) chromosomes. These babies usually die within the first year. E-TRISOMY SYNDROME

Prominent occiput, low set "field-mouse" ears, small mandible, inguinal hernia, cardiac and renal anomalies, index finger overlapping the third digit, "rocker-bottom" feet, failure to thrive, and retardation characterize this condition. There is a characteristic dermal pattern. A karyotype reveals a trisomy of group E ( 16-18) chromosomes. Most of these babies die before 1 year of age. TuRNER'S SYNDROME ( Gonadal

Dysgenesis) A female infant with loose skin folds at the sides of the neck and oedema of the dorsum of the hands or feet should be suspected to have this syndrome. Delayed linear growth becomes evident later. Coarctation of the aorta, renal, and skeletal anomalies also may be present. The sex chromatin pattern usually is negative. The usual karyotype is XO, with a chromosomal count of 45. Unless concomitant renal or cardiac defects require surgery, no treatment is necessary until puberty. LE CRI DU CHAT SYNDROME

This congenital anomaly, described by Lejeune et al. in 1963,3 is associated with a deletion of one short arm of one SJ. Lejeune et al., "Trois cas de deletion partielle du bras court d'un chromosome S," C.R. Academie Sci. Paris, 257 (1963), 3098-3102.

148

THE NEW-BORN

chromosome of Group V. The baby is mentally retarded and his cry resembles that of a cat. Other features are growth retardation, microcephaly, "moon-like" facies, hypertelorism, oblique palpebral fissures, epicanthic folds, low set ears, and abnormal palmar dermatoglyphs.

CONGENITAL ANOMALIES ASSOCIATED WITH SEXUAL DEFECTS A failure to examine the external genitalia of the newborn carefully and an incorrect sexual identification may lead not only to the embarrassment of the physician, but in some cases to serious physical and emotional disability for the child and family. Accurate assessment has moral and legal as well as medical implications. Cases of doubt are uncommon but require skill and judgment, which can be acquired only by special training and experience. A consultation by an expert in this field is mandatory if there is any doubt about the sex of the infant. BENIGN CONDITIONS

A female premature infant may have a clitoris which appears abnormally large. Labial adhesions occasionally give the appearance of a male with hypospadius and an empty scrotum. These adhesions may be confused also with an imperforate hymen. The identity of the "chubby" male infant with external genitalia buried in fat can appear ambiguous until the external genitalia are examined carefully. If a physician has any reservations about these "normal" variations, or if a baby has a recognizable defect, further investigation must be made.

CONGENITAL DEFECTS

149

CLASSIFICATION

A. Female pseudohermaphroditism 1. Congenital adrenal hyperplasia 2. Hormonal effects from the mother 3. Unexplained structural defects B. Male pseudohermaphroditism C. True hermaphroditism

Investigation 1. A review of the family history may give information to aid in the diagnosis of such conditions as congenital adrenal hyperplasia and feminizing testes. Hormones given to the mother during pregnancy should be noted. (See p. 137, "Medications for the Mother.") 2. A careful physical examination usually can distinguish between the female with an enlarged clitoris with fused labiae and the male with hypospadius with a poorly developed empty scrotum. If a gonad is palpable ( at times near the internal inguinal ring or even ectopically located in the groin) the sex is usually male. Combined abdominal and rectal palpation may reveal an adrenal or gonadal mass. 3. An examination for chromatin masses on buccal smear usually will indicate sex; however, chromosomal analysis of cultured cells is required in the more complicated cases. 4. Radiographic studies and endoscopy by a paediatric urologist often are required. Laparotomy and gonadal biopsy occasionally are indicated. A. FEMALE PSEUDOHERMAPHRODITISM

1. CONGENITAL ADRENAL HYPERPLASIA

Most cases of female pseudohermaphroditism and macrogenitasomia praecox are the result of congenital adrenal hyperplasia. Infants with female pseudohermaphroditism have ovaries but the external genitalia has masculine

150

THE NEW-BORN

features. The male infant with this disorder shows increased growth of genitalia but genital changes are not obvious in the neonatal period. Congenital adrenal hyperplasia is caused by an inherited metabolic block in the production of hydrocortisone by the adrenal. In the usual type, the defect is in hydroxylation at the twenty-first carbon position of the steroid nucleus. Lack of normal production of hydrocortisone causes increased adrenocorticotrophic hormone (ACTH), which in turn stimulates the adrenal cortex, resulting in excess production of many adrenal steroids, especially the androgens. The excess androgens cause virilization in the female and macrogenitasomia praecox in the male. In about a quarter of the cases there is a salt-losing adrenal insufficiency. Clinical Picture The genitalia of a female with this disorder may vary from slight clitoral enlargement to gross masculinization and appear as that of a male with undescended testes. In the male new-born the enlargement of the penis is usually unrecognized. In both male and female, however, androgen production continues after birth with progressive changes becoming more obvious with age. Those infants, with an association of salt wasting, have polyuria resulting in dehydration which may be intensified by vomiting and diarrhoea. Serum sodium and chloride levels are low, potassium is elevated and there is a metabolic alkalosis. Hypoglycaemia rarely occurs. In the male new-born infant, anorexia, loss of weight, and continually wet diapers suggest the diagnosis. Treatment (a) With the salt-losing type the replacement of fluid and salt losses is required, often as a life-saving measure. (i) After taking a sample of blood for estimation of

CONGENITAL DEFECTS

151

sodium, chloride, potassium, and blood sugar, an intravenous infusion of 5 % glucose in normal saline should be started immediately. (Seep. 80, "Intravenous Therapy.") (ii) DOCA (Desoxycorticosterone acetate) should be given intramuscularly daily, starting with 0.5 mg. and gradually increasing to 1-5 mg. (iii) If the baby is acutely ill, 25 mg. of hydrocortisone (Solucortef) should be given intravenously and a further 20 mg. added every 8 hours. If he is not acutely ill, the hydrocortisone should be withheld until the diagnosis is confirmed by finding elevated levels of 17-ketosteroidst and pregnantriolt in a 24-hour urine collecti'On. The 17hydroxycorticoidst may be low or normal but do not increase with stress or stimulation with exogeneous ACTH. (iv) Electrolyte estimation is required daily or oftener until the infant's condition is stabilized. ( v) After the baby has been hydrated, the intravenous infusion may be stopped and salt is added to the formula (usually 1-3 grams daily). (vi) When the infant is stabilized, the DOCA may be given as a pellet (125 mg.) implanted subcutaneously. Its effect usually lasts for about 9 months. Occasionally an additional pellet may be required. (vii) Hydrocortisone replacement must be given in a dose sufficient to suppress androgen production. This may vary from 10 to 25 mg./day. The dosage is determined by the amount necessary to reduce the 17-ketosteroid excretion to normal. In times of stress ( infection, accidents, operations) the dose of cortisone must be increased. Usually hospitalization is required if the baby begins to vomit. (viii) Because of the delineated variations and difficulties of management, a consultant should assist in the care of the infant during the acute phase. ( b) With the non-salt-losing type daily hydrocortisone or prednisone is given in sufficient dosage to suppress

152

THE NEW-BORN

androgen production. This is necessary for normal sexual development and growth. These infants also may show signs of adrenal insufficiency during times of stress. 2.

HORMONAL EFFECTS FROM THE MOTHER

Progesterone derivatives given to the mother during pregnancy may produce varying degrees of masculinization of the female new-born. The changes are maximum at birth and with general body growth tend to become less obvious. The use of these hormones is occasionally necessary in spite of this hazard, to prevent abortion. Similar effects have been associated with anabolic drugs ( androgen derivatives) and oestrogens. 3. UNEXPLAINED STRUCTURAL DEFECTS

Genetic errors in development may result in a female with various degrees of masculinization. Summary of Female Pseudohermaphroditism In all cases proper sex should be established by buccal smear and/or chromosomal studies. The adrenogenital syndrome is differentiated from other causes by the raised 17-ketosteroids and pregnantriol, the progression of virilization, and salt wasting ( if present). A detailed history of medications given to the mother is essential. Structural defects are often complicated and require radiographic studies and direct examination of the urogenital structures. Laparotomy may be necessary for diagnosis and treatment. Occasionally clitorectomy and/or plastic repair of the external genitalia may be required in treatment of female pseudohermaphroditism.

B. MALEPSEUDOHERMAPIIRODfflSM These babies have testes but the external genitalia simulate the female. The defect usually is due to a genetic aberration but may be related to a metabolic disturbance of testosterone production such as occurs in feminizing testis.

CONGENITAL DEFECTS

153

Investigation should include pyelography, endoscopy, chromosomal analysis, and often biopsy. Plastic repair of the external genitalia usually extends over many years. C. TRUE HERMAPHRODITISM

These babies with mixed features of male and female have both ovary and testis. Chromosomal analysis will reveal a mosaicism. In most cases, treatment is directed towards making the infant a female after a complete investigation as outlined in section B above.

OTHER ABNORMALITIES MEATAL STENOSIS

This is recognized by a "fine wire" urinary stream. The infant strains when voiding. A meatotomy is indicated when the diagnosis is made. LABIAL ADHESIONS

These should be separated when diagnosed by a blunt dissecting probe and vaseline should be applied twice a day to the raw surfaces to prevent reforming. In resistant cases, estrogen cream is more effective than vaseline. HYPOSPADIUS

If the displaced urethral meatus is located close to the glans, and the orifice is adequate, no treatment is required. Other variants, and especially when chordee is present, should be referred to a surgeon. The baby with hypospadius should not be circumcised. If the defect is marked, an intravenous pyelogram and chromosomal analysis are indicated. CRYPTORCHIDISM

If one testicle is undescended but the other is in the scrotum no treatment is required in the neonatal period.

154

THE NEW-BORN

With episodes of pain and vomiting the area should be examined carefully for evidence of torsion or hernia. In bilateral cryptorchidism chromosomal studies should be done. Definite surgical treatment is not indicated in the neonatal period unless the condition is associated with an inguinal hernia. METABOLIC

DEFECTS

AND

PREVENTABLE MENTAL RETARDATION

Retardation, caused by phenylketonuria, galactosaemia, cretinism, and hypoglycaemia, can be prevented by specific treatment. With any unrecognized congenital defect, urinary aminoacid and sugar chromatograms will provide information of metabolic involvement if abnormalities of the amino-acids or carbohydrates are present. PHENYLKETONURIA

This disease, which occurs in about 1 in 10,000 babies, is caused by an enzymal deficiency whereby phenylalanine, an aminoacid found in milk and other proteins, cannot be metabolized. The high blood levels of phenylalanine are cleared partially by excretion of phenylketones in the urine. If it is not feasible to do the Guthrie testt on all babies in the new-born nursery, the urine should be tested for phenylketones with ferric chloridet or test tape (Phenistix) t when the baby is 3 weeks of age, and again at 6 weeks of age. The disease should be suspected in any infant with a familial history or in any "fair-haired, blue-eyed" baby with a maturational lag. Eczema frequently is present and the baby has a "mousy" odour. The diagnosis must be confirmed by a test of blood level. The normal blood level of

CONGENITAL DEFECTS

155

phenylalanine is 0.7-4mg./100ml.; most patients with the disease have a level of over 20mg. / 100ml. A milk substitute such as Lofenalac will reduce the blood level toward normal. Monthly blood tests should be taken so that the blood level may be maintained under 7mg. / 100ml. GALACTOSAEMIA In galactosaemia there is an enzymal lack whereby the baby cannot convert galactose to glucose. Unless galactose ( contained in milk) is eliminated from the diet, vomiting, diarrhoea, cataracts, jaundice, mental retardation, and hepatosplenomegaly develop. The baby fails to thrive. (For test and management see p. 123, "Jaundice.")

CRETINISM Although the cretin usually cannot be recognized in the new-born nursery, the diagnosis should be suspected in cases of prolonged physiological jaundice. Within a few weeks of age constipation, inactivity, and a hoarse cry may be noted in conjunction with pallor, slight icterus, coarse features, a flat nasal bridge, a big tongue, and an umbilical hernia. The radiograms of knee often show an absence of the lower femoral epiphysis ( normally present at birth). There may be cardiac enlargement. The diagnosis is substantiated if the protein bound iodine is less than 3.5 µ,g per cent. Treatment should be started immediately after taking a sample of blood for estimation of the protein bound iodine. Thyroid gr. ¼ is given daily per os and increased in 2 weeks to gr. ¼ daily. Dosage should be increased at 2-3 week intervals to the point of toleration. Hyperactivity and diarrhoea are signs of overdosage. HYPOGLYCAEMIA For description and management see p. 132, "Twitching and Convulsions."

156

THE NEW-BORN

INDICATIONS FOR SURGERY IMMEDIATE SURGERY

1. Omphalocele. 2. Tracheo-oesophageal fistula, diagnosed by catheter. 3. Intestinal obstruction ( atresia, band, volvulus, Hirschsprung's, meconium ileus, imperforate anus). (See p. 48, "Dysphagia, Vomiting, Diarrhoea.") 4. Choanal atresia, diagnosed by catheter. 5. Diaphragmatic hernia, diagnosed by radiogram of the chest. 6. Severe respiratory tract obstruction which may be caused by a goiter or abductor paralysis of vocal cords. A tracheotomy is required. 7. Intractable failure in certain cardiac lesions or in arterio-venous aneurysm. (See p. 100, "Cyanosis.") 8. Vesicular obstruction due to posterior urethral valves. (Seep. 68, "The Ill Baby.") 9. Imperforate anus. 10. Reparable meningocele devoid of skin. Imper/orate Anus In a female infant with imperforate anus the frequent association of a recto-vaginal fistula often obscures the diagnosis. Since a urethral or vesicular communication may exist also, a urological investigation is indicated. Meningocele If devoid of skin, the area should be covered with sterile vaseline gauze and "saran wrap," and protected by a "dough-nut" dressing. The neurosurgeon must decide promptly whether an operation is feasible before the lesion becomes infected. The size of the lesion and the neurological deficit are the chief considerations when deciding if surgery is necessary.

CONGENITAL DEFECTS

157

If the lesion is large, devoid of skin and "leaking" spinal fluid ( and it is unlikely that a viable skin flap is obtainable), if there is no anal tone, if there is no withdrawal to "pin prick" of the feet and if the legs are flaccid, surgery is not indicated. Less complex defects require a neurosurgical consultation. ELECTIVE TREATMENT

Hydrocephalus A congenital defect of the aqueduct (obstructive) or an abnormality of the balance between production and absorption of cerebro-spinal fluid ( communicating) are the common causes of the hydrocephalus. Other causes are congenital toxoplasmosis, cytomegalic inclusion disease, and haemorrhage, which may be associated with radiographic calcification. Meningitis may cause a self-limited hydrocephalus. In either obvious or doubtful cases, a neurosurgical consultation is indicated. In some cases of obstruction a by-pass operation using the Pudenz valve is indicated. Craniostenosis An abnormally shaped head associated with an elevated ridge along the suture line suggests craniostenosis. The defect varies from the minimal synostosis of a single small suture which results in a slight cranial asymmetry to the gross deformity of Cruzon's disease with synostosis of all the cranial sutures. Radiograms will confirm the diagnosis. Early neurosurgery (linear craniectomy) which will provide for a more normal growth of skull and prevent retardation is indicated. Congenital Dislocation of Hip: Forefoot Adduction For the diagnosis see p. 12, "Care of the Full Term Baby." These conditions, which require plasters, should be referred to an orthopaedic surgeon.

158

THE NEW-BORN

External Femoral Torsion This condition is suspected by the increased degree of passive external rotation of the femur compared with the normal. The baby should be examined at 3 months of age by an orthopaedic surgeon. Internal Tibial Torsion The condition is suspected by pseudo-adduction of the feet without intrinsic forefoot adduction. If the baby is placed supine in the anatomical position, the patella is found to be lateral to its position in the normal infant. The baby should be examined at 3 months of age by an orthopaedic surgeon. Phocomelia and Congenital Amputation These conditions require an orthopaedic assessment for future prosthesis. Polydactylism This may be part of a generalized condition such as Laurence-Moon-Biedl syndrome (dwarfism, retardation, retinitis pigmentosa), or Ellis-Van Creveld syndrome ( chondro-ectodermal dysplasia) . Before surgery, a medical investigation may be indicated.

Harelip It is usually repaired by the plastic surgeon at 3-4 months of age when baby weighs 6 kg. (13.5 lbs.). Cleft Palate It is usually repaired at about 18 months of age. Haemangioma The small flat or elevated haemangioma which is not increasing in size usually disappears spontaneously and needs

CONGENITAL DEFECTS

159

no treatment. Resolution of larger lesions that are increasing in size may be expedited by the application of carbon dioxide snow. A large unsightly haemangioma, especially if located on the face, should be referred early to a plastic surgeon.

Pigmented Naevus If large and unsightly or if black and hairy, pigmented naevi should be referred early to a plastic surgeon. CONCLUSION

The diagnosis of many acute and critical congenital defects is facilitated often by their gross signs ( vomiting, distension, or respiratory distress). The baby's survival depends upon recognition and surgery. There is another group of less obvious congenital abnormalities, where survival is not a problem; here the chief concern is prevention of intellectual and physical disability. TERMINOLOGY Brushfield Spots In 1924 Dr. Thomas Brushfield described a circle of tiny white pin-point specks, linearly arranged, near the outer border of the iris of blue-eyed mongoloid infants. The lack of stromal tissue accounts for the "speckling." Although seen occasionally in the normal infant, these specks are usually present in the mongolian idiot with blue eyes.

Pudenz Valve This apparatus is used frequently in neurosurgery for relief of obstructive hydrocephalus. The valve connects the lateral ventricle with the right atrium via the jugular vein. Incorporated in the valve is a digital pump which may be used to keep the

160

THE NEW-BORN

bypass clear. The valve is set so that at 80 mm. of water the cerebrospinal fluid is released from the lateral ventricle into the right atrium.

Simian Crease This is a transverse line that extends completely across the palm. The finding is unusual in the normal infant but common in the mongolian idiot. Ferric Chloride Test Three to four drops of 10% ferric chloride solution on the wet diaper of a baby with phenylketonuria will produce a deep bluish-green colour. The test may not be positive until the baby is 3-6 weeks old. Guthrie Test A blood sample from a new-born infant with phenylketonuria taken after the fourth or fifth day will inhibit the growth of bacillus subtilis. Dr. R. Guthrie has evolved a fairly simple and reliable method for mass testing of all babies for this defect in the new-born nursery. In those hospitals where this test is not performed the physician should test the wet diaper of the baby at 3 weeks of age and again at 6 weeks of age by the ferric chloride or Phenistix test. Phenistix Test This commercially available test stick is moistened in urine by squeezing the wet diaper. If the test is positive, a grayishblack colour develops. The test may not be positive until the baby is 3-6 weeks old. 17-Ketosteroids The urinary 17-ketosteroids are derived from androgen production, and to an extent reflect the level of circulating androgens. The normal 24 hour urinary excretion by the end of the second week is less than 0.5 mg. Increased excretion is associated with virilization in the female and macrogenitasomia praecox in the male.

CONGENITAL DEFECTS

161

17-Hydroxycorticoids The 17-hydroxycorticoids reflect the activity of the adrenal cortex in the production of hydrocortisone ( compound F). This metabolite can be measured in the blood or urine. Normal urinary excretion from birth to two years is 2-4 mg. per 24 hours. Increased production is associated with Cushing's syndrome; decreased production with adrenal insufficiency. Pregnantriol This intermediate metabolite of the adrenal corticoids is excreted in the urine. In the metabolic block in production of hydrocortisone in the common type of adrenogenital syndrome, the excretion of pregnantriol is increased. Normal urinary excretion until six years of age varies from 0 to 2 mg. per 24 hours with an average of 0.02 mg.

15. Procedures

RESUSCITATION IN THE NEW-BORN

Immediately after delivery the baby should be held tightly by his ankles, with the head dependent, thus allowing mucus to drain from the mouth as the neck is stripped to free the throat and mouth from amniotic fluid. The cord is clamped and cut sufficiently long to allow for replacement transfusion if this is found to be necessary. If the baby does not cry immediately, he is turned on his side and his airway cleared with a mouth suction apparatus. An infant oral airway is inserted to keep the tongue forward and the baby is stimulated gently by rubbing his back or flicking the soles of his feet. If, after one minute, the baby is not breathing, a laryngoscopic examination should be done, gently passing the tip of a rubber catheter into the pharynx. Clearing the mucus in the region of the vocal cords often initiates breathing. Too vigorous "jamming" of the catheter into the baby's larynx, at times, may produce severe laryngeal spasm with marked cyanosis. However, if the baby has aspirated meconium or considerable sticky mucus, a catheter attached to a mechanical aspirator may be needed. If this does not stimulate the baby to breathe and cough, then a Portex tube (#12 French catheter, which is about 3.5 ml. in diameter) may be passed between the vocal cords under direct laryngoscopic examination and left in the trachea. A gentle puff of air into the tube, which can be

PROCEDURES

163

noted to inflate the chest slightly, often will initiate breathing. This may be repeated at the rate of about 30 puffs per minute. If no chest movement occurs, gentle suction through the tube should be done using a #8 French aspirator. This suction may remove some mucus from the trachea and another gentle puff of air usually will initiate spontaneous breathing. If there is still no response, the baby should be ventilated by mouth to mouth respiration or, better, by means of a small mask with a hand ventilating rubber bag to which is attached a source of oxygen flowing at a rate of 6 litres per minute. If the baby makes no intrinsic effort to breathe, then a mechanical respirator, attached to the end of the tracheal tube, should be used until the baby is breathing spontaneously. The nurse puts the stethoscope over the heart and listens for the heart sounds, tapping with a finger to correspond to the heart rate. If no heart sounds are heard, or if the heart sounds which were heard once cease, then external cardiac massage must be given. (See below, "Cardiac Massage.") CARDIAC MASSAGE

With the doctor standing at the baby's head and the baby cradled in his hands, with the fingers at the back and the thumbs over the upper third of sternum, a thumb-squeezing manoeuvre, at the rate of 60 per minute, is done. Pressure must not be exerted near the xiphoid process, since compression of lower sternum results in injury to or rupture of the liver. 1 Caffeine and sodium benzoate (seep. 176, "Dosages of Drugs") may be given intramuscularly but its value is questionable. tM. M. Thaler and G. H. C. Stobie, New England Journal of Medicine 269 (Sept. 19, 1963), 606.

164

THE NEW-BORN

Artificial ventilation (20 per minute) should be performed at the same time as the massage, ventilating by mouth to mouth respiration until the infant is intubated. The tube then is attached to a mechanical respirator such as the "Bird." If the onset of the cardiac arrest is sudden the response often is immediate. If the arrest is prolonged and the response to treatment is not immediate, 3 ml. of a 1 in 20 dilution of adrenalin 1 : 1000 is given into the heart. Since prolonged arrest is associated with myocardial cellular acidosis, it may be necessary to inject into the left ventricle 15 ml. of 5% sodium bicarbonate followed by 5 ml. of 10% calcium gluconate. REPLACEMENT TRANSFUSION

This procedure should be performed by an experienced physician and only in conjunction with adequate laboratory facilities. NOTE.

Personnel Requirement One physician acts as medical supervisor of the general well-being of the baby, that is, he records the exchange by increments, takes and records the heart rate, watches respirations, and ensures that the baby does not choke on its mucus or vomitus. A second physician performs the exchange transfusion. A nurse assists the doctor performing the transfusion and informs him if the baby becomes hypothermic. Pre- and Postoperative Medication Before the replacement is started, the baby is given intramuscularly the following medications: 1. Two mg. of vitamin K1 oxide, if this has not been given after delivery ( 1 mg. if the baby is premature).

PROCEDURES

165

2. 200,000 units of aqueous penicillin G combined with 60 mg. of streptomycin sulphate. Following the replacement transfusion 100,000 units of aqueous penicillin G with 30 mg. of streptomycin is given intramuscularly every 12 hours for the next 2 days (half the dose is given to premature babies). Blood for Replacement Transfusion

Fresh, citrated whole blood, semi-packed by removing 100 ml. of plasma from 500 ml. of whole blood after the cells have settled by gravity, is used allowing 150 ml./kg. (70 ml./lb.) body weight. Fresh heparinized blood is physiologically more satisfactory but is not routinely available. Because of a greater concentration of antibodies in the mother's blood it is best to use the mother's serum for crossmatching with the donor's blood if possible. In haemolytic disease due to ABO incompatibility, the blood used should be the same type as the mother's or a type compatible with the mother's ABO and Rhesus groupings. In practice neutralized group 0, Rhesus-compatible blood is used. In rare cases such as incompatibility where the baby is group AB and mother group A, neutralized group A blood could be given instead of group 0. In haemolytic disease due to Rhesus incompatibility the blood used in the replacement transfusion is Rhesus negative, is compatible with the mother's blood, and is usually of the same ABO group as the baby. However if the haemolytic disease is due to a combination of both Rhesus and ABO incompatibilities, neutralized group O Rhesus-negative blood is used. In the rare instances of sensitization such as to c or other factors, the blood used must be compatible with the mother's blood. If a sample of maternal blood is not avail-

166

THE NEW-BORN

able, the blood compatibility is determined by the antibodies present in the baby's serum, but this method is less satisfactory because the baby has lower concentration of antibodies in the serum than the mother. In all situations if a negative indirect Coombs' test is obtained on the donor's cells after incubating them in the mother's serum, this blood may be given to the baby. If a severely affected Rhesus incompatibility is suspected, the donor blood may be cross-matched with the mother's serum prior to delivery so that blood for the exchange will be available immediately. However when a baby is born unexpectedly and is severely affected neutralized group 0 Rhesus negative blood may be used immediately. Repeated replacements or subsequent transfusion will necessitate the use of this same blood type used in the original replacement regardless of the baby's group until the baby is three months of age.

Equipment Most hospitals in which replacement transfusions are performed have their own established trays with doctors' gowns, draping sheets, towels, forceps, syringes, three-way valves, polyethylene tubing, catgut with needle, and manometer. However, disposable units now are available at a very reasonable cost and probably are preferable. Procedure After a doctor and nurse have checked the baby's identity, the baby is placed on a restraining board (similar to a circumcision board), with the arms and legs extended from the body and held by a towel. The baby's stomach is aspirated of its contents. An electric blanket is used to keep the baby warm during the procedure; the abdomen is left bare. The stethoscope is fastened to the chest over the heart by adhesive tape.

PROCEDURES

167

The assistant removes the cord tie or clamp and the skin is prepared by washing with Phisohex, drying, and applying tincture of Zepherin chloride. The excess cord stump is excised. The bare umbilical area is draped using sterile towels and a laparotomy sheet. The assistant, seated beside the patient, records the amount of blood given and the amount of blood taken out; he also records the heart rate which is monitored by listening with the stethoscope. The nurse and the physician check the blood to see that it is correct type and group allotted to the patient. The umbilical vein is located; if it is thrombosed, the clot is removed and the special polyethylene tubing is inserted into the umbilical vein to a length sufficient to put the tip just within the inferior vena cava. Five ml. of blood are removed with a dry syringe and sent for van den Bergh test. The venous pressure is measured and should be less than 6 cm. of water. If it is in the range of 10 cm., the baby is in danger of heart failure and a gradual deficit should be allowed (removing more blood than replacing) until the pressure returns to physiological limits. The system is filled with heparinized normal saline to remove the air in the tubing, which then is filled with blood. The operator takes 20 ml. of blood from the baby slowly and discards this to ensure not overloading the baby's circulatory system. After pausing for a moment, 20 ml. of donor blood is injected slowly into the baby's circulation. The 20 ml. exchange continues until the allotted blood is used, the procedure taking usually 1½-2 hours. The assistant must record carefully each 20 ml. taken out and each 20 ml. increment given to the baby. At the end of every 100 ml. the syringes are disconnected and 5 ml. of 2 % calcium gluconate is given slowly to counteract the citrate used in the stored blood. The baby's cardiac rate, colour, and cry must be observed

168

THE NEW-BORN

carefully since the calcium sometimes causes the baby's heart rate to slow unduly. To prevent clotting, the syringes are flushed after every 100 ml. with heparinized saline (2½ ml. of heparin added to 250 ml. of normal saline). Venous pressures should be recorded at the start, halfway through and at the end of the procedure. At the termination of the procedure a purse-string suture is inserted into the umbilicus about the polyethylene tubing, which is removed, and the suture drawn tightly. The umbilicus is painted with tincture of zepherin and allowed to dry. A dry dressing is secured over the operative site by means of elastoplast. The baby is placed in an incubator. The baby should not be given a prolonged exposure. The operative time should not exceed 2 hours. If difficulties are encountered, it is advisable to stop the transfusion and repeat it at a later date. There is danger of chilling the baby but this may be reduced by having the polyethylene tubing used in the procedure pass through a warm bath, kept from 35.5-36.5°C. (96-98°F.), before the blood enters the baby's circulatory system. Exchange for small or shocked babies should be done in 10 ml. increments rather than 20 ml. On certain occasions there are indications for doing a multiple stage replacement transfusion rather than replacing the blood at a total volume at one time. The operator replaces 100-150 ml., waits for a period of 45 minutes to allow diffusion of the bilirubin from the tissues back into the circulation, and then does another 150 ml. exchange. Finally, a third time, after a similar wait, another 150 ml. is exchanged. Such a method may reduce the frequency of replacement transfusion in a highly sensitized baby and allow time for the baby to re-establish its circulatory equilibrium before the procedure is completed. Where the bilirubin level remains high after replacement

PROCEDURES

169

transfusion, album.in priming may be indicated in some cases. 2 In general, the compatibility of blood to be useq in replacement transfusions for babies suffering from haemolytic disease, is established best by the indirect Coombs' method. In the rare cases of Kell, Duffy, etc., incompatibility, this is the only adequate method of cross-matching. For these reasons 10 ml. of whole clotted mother's blood always should be sent if the baby is transferred for replacement. CIRCUMCISION

Where ritual is not a factor, the wishes of the parents rather than necessity has accounted for the great increase in circumcisions performed in new-born nurseries. While it is easier for the mother to bathe the penis of her infant if he is circumcised, it is also true that during the teething period ulceration of the urethral orifice and glans is much more common. However, if the foreskin is long, tight and very adherent, especially to the external urethral orifice, a circumcision is indicated. There is less danger of bleeding if the circumcision is performed on the fourth or fifth day. After a dorsal slit is made, the foreskin should be separated completely from the glans by a blunt dissecting probe. The use of a 1.3 cm. circumcision clamp ( such as the Gomco) facilitates the procedure. Total removal of the foreskin is to be avoided as it is best to leave the posterior third of the glans covered. After the remaining foreskin proximal to ridge of the glans is retracted, a dry gauze bandage is wound carefully around the penis at the site of the circumcision leaving the tip of 2 G. B. Dell and S. Cohen, "Albumin priming in the management of hyperbilirubinaemia by exchange transfusion," Amer. Dis. Child. 102 (1961), 699-700.

170

THE NEW-BORN

the glans free. Twenty hours later the dry dressing is removed and vaseline gauze is applied daily for 4 days. The premature baby is circumcised usually after his weight exceeds 5 lb. using a 1.1 cm. circumcision Gomco clamp. Postoperative bleeding, on most occasions, can be controlled by clamping any local bleeding point or, if there is general oozing, by wrapping the penis very carefully using a 1-inch gauze bandage. If bleeding continues, the infant should be given 2 mg. of vitamin K1 oxide intramuscularly after obtaining a sample of blood to determine a possible coagulation defect. If severe blood loss has occurred or if oozing is not controlled readily by local measures a transfusion of fresh whole compatible blood ( 20 ml. /kg.) should be given without delay. INTRAVENOUS TECHNIQUES

When administering fluid intravenously to infants, especially in the new-born period, the use of the Pedatrol and the "Micro Drip" type of apparatus is essential to avoid flooding accidents and to keep the rate accurate. Stab Intravenous There are numerous commercially available sets, packaged with a swedged needle ( #23), adapter and polyethylene tubing. The superficial veins of the scalp, dorsum of feet or hands, may be used. After the area is cleansed with hexachlorophene and rubbing alcohol applied, the needle is inserted through skin, almost parallel to its surface, and then "angled" into the vein. When the vein is pierced, blood flows back into the plastic tubing. The needle is held in place by narrow strips of adhesive tape attached to skin and

PROCEDURES

171

is then connected to the polyethylene tube from the Pedatrol.

Venous Cut-Down When the infant is critically ill and it is essential for intravenous therapy to continue without interruption, or where medication (e.g. calcium gluconate) administered may cause necrosis if it tracks subcutaneously, a "cutdown" is advisable. The long saphenous vein at the ankle is usually used. The operator should scrub, gown, and wear sterile gloves. After the leg is restrained and the skin is sterilized, the area should be exposed with sterile drapes. The site of the incision is infiltrated with 1 % procaine. A transverse incision about 0.5 cm. long is made through the skin over the anterosuperior border of the medial malleolus. The vein is exposed by blunt dissection and mobilized. A small incision is made in the vein, into which a finely tapered polyethylene tubing with adapter is threaded and tied in place. The skin is sutured and a dry dressing is applied. The adapter is connected to the polyethylene tubing of the Pedatrol. INTERSTITIAL INFUSION

Using sterile technique, the skin over the back or front of the thorax is cleansed with 3 % hexachlorophene and water followed by an application of rubbing alcohol. About 1 inch below the angle of the scapula and 1 inch lateral to the spine, a #20 short-bevelled needle is inserted subcutaneously. A second needle is placed similarly in relation to the other scapula. These needles are connected by a Y polyethylene tubing to a Pedatrol, filled with either % 5% glucose, ¾ normal saline or Ringer's lactate solution. The total volume given is normally about 20 ml./kg. or the volume that is absorbed in 20 minutes. The front of the

172

THE NEW-BORN

chest may be used, with a needle inserted subcutaneously about 1 inch below each nipple. Interstitial infusions should not be given where the skin is infected, or near operative sites or to a baby with respiratory distress. Usually if more than two interstitial infusions a day are required to maintain hydration, the infant requires a continuous intravenous infusion. LUMBAR PUNCTURE

Usually when performing a lumbar puncture the nurse holds the patient on the table on his side with neck and thighs flexed, but with the neonate it is easier, sometimes, to steady the baby "flexed" in a sitting position. Using sterile technique and sterilizing the skin with tincture of iodine followed by alcohol, the interspinous space, in line with height of the iliac crests, is determined. A short (2½") lumbar puncture needle (#22 or #20) is advanced in exactly the midline, through the skin, in the direction of the umbilicus. After the needle is pushed just through the resistant intervertebral membrane and dura, the stilette is withdrawn and spinal fluid will appear. If no fluid appears, the needle should be rotated gently. Often fluid does not appear for a few seconds. If it still does not appear then, the stilette is replaced and the needle is advanced another 1-2 millimeters (without altering direction) and the stilette again withdrawn. If the procedure still is unsuccessful, another attempt can be made through the intervertebral space immediately above or below. A traumatic tap occasionally results in spinal fluid mixed with blood. An accurate count of the white blood cells in the spinal fluid will then be difficult, but a culture should be made if meningitis is suspected. SUBDURAL TAP

The scalp is shaved in front of the anterior line of the

PROCEDURES

173

ears. With the baby restrained and lying supine on the table, the nurse immobilizes the head. Using sterile technique and after sterilizing the skin with tincture of iodine followed by alcohol, the skin over the lateral angle of the anterior fontanelle is infiltrated with 1 % procaine. A 2½ inch short-levelled lumbar puncture needle (#20) held at right angles at a point ¼ inch lateral to the lateral angle is pushed through the skin and just through the dura. The line of direction of the needle should not be changed after it goes through the dura. The stilette is removed. If a subdural haematoma is present bloody or xanthochromic fluid will appear. If clear fluid is obtained (up to 1 ml. normal) a sample should be sent for protein estimation. PROCURING BLOOD FOR CULTURE OR ELECTROLYTES

Obtaining a sample of blood by venous puncture in a new-born is difficult and sometimes dangerous. Micro-techniques using blood obtained from finger or heel prick should be used whenever possible. Blood for pH, carbon dioxide content, potassium, blood culture and certain other tests requires a venous puncture. Occasionally superficial veins of scalp or limbs are adequate. Usually a larger vein such as the external jugular or the femoral vein is used. To obtain a sample from the external jugular vein, the baby is wrapped snugly in a blanket with his arms at his sides and placed on his back. With his shoulders even with the edge of the table, the head is rotated 90 degrees and supported in a dependent position. A sharp long-bevelled needle facilitates the venipuncture of the distended vein. To obtain blood from the femoral vein, the skin of the groin is sterilized with alcohol. The pulsation of the femoral artery is noted at a point about 2 cm. below the inguinal ligament with the thigh slightly abducted and externally rotated. A sterile, sharp, short-bevelled needle, with syringe

174

THE NEW-BORN

attached, is inserted through the skin just medial to the pulsation into the vein. The plunger is slowly withdrawn as the sample is obtained. The needle is withdrawn and a dry "pressure bandage" is applied for a few moments. COLLECTING URINE FOR BACTERIAL COUNT, MICROSCOPIC EXAMINATION AND CULTURE

Routine catheterization of infants should be avoided. Much useful information may be obtained from a "clean specimen." After the external genitalia have been cleansed with liquid green soap and water and rinsed with normal saline, a "plastic attachable urinal" is fitted over the external genitalia. As soon as the infant voids, the specimen is taken immediately to the laboratory for examination and culturing. Although frequently contaminants are cultured, a significant growth of pathogenic organisms is also obtained. NORMAL

VALUES

NORMAL SERUM LEVELS

Ph CO2 content Sodium Chloride Potassium Calcium

7.35-7.45 20-25 mM. per litre 135-145 meq. per litre 98-108 meq. per litre 4-4.5 meq. per litre 9-11 mg./100 ml. (4.5-5.5 meq. per litre) Phosphorus (inorganic) 4.5-6.7 mg./100 ml.

NORMAL BLOOD V ALOES

Haemoglobin Normal value is 17-19 gm./100 ml. at birth, gradually decreasing to about 11 gm. at 3 months of age.

PROCEDURES

175

White Blood Cell Count Normal value is 15,000 to 40,000/cu.mm. at birth, and about 25,000 at 24 hours of age, with relative neutrophilia. After the first week it is usually below 13,500 with relative lymphocytosis. Often an overwhelming infection in the neonatal period is associated with little or no leukocytosis and, at times, with a leukopoenia. Toxic granulation of the white blood cells, noted on smear, often is a better sign of infection. Platelet Count At birth it is 300,000 to 400,000 per cu.mm. From two weeks until three months it is 200,000 to 400,000 per cu.mm. Prothrombin time is 11 to 15 seconds.

16. Dosages of Drugs

=

Weight conversion

1 Kg.

P.O.= per os I.V. = intravenous

Sbc. = subcutaneous I.M. = intramuscular

ATROPINE SULPHATE

2.2 lbs.

P.O.

0.01 mg./kg. q.6.h. Sbc. Pre-anaesthetic 1-3 kg.-0.1 mg. 3-5 kg.-0.15 mg. 5-10 kg.-0.2 mg. CAFFEINE WITH SODIUM BENZOATE CHLORAL HYDRA TE

J.M. 8 mg./kg. dose P.O., rectal

20 mg./kg. q.8.h. CORAMINE ( NIKETHAMIDE)

I.M., J.V. 25% solo.

0.1 ml./kg./dose DEMEROL (MEPERIDINE)

P.O.,J.M. 1.5 mg./kg. q.6.h.

DESOXYCOR TICOSTERONE ACETATE (D.O.C.A.)

J.M. 0.5-5 mg./24 hours Sbc.-long acting Pellet125 mg., 1-2 q.9.mos. approx.

DOSAGES OF DRUGS

177

P.O.,I.M.

DIGOXIN

0.06 mg./kg. is the total digitalizing dose until 2 years of age. One-half total dose is given stat, then ¼ total dose in 4-6 hours, then ¼ total dose in further 8-12 hours. Maintenance dose is 115th total digitalizing dose given in 2 divided doses daily DILANTIN

(DIPHENYLHYDAN-

TOIN SODIUM)

P.O., I.M.

1-3 mg./kg. q.8.h.

DIURIL ( CHLOROTHIAZIDE)

P.O.

10 mg./kg. q.6.h. P.O., Sbc.

EPHEDRINE

0.5 mg./kg. q.4.h. Sbc. 1: 1000 aq. soln.

EPINEPHRINE HCL (ADRENALIN CHLORIDE)

1.0% aq. soln. for topical application; prolonged use over 10 days may cause irritation of mucous membrane

GENTIAN VIOLET

HYDROCOR TISONE TIONS

( also

SONE)

0.1 ml./dose

PREPARA-

see

PREDNI-

P.O.

( 1) physiological replacement 5-10 mg./day in divided doses ( 2) congenital adrenal hyperplasia

178

THE NEW-BORN

10-25 mg./ day in divided doses

HYDROCOR TISONE PREPARATIONS

(continued)

( 3) therapeutic dose P.O., J.M., or I.V. 0.5-2 mg./kg. q.6.h. (larger doses are occasionally required) In severe illness 15-25 mg. of Solucortef may be required as an initial dose

INTERTRIGO LOTION

Burow's solution

15 ml.

Zinc oxide

30gm.

Talc

30gm.

Glycerin

24ml.

Lime water

ad 120 ml.

Apply to skin folds q.i.d. IRON

Normal requirement is 6-16 mg. elemental iron daily Anaemia: 100-200 mg. elemental iron/24 hours P.O.: Fer-In-Sol or Mol-Iron 125 mg. FeSO4 1 ml. ( about ½ of this is elemental iron)

=

J.M.: lmferon 1 ampoule 2 ml. 100mg. Fe

=

=

If baby is 3.5 kg.: 0.5 ml. 3.5-10 kg.: 1 ml. MAGNESIUM SULPHATE

I.M.: 50% solo. 0.2 ml./kg. q.6.h.

DOSAGES OF DRUGS METHADONE HYDROCHLORIDE

P.O., sbc. 0.15 mg./kg. q.4.h.

METHYLENE BLUE

I.V.

179

0.1-0.2 ml./kg. of 1 % soln. MERCUHYDRIN

I.M.

Below 3 kg.: 0.125 ml. 3-7 kg.: 0.25 ml. MONODRAL ELIXIR

P.O.

0.125 mg./kg. q.6.h. MORPHINE SULPHATE

Sbc.

0.1-0.2 mg./kg./dose NALLINE {NALORPHINE HCL)

I.V. or J.M.

0.1 mg./kg./dose May be repeated in 15 minutes NEOSTIGMINE METHYL SULPHATE PANCREATIC ENZYMES

I.M.

0.04 mg./kg.-Max. 0.5 mg. Intrazyme, Viokase, Cotazym Usual dose in the neonate: 1-3 capsules per feeding

PARALDEHYDE

(soluble tablet containing phenobarbital and homatropine)

PHENAT

J.M. 0.2 ml./kg. up to 10 ml. P.O.

All tablets contain ¼oo gr. of homatropine methyl bromide. The strength of the phenobarbital varies gr. ¼; gr. ¼; gr. ¼. Usual dose gr. ¼ a.c. in the neonate.

180

THE NEW-BORN

PHENERGAN

P.O.

0.5 mg./kg. q.6.h. PHENOBARBITAL

P.O.

0.5-2 mg./kg. q.6.h. PREDNISONE

2 mg./kg./ day in 4 or more divided doses

PRO-BANTHINE

P.O.

0.4 mg./kg. q.6.h. SECOBARBITAL SODIUM

Rectal 6 mg./kg.

(SECONAL) THIOMERIN

J.M. Below 3 kg.: 0.125 ml. 3-7 kg.: 0.25 ml.

THYROID (DESICCATED)

P.O.

Start with 15 mg. once daily. Increase by 15 mg. q.2. weeks to level of tolerance.

17. Antimicrobial Drugs for the Premature and Full-Term Infant

many antibiotics can be given orally, the parenteral route is usually preferential in the ill neonate who requires therapy. Sensitivity studies of the causative organism should be used whenever possible to assist in selection of the most useful and least toxic drug. Toxic effects of the antimicrobial agents used should be known, recognized in their early manifestations, and then appropriate changes made. AL THOUGH

CHLORAMPHENICOL

May be used in a severe generalized infection until sensitivity of causative organism is known. Occasionally associated with bone marrow depression. Overdose may cause gray syndrome. Avoid prolonged use. Check haematological status frequently. ROUTE AND DOSAGE

Intramuscular, intravenous (succinate) Premature Full term

25 mg./kg./day) ) 50 mg./kg./ day)

divided q.12.h.

Oral route, same dosage, divided q.6.h. (use powder contents of chloramphenicol capsules) . In the full-term baby, after three weeks of age, with a severe infection e.g., meningitis, 80 mg./kg./day is indicated, divided q.12.h.

182

THE NEW-BORN

COLISTIMETHATE

Despite nephrotoxicity and neurotoxicity this drug may be required for infections with pseudomonas aeurginosa or aerobacter klebsiella. There is a cross resistance with Polymyxin B. Check

B.U.N.

Coly-mycin contains dibucaine and should not be given intravenously or intrathecally. ROUTE AND DOSAGE

Intramuscular 2.5-5 mg./kg./day, divided q.12.h. ERYTHROMYCIN

In sensitive staphylococcal infections may be used alone or in combination with chloramphenicol. ROUTE AND DOSAGE

Oral-30 mg./kg./day, divided q.6.h. Intramuscular or intravenous-12 mg./kg./day, divided q.8.h. Toxic reactions-vomiting, drug fever, overgrowth of monilia. KANAMYCIN

May be used in severe infections, where it is considered or proven to be associated with a septicaemia due to gram negative organisms other than pseudomonas. High serum levels may develop with impaired renal function and cause deafness. There is a cross resistance with neomycin. ROUTE AND DOSAGE

Intramuscular 15 mg./kg./day, divided q.12.h. for 48 hours.

ANTIMICROBIAL DRUGS

183

If continued dosage is necessary reduce to 6 mg./kg./day, divided q.12.h. Prolonged treatment is to be avoided.

NEOMYCIN For susceptible enteropathogenic coliform organisms. Cross resistance with kanamycin. Watch for nephrotoxicity as some absorption from bowel occurs. ROUTE AND DOSAGE

Oral (not appreciably absorbed) 25 mg./kg./day in the premature divided q.6.h. 50 mg./kg./day in the full term divided q.6.h. ( unless azotaemic or oliguric) . NOTE

This drug should not be given parenterally.

PENICILLINS 1 Gram= 1,500,000 Units Rarely is it necessary to use more than 0.5 G./kg./day (750,000 U./kg./day). Use Na derivatives.

Penicillin G ROUTE

Intramuscular, intravenous, oral DOSAGE

20,000-30,000 U./kg./day, divided q.8.h. Ampicillin ROUTE

Intramuscular, intravenous, oral

184

THE NEW-BORN

DOSAGE

50-250 mg./kg./day, divided q.6.h. NOTES

Nearly the same gram positive but more gram negative activity, particularly for haemophilus influenza, than penicillin G; destroyed by staphylococcal penicillinase.

If the organism is sensitive in neontal meningitis, ampicillin should be given 400 mg./kg./day intravenously in the full term, divided q.4 to 6.h., in the premature 300 mg./kg./day intravenously divided q.6.h., in addition add 5 mg. intrathecally daily for sensitive coliform organisms. Cloxacillin For resistant staphylococcal infections. Penicillinase resistant. ROUTE AND DOSAGE

Oral, intramuscular, intravenous 50-250 mg./kg./day, divided q.6.h. Methicillin ROUTE

Intramuscular, intravenous Indications and dosage are the same as cloxacillin. In staphylococcal pneumonia or meningitis give intravenously, 400 mg./kg./day, in the full term, divided q.6.h.; 200 mg./kg./ day, in the premature. For staphylococcal meningitis add 5 mg. intrathecally daily, as necessary. POLYMYXINB

Indicated in meningitis or septicaemia due to pseudomonas aeruginosa, aerobacter, or klebsiella, which are resistant to other

ANTIMICROBIAL DRUGS

185

antibiotics. There is a cross resistance with colistimethate. May cause fever; check for nephrotoxicity. ROUTE AND DOSAGE

Intramuscular 2.5 mg./kg./day, divided q.8.h. In meningitis due to pseudomonas must be given intrathecally 1-1.5 mg./day, as well as parenterally.

SULPIDSOXAZOLE INDICATIONS

In combination with penicillin and chloramphenicol in treatment of the "unknown" meningitis and in urinary tract infections. ROUTE

Intravenous, oral DOSAGE

50 mgm./kg./day, divided q.6.h. NOTES

Should not be used in the first week or given to jaundiced infants. In older full-term infants with meningitis the dosage may be increased to 120 mg./kg./day.

STREPTOMYCIN Useful in combination with penicillin in treatment of a generalized infection until sensitivity studies are completed. Continued high dosage should be avoided because of possibility of 8th nerve damage. ROUTE

Intramuscular DOSAGE

20 mg./kg./day, divided q.12.h.

Index

Aero BASE IMBALANCE: metabolic acidosis, 83-4; metabolic alkalosis, 8S; respiratory acidosis, 84-5 ABO incompatibility, 117-18 Adrenal, congenital hyperplasia, 149-52 Antibiotic dosage, 181-5 Anus, imperforate, 156 Apgar chart, 7 Apgar rating, 20 Atelectasis, 92 Atresia, biliary, 119-20

Coombs' test; direct and indirect, 125 Craniostenosis, 157 Cretinism, 155 Crigler-Najjir syndrome, 122 Cryptorchidism, 153 Cyanosis: cardiac disease, 102; central nervous system dysfunction, 106-7; clinical causes, 1001; methaemoglobinaemia, 108-9; respiratory disease, 101-2; shock, 106; tetany and convulsions, 127; treatment, 104-6

BABY, FULL TERM: breast feeding, lS-16; care in delivery room, 7; care in nursery--eyes, skin, suction, 8-10; examination, 10; formula feeding, 17-18; isolation, 10; vitamin requirements, 18 Blalock operation, 109 Blood loss, 75 Blood, normal values, 174; procuring for culture or electrolytes, 173 Breast engorgement, 19 Brushfield spots, 159

DAMAGE, INTRACRANIAL, 74, 98, 130 Defects, congenital: environmental influences, 140-1; genetic considerations, 141-3; risk of recurrence, 142-3 Dehydration, 80-1 Dermatitis, perianal, 44-5 Diarrhoea: benign, 63; serious, 64-5; management, 65-7 Dosages of drugs, 176-80 Down's syndrome, 144-5 D-trisomy syndrome, 147 Drugs, teratogenic, 137-8 Dubin-Johnson syndrome, 122 Dysphagia: mechanical problems, 48; neuromuscular problems, 4950

CARDIAC FAil..URE, 76 Cardiac massage, 163-4 Chromosomal abnormalities, 142 Circumcision, 169 Cleft palate, 158 Convulsions: classification and management, 128; pathophysiology, 127; recurrent seizures, 135

ELECTROLYTE DISTURBANCES:

hypernatraemia, 86; hypocalcaemia, 86; hypokalaemia, 8S; hyponatraemia, 86 Epidermolysis bullosa, 46 Epithelial pearls, 19

INDEX Erb's palsy, 20 E-trisomy syndrome, 147 FAT NECROSIS, SUBCUTANEOUS, 45 Ferric chloride test, 160 Fluid requirements, 81 Formulae: for cardiac failure, 28; for diarrhoea, 26; for phenylketonuria, 27; hypoallergic feedings, 25; powdered milk, 25; simulating breast milk, 24; with lower fat and higher protein, 24 GALACTOSABMIA, 123, 155 Genital malformations: benign conditions, 148; classification, 149; indications for surgery, 156-7 Gilbert's disease, 122 Glenn procedure, 109 Glucose-6-phosphate dehydrogenase deficiency, 123 Guthrie test, 160 liABMOLYSIN TEST, 125 Harelip, 15 8 Heart: congenital malformation, 98-9; failure, 76; massage, 163-4 Hepatitis, neonatal, 119-20 Hermaphroditism, 153 Hiatus hernia sling, 67 Hip, congenital dislocation, 12 Hydrocephalus, 157 Hypematraemia, 86 Hypocalcaemia, 86 Hypoglycaemia, 132, 155 Hypokalaemia, 85 Hyponatraemia, 86 Hypospadius, 153 IMPETIGO, 45-6 Incubator: temperature and humidity, 36-7; rotation and care, 37 Infection, 135 Intertrigo, 43-4 Intravenous therapy: cut down, 171; micro-drip, 87; stab, 170 JAUNDICE: ABO incompatibility, 117-18; biliary obstruction, 119-20; classification, 111;

187

haemolytic disease, 112-18; hyperbilirubinaemia, 120-4; infection, 118-19; management, 111-12; physiological, !12; phy~ology, 110; rhesus mcompatibility, 113-115 LABIAL ADHESIONS, 153 Le cri du chat syndrome, 147 Lumbar puncture, 172 Lung: aspiration, 92-3; atelectasis, 92; expanding lesions, 92; idiopathic respiratory distress, 93-8; obstructive lesions, 91; staphylococcal pneumonia, 72-3 MAZZINI TEST, 125 Meatal stenosis, 153 Meningitis, 72 Meningocoele, 156-7 Milia, 42 Miliaria, 43 Moro reflex, 20 NARCOTIC

WITHDRAWAL,

138

OBSTRUCTION OF BOWBL,NBONATAL, 78 Omphalitis, 71 Osteomyelitis, 73 PAPAIN TEST, 125-6 Pedatrol, 87 Phenistix test, 160 Phenylketonuria, 154 Pneumonia: aspiration, 92; staphylococcal, 72 Poisoning, unsuspected, 76 Prematurity: anaemia, 37; antibiotics 33; definition, 29; foetal growth, 30; infection, 38; mortality and morbidity, 30-1; stimulation, 32-3; the large premature, 33-4; the small premature, 34-5; the very small premature, 35; thrush, 38; vitamin K, 32; vitamins, 35 Pseudohermaphroditism: female, 149; male, 152 Pudenz valve, 159-60 Pyruvate kinase deficiency, 123

188

INDEX

RESPIRATORY DISTRESS: aetiology and management, 89 Resuscitation in the new-born, 162 Rhesus incompatibility: antepartum recognition, 113; clinical evaluation, 114; investigative backuound, 112; laboratory findings, 114-15; severe cases, 114; treatment, 115-18 Rose Bengal test, 126 SEBORRHOEA, 44 Seizures, recurrent, 135 Serum, normal values, 174 Simian crease, 160 Spheroctosis, congenital, 121 Skin, general principles in care of, 42 Subdural tap, 172-3 Steroids: 17-hydroxycorticoids, 161; 17-ketosteroids, 160

Syndromes: Crigler-Najjir, 122; Down's, 144; D-trisomy, 147; Dubin-Johnson, 122; E-trisomy, 147; Gilbert's, 122; le cri du chat, 147; Turner's, 147 TETANY, 127-31 Toxic erythema, 43 Transfusion, replacement, 164-9 Turner's syndrome, 147 URINARY TRACT INFECTION, 72 Urine collection, 174 Urobilin and bilirubin, 126 VAN DEN BERGH TEST, 126 Vitamin K, 6 Vomiting: clinical assessment, 51-6; management, 57-8; pathophysiology, 51