Neonatology

(B) at birth, the blood volume is about 85 mL/kg body weight, falling to about 75 mL/kg after the first month.

(D) for neonates isolation of normally sterile fluid from cerebrospinal fluid, urine, and venous blood will help in identifying the bacterial agent responsible for infection. In children less than 2 months with temperatures of 38oC a complete evaluation includes a lumbar puncture, chest X-ray, urinalysis, and blood culture when the child has no focal signs. An elevation of total bilirubin causing jaundice is associated with sepsis or urinary tract infection.

(C) The Apt test is useful in distinguishing maternal blood from newborn blood.

(B) Polycythemia is seen in infants of diabetic mothers and for whom there was late clamping of the umbilical cord. Neurologic symptoms include jitteriness and poor feeding and appear with plethora and acrocyanosis. Consequences include heart failure and necrotizing enterocolitis. Elevated viscosity occurs at hematocrits above 65%. It is accentuated by the decreased deformability and filterability of neonatal red blood cells predisposing to stasis in small capillaries.

(A) While breast-feeding can cause jaundice, no cases of kernicterus have occurred in healthy breast-feeding infants. Acidosis and sepsis along with asphyxia, hypoglycemia, respiratory distress syndrome, intraventricular hemorrhage, and meningitis predispose an infant to kernicterus. Since bilirubin is bound to albumin, decreased albumin levels or loss of binding sites through displacement by drugs increases the amount of bilirubin that may enter the brain

(D) Detection of low levels obtained from maternal reported a higher incidence of trisomy 21. Low levels also accompany increased incidence of fetal growth disturbance, fetal hypoxia, and other poor pregnancy outcomes. Elevated levels obtained at 16 to 18 weeks gestation identify 80 to 90% of fetuses at risk for anencephaly and meningomyelocele. When the level is greater than 2.0 times the median value, an ultrasound and amniocentesis are used to examine the fetus.

(A) Mongolian spots usually disappear within the first year of life. There is no increased incidence of these lesions with the various trisomy syndromes.

(D) The tests are usually not completely descended until after 36 weeks and scrotal rugae are few and limited to the anterior and inferior aspects of a relatively small scrotum.

(D) The edema of caput succedaneum usually disappears within the first few days of life and requires no specific therapy.

(C) The first step is to consider that the tube has slipped down the right mainstem bronchus. This is easy to remedy. Pneumothorax is certainly a consideration but should not be the first one should consider in this situation.

(A) There is a positive correlation between maternal vaginal and infantile oral moniliasis. This is the primary means of infection in a newborn.

(B) Meconium ileus is associated with cystic fibrosis. Deficiency of pancreatic enzyme limits normal digestive activities in the intestine. As a result, meconium is left in a viscid mucilaginous state, and it clings to the walls of the intestine. Movement is difficult or impossible.

(E) All but penicillin therapy has been associated with persistent jaundice during the first month of life. Cytomegalic inclusion disease causes a hepatitis, hepatosplenomegaly, and a jaundice from conjugated hyperbilirubinemia. Congenital atresia results in accumulation of conjugated bilirubin associated with the obstruction of or reduced numbers of bile ducts. Galactosemia resulting from the absence of galactose-1-phosphate uridyl transferase causes injury to the liver from the accumulation of galactose. It persists until the initiation of a glactose free diet. Finally, a sequela of hemolytic disease is the significant elevation of direct and indirect bilirubin causing a disorder called inspissated bile syndrome. The jaundice clears within a few weeks or a month.

(D) Direct bilirubin is not elevated. Unconfirmed evidence suggests that some mothers have substances in their milk that competitively inhibit glucoronyl transferase conjugation. Other sources identify the relatively low fluid and calorie intake in the first week as the etiology. The management should emphasize frequent feedings 2 to 3 hours apart day and night to increase the amount of fluid and to stimulate stooling with elimination of bilirubin from the intestines. The rise in bilirubin by the third day of life is closely associated with breast-feeding. Kernicterus has not been seen in healthy, jaundice breast-fed infants.

(D) Neonatal tetany characterized by irritability, poor feeding, vomiting, and occasionally seizures is usually associated with transient hypoparathyroidism and not thyroid deficiency.

(E) Newborns with severe illnesses may develop hypoglycemia as a result of increased metabolic needs that are out of proportion to substrate stores. Hyperoxia by itself is not a stimulus for hypoglycemia.

(B) Weight loss occurs during the first 3 to 4 days of life. Jaundice may be present at birth or rise at any time in the neonatal period depending on the condition producing the elevation in bilirubin level.

(C) The course of breast-feeding jaundice can have prolonged elevation of bilirubin levels through the third week of life. Breastfed infants should regain their birth weight by 14 days of life compared to 10 in the formula fed infant.

E. new born infants have a relative deficiency of vitamin K, especially those who are breast-fed; most infants are routinely administrated vitamin K at birth to prevent bleeding complications associated with deficiency.

D. The case describes a child with diaphragmatic hernia. As a result of herniated bowel contents into the chest, children with this condition often have pulmonary hypoplasia. Bag and mask ventilation will cause accumulation of gas into the bowel (which is located in the chest) and further respiratory compromise. Therefore, endotracheal intubation is the best course of action

A. If the heart rate is still below 60 bpm despite positive pres¬sure ventilation with 100% oxygen, then chest compressions are used for 30 seconds. If the heart rate is still below 60 bpm, then drug therapy (usually epinephrine) is indicated.

A. Infants are obligate nose breathers. When crying they can breathe through their mouth, but when quiet they must have a patent nose. Choanal atresia is demonstrated by attempting to pass a feeding catheter through each nostril. When choanal atresia is diagnosed by failure to pass the feeding tube, endo¬tracheal intubation to bypass the airway obstruction is done until surgi¬cal repair can be completed.

A. Intraventricular hemorrhage is a frequent complication in preterm infants, and is associated with seizures, hydrocephalus and periventricular leukomalacia (PVL). A grade IV bleed in¬volves the brain parenchyma and puts this child at higher risk for neurodevelopmental handicap.

C. Infants of diabetic mothers, especially when poorly con¬trolled during pregnancy, are at risk for respiratory distress syndrome.

B. Infants born to mothers with poorly controlled gestational di¬abetes are at risk for congenital heart anomalies, cardiomyopa¬thy, septal hypertrophy, and subaortic stenosis. This child’s symptoms of failure of heart rate and color to fully respond to re¬suscitation efforts and the maternal history of diabetes are clues that suggest the infant is at higher risk of cardiac problems. Sep¬sis can present in a similar fashion and certainly should remain in the differential diagnosis until further evaluation is complete, but no particular risk factors for infectious disease are known in this case. This child is also at high risk for hypoglycemia in the first hours of life, but hypoglycemia alone would be a less likely explanation for his constellation of symptoms

E. Administration of phenobarbitol induces glucuronyl transferase in newborn infants, thus reducing rather than exacerbating neonatal jaundice

C. Gilbert disease is associated with abnormality of bilirubin transport in hepatocye.

B. Although the etiology of the hyperbilirubinemia must be investigated, phototherapy should be started.

D. The time of onset of symptoms in a neonate with conjunctivitis is somewhat helpful in the diagnosis of ophthalmia neonatorum. Chemical conjunctivitis is a self-limited condition that presents within 6 to 12 hours of birth; it is a consequence of irritation secondary to silver nitrate or erythromycin prophylaxis applied to the eyes. Gonococcal conjunctivitis usually has its onset within 2 to 5 days of birth and is the most serious of the bacterial infections; prompt and aggressive topical treatment and systemic antibiotics are indicated to prevent serious com¬plications such as corneal ulceration, perforation and resulting blindness. Parents should be treated for gonococcal disease to avoid the risk to the child of reinfection. Chlamydial conjunc¬tivitis often presents 5 to 14 days after birth and is usually treated with systemic erythromycin (in part, to reduce the risk of the in¬fant’s developing chlamydial pneumonia at 2 to 3 months of age). The benefits of using oral erythromycin in infants must be weighed with the increased risk of their developing hypertrophic pyloric stenosis, a condition found to be more common in chil¬dren having received oral erythromycin. Both parents of a child with chlamydial conjunctivitis must also be treated.

B. Transient tachypnea of the newborn (TTN) is a respiratory condition that results from incomplete evacuation of fetal lung fluid in full-term infants. It is more common in cesarean deliveries. It usually disappears within 24 to 48 hours of life. Often no treatment is indicated unless the infant requires low amounts of supplemental oxygen.

C. HIV transmission from mother to infant has decreased by more than 50% over the past 15 years, probably as a result of perinatal administration of antiretroviral medications to the mother and a course of zidovudine to the exposed infant. ELISA is an antibody test that will be positive in the infant because ma¬ternal antibodies for her HIV disease are passed through the pla¬centa; it is not a useful test in the newborn infant to determine neonatal infection. IVIG has not been shown to have a role in decreasing perinatal transmission. Healthy infants born to HIV¬ infected mothers do not need special monitoring nor do they need routine radiographs.

D. Home cardiorespiratory monitoring has not been shown to decrease the incidence of SIDS. Therefore, the potential bene¬fits of decreased caregiver anxiety and possible avoidance of an adverse event must be considered against the inconvenience and cost involved. Symptomatic premature infants (i.e., those with apnea and bradycardia events) are among those who are consid¬ered to be at higher risk for an adverse event, and for whom monitoring is therefore recommended. Monitors are also rec¬ommended by some. but not all experts when more than one child in a family has died from SIDS. Monitoring is not recommended for the infants in choices A, B, or C.

C. The terminal ileum is the location of B12 absorption; the child in this case has had the terminal ileum resected. Oral re¬placement of vitamin BI2 would be ineffective. Folate is ab¬sorbed throughout the small intestine. The amount of resected intestine is insufficient to cause the need for long-term par¬enteral nutrition, as the infant still has an adequate amount of small intestine remaining.

C. This infant potentially has a serious bacterial infection, and a complete evaluation including a lumbar puncture is performed. Infants do not reliably demonstrate Kernig or Brudzinski signs; thus. a lack of nuchal rigidity should not preclude a lumbar puncture. Performing a computerized tomography scan before lumbar puncture in an infant with an open anterior fontanelle is rarely necessary as herniation of the brain is exceedingly rare.

A. C. difficile colonizes approximately half of normal healthy infants in the first 12 months. In this infant with no history of antibiotic treatment and no current symptoms, treatment is unnecessary. C. difficile colitis rarely occurs without a history of recent antibiotic use.

C. unlike older children and adults with localized cutaneous disease, infants with suspected herpes skin lesions requires par¬enteral antiviral therapy to prevent more serious sequelae.

D. Even though the risk of viral transmission in the setting of a recurrent outbreak of herpes is low, cesarean section is indicated if lesions are present at the time of delivery. Surveillance cul¬tures are not likely to be helpful as negative results obtained a few days prior do not preclude an outbreak at the time of deliv¬ery, and results of a more recently obtained specimen may not be available. Culture specimens may be indicated at 48 hours of life from the infant born to herpes positive mother and treat¬ment with acyclovir initiated if any culture is positive or there are signs of neonatal HSV 1

D. Seborrheic dermatitis presents in infancy and adolescence. The chronic symmetric eruption, characterized by overproduction of sebum, affects the scalp, forehead, retroauric¬ular region, auditory meatus, eyebrows, cheeks and nasolabial folds. The treatment includes softening the scales with mineral oil and daily shampooing with a mild shampoo; some suggest shampoos containing ketoconazole (Nizoral). Low to medium potency topical corticosteroids may be used. Avoid scrubbing the affected areas.

D. Approximately 20% of normal neonates develops at least a few comedones within the first month of life. The cause of neonatal acne is unknown but has been attributed to placental transfer of maternal androgens, hyperactive adrenal glands, and a hypersensitive neonatal end-organ response to androgenic hormones

B. A chlamydial infection is the most likely cause of conjunc¬tivitis in this 2-week-old infant. Chlamydia trachomatis causes pneumonia in infants, generally between the ages of 1 and 3 months. Infants present with cough, tachypnea, and rales and are afebrile. A complete bood count might show eosinophilia. A 2- to 3-week course of’ oral erythromycin is given.

D. The Apgar score at 1 minute reflects the neonatal environment immediately prior to birth and the 5-minute score correlates with the infant’s response to resuscitation. The Apgar scores are not an accurate reflection of morbidity. Your exami¬nation is a much better indicator of the child’s outcome, but you cannot rule out the possibility of cerebral palsy on· the basis of a normal neonatal physical examination. It is always best to leave discussion of the events of delivery to the obstetrician, keeping in mind that the majority of difficult deliveries are the result of a previously unidentified-antenatal insult. Avoidance of the parents at this emotional time will likely only further their anxiety and may impede your future efforts to provide for the child.

C. Meconium ileus occurs when inspissated meconium ob¬structs the distal ileum. It is thought to be caused by deficiency of proteolytic enzymes. Obstruction begins in utero, resulting in underdevelopment of distal lumina. It is almost always associ¬ated with cystic fibrosis. Intestinal atresia and Hirschsprung (congenital aganglionic megacolon) cause clinical pictures sim¬ilar to this baby’s, but the radiographic findings for this child are most consistent with meconium ileus. Necrotizing enterocolitis also causes emesis and abdominal distension. but would be un¬likely in a term infant with no history of ischemic injury, and the colon would be expected to be of normal size. Constipation would not be consistent with the severity of this baby’s clinical picture, nor would it produce the described radiographic picture.

C. Meconium ileus is a surgical emergency, as volvulus and per-foration peritonitis are not uncommon complications.

C. This baby most likely has hyperbilirubinemia secondary to liver immaturity, possibly complicated by polycythemia. You would expect him to have a high level of unconjugated bilirubin (included in the total bilirubin test), but his conjugated (or di¬rect) portion should be normal in the absence of intrahepatic disease.

D. Renal vein thrombosis can present as an abdominal mass (hydronephrosis) in neonates after an episode of asphyxia. Such in-fants may have gross hematuria (or no hematuria at all), but mi-croscopic hematuria is more common. Hypertension is uncommon in the acute period following renal vein thrombosis but may occur as a late complication. The affected kidney may recover normal function. or it may atrophy. Bilateral renal vein thrombosis can lead to chronic renal failure. If the infant in this question had delayed passage of its first stool, small left colon syndrome, another complication of the infant of a diabetic mother, might also be considered as a cause of the abdominal mass.

D. Although all newborn infants are relatively deficient in the enzyme uridine diphosphate glucuronosyl transferase; those with Crigler-Najjar syndrome type 1 have as severe deficiency, leading to high bilirubin levels and encephalopathy :the mainstay of therapy is photoherapy, although research for other treatment options is going. Encephalopathy is rare in infants with Crigler-Najjar syndrome type II, in which serum biliribun levels rarely exceed 20 mg/dL.

C. Listeria is a gram-positive rod isolated from soil, streams, sewage, certain foods, dust, and slaughterhouses. The food-borne transmission of disease is related to Mexican (soft ripened) cheese, whole and 2% milk, undercooked chicken and hot dogs, raw vegetables, and shellfish. The newborn infant ac¬quires the organism transparently or by aspiration or inges¬tion at the time of delivery. The mortality rate of early onset disease is approximately 30%.

E. This infant is likely to have esophageal atresia. The VATER or VACTERL association of congenital anomalies includes vertetebral anomaly, anorectal malformation, tracheoesophageal fistula, cardiac (congenital heart disease), renal abnormalities, and limb defects.

B. Evaluation of the neonate born with respiratory distress with unilateral breath sounds must include examination of the ab¬domen. With asymmetric breath sounds both pneumothorax and congenital diaphragmatic hernia must be urgently considered. In this case, the infant was found to have a scaphoid abdomen increasing the likelihood that the infant has congenital dia¬phragmatic hernia; needle thoracostomy is contraindicated as intestinal perforation may occur. The patient must be stabilized immediately and the need for ECMO can only be ascertained af¬ter evaluating the infant’s response to initial therapy.

A. Endotracheal intubation with gentle ventilation should be initiated in an infant with congenital diaphragmatic hernia and respiratory distress. Bag-mask ventilation can lead to aeration of the thoracic intestinal contents leading to increased respira¬tory distress. Needle thoracostomy is contraindicated as intes¬tinal perforation may occur.

B. Conjunctivitis presenting in the first few days of life is most likely caused by chemical irritation. Laboratory evaluation of the eye discharge is performed; treatment usually can be with¬drawn and is based upon culture results.

The answer is c. It is important to make the diagnosis of choanal atresia quickly because it responds to treatment but can be lethal if unrecognized and untreated. Most neonates are obligate nose breathers and cannot breathe adequately through their mouths. Infants with choanal atresia have increased breathing difficulty during feeding and sleeping and improved respirations when crying. A variety of temporizing measures to maintain an open airway have been used, including oropharyngeal airways, position¬ing, tongue fixation, and endotracheal intubation, but surgical correction with placement of nasal tubes is most effective. The diagnosis can be made by failure to pass a catheter through the nose to the pharynx. Bronchoscopy would help diagnose lower airway anomalies. A ventilation-perfusion scan would be the appropriate examination if you were concerned about mismatch (e.g., that associated with pulmonary em¬bolism). A newborn with a hemoglobinopathy such as sickle cell or thalassemia would not present as this infant did, so an electrophoresis would not be helpful. An echocardiogram would be useful if you suspected con¬genital cyanotic heart disease. The lack of a murmur in a newborn does not rule out pathology: this would be a reasonable next step if the catheter passed through both nares without difficulty.

The answer is c. It is important to make the diagnosis of choanal atresia quickly because it responds to treatment but can be lethal if unrecognized and untreated. Most neonates are obligate nose breathers and cannot breathe adequately through their mouths. Infants with choanal atresia have increased breathing difficulty during feeding and sleeping and improved respirations when crying. A variety of temporizing measures to maintain an open airway have been used, including oropharyngeal airways, position¬ing, tongue fixation, and endotracheal intubation, but surgical correction with placement of nasal tubes is most effective. The diagnosis can be made by failure to pass a catheter through the nose to the pharynx. Bronchoscopy would help diagnose lower airway anomalies. A ventilation-perfusion scan would be the appropriate examination if you were concerned about mismatch (e.g., that associated with pulmonary em¬bolism). A newborn with a hemoglobinopathy such as sickle cell or thalassemia would not present as this infant did, so an electrophoresis would not be helpful. An echocardiogram would be useful if you suspected con¬genital cyanotic heart disease. The lack of a murmur in a newborn does not rule out pathology: this would be a reasonable next step if the catheter passed through both nares without difficulty.

The answer is e. There are few contraindications to breast-feeding. Active pul¬monary tuberculosis and HIV are two examples of infectious contraindica¬tions in developed countries, as well as malaria, typhoid fever, and septicemia. All medications taken by the mother will be secreted in breast milk, but usually not in amounts significant enough to affect the infant. Mothers taking antineoplastic agents should not breast-feed. Mothers with mastitis can continue to breast-feed; frequent feedings may actually help the condition by preventing engorgement. Mothers with mild viral illness may also continue to breast-feed. Cracked or bleeding nipples may make breast-feeding uncomfortable, but are not contraindications. Inverted nip¬ples can usually be remedied, and only rarely prohibit breast-feeding.

The answer is a. The low Apgar scores, meconium stain¬ing, and ensuing respiratory distress suggest that asphyxia has occurred. During a period of asphyxia, the resulting hypoxemia, acidosis, and poor perfusion can damage a neonate’s brain, heart, kidney, liver, and lungs. The resulting clinical abnormalities include cerebral edema, irritability, sei¬zures, cardiomegaly, heart failure, renal failure, poor liver function, dis¬seminated intravascular coagulopathy, and respiratory distress syndrome. There can be excessively high pulmonary arterial pressure at the same time systemic blood pressure begins to fall, resulting in a persistent right-to-left shunt across a patent ductus arteriosus or foramen ovale.

The answer is a. Cytomegalovirus infection is the most common cause of congenital infection, occurring in 0.2 to 2.4% of all live births. Cytomegalic inclusion disease is a constellation of findings including hepatomegaly, splenomegaly, jaundice, petechiae, purpura, and micro¬cephaly. In the United States, 20 to 90% of women of childbearing age have serologic evidence of a past infection with CMV. Symptomatic congenital disease usually occurs when a mother has a primary CMV infection in the first trimester of pregnancy. Many of these babies die. and those who survive are severely affected. In the event of reactivation of CMV infection during pregnancy, maternal IgG, passed transplacentally, protects the infant from serious infection. Although most infants infected during this sec¬ondary maternal infection are asymptomatic, about l% of them eventu¬ally manifest hearing and neurologic problems. Some recommend keeping a child with congenital CMV infection away from susceptible pregnant (or about to become pregnant) women because CMV excretion can persist for months to years; at the very least, good hand-washing practices should be instituted. If infected shortly after birth, the younger sibling will probably be asymptomatic since he or she has maternal IgG in the circulation. CMV is primarily an occult infection. Of toddlers in day-care centers, 20 to 80% acquire CMV and shed it in saliva and urine for years. Diagnosis is made with isolation of the virus from urine, saliva, or other secretions.

The answer is b. Hematemesis and melena are not uncommon in the neonatal period, espe¬cially if gross placental bleeding has occurred at the time of delivery. The diagnostic procedure that should be done first is the Apt test, which differ¬entiates fetal from adult hemoglobin in a bloody specimen. The test is based upon the finding that fetal hemoglobin is alkali resistant, while adult hemoglobin will convert to hematin upon exposure to alkali. If the blood in an affected infants gastric contents or stool is maternal in origin, further workup of the infant is not needed.

The answer is c. Most medica¬tions are secreted to some extent in breast milk, and some lipid-soluble medications may be concentrated in breast milk. Although the list of contraindicated medications is short, caution should always be exercised when giving a medication to a breast-feeding woman. Medications that are clearly contraindicated include lithium, cyclosporin, anteneoplastic agents, illicit drugs including cocaine and heroin, ergotamines, and bromocreptine (which suppresses lactation). Although some suggest that oral contracep¬tives may have a negative impact on milk production, the association has not been proven conclusively. In general, antibiotics are safe, with only a few exceptions (such as tetracycline). While sedatives and narcotic pain medications are probably safe, the infant must be observed carefully for sedation. All of the medications listed in the question are considered safe, except for lithium.

The answer is d. The infant presented in the question has necrotizing enterocolitis. NEC, a potentially life-threatening disease of the neonate. It is more common in premature infants, but has been described in term infants as well. Although several organisms have been isolated from NEC patients, no clear cause for this condition has been identified. Patients present with feeding intolerance and a distended abdomen, about a quar¬ter have grossly bloody stool. Pneumatosis intestinalis is found on plain radiograph of the abdomen and is diagnostic for NEC in this age group. Management depends initially on the presence or absence of perforation; it no evidence of free peritoneal air is found, the infant should be put on bowel rest with nasogastric decompression, and systemic antibiotics are initiated. Electrolytes and vital signs should be monitored closely, and se¬rial abdominal films should be performed to evaluate for perforation. If free air is identified on plain radiographs or if the infant clinically worsens with medical management, surgical consultation is required. An exploratory laparotomy is usually performed, and any necrotic intestinal tissue is removed. Occasionally, removal of necrotic gut will result in an infant with¬out adequate intestinal surface area to absorb nutrition, a condition known as short bowel syndrome.

The answer is b. It usually is impossible with any combination of parenteral and enteral nutrition to match what the infant would have accumulated in utero. The average healthy, low-birth-weight infant of this size requires a daily intake of cal¬cium of about 200 mg/kg. Breast milk has much less calcium (and phos¬phorus) than do commercial formulas. The breast milk can be directly supplemented with calcium, or it can be supplemented with commercial fortifiers. Alternatively, it can be mixed with formulas designed for the premature infant. Breast milk promotes gut maturation and prevents intestinal atrophy induced by lack of enteral feeding; however, it is likely to have insufficient calcium and phosphorus for catch-up growth

The answer is d. A room temperature of 24°C provides a cold environment for newborn infants. Aside from the fact that these infants emerge from a warm, 37.6°C (99.5°F) intrauterine environment, at birth, infants (and especially preterm infants) are wet, have a relatively large surface area for their weight, and have little subcutaneous fat. Within minutes of delivery, the infants are likely to become pale or blue and their body temperature will drop. In order to bring body temperature back to normal, they must increase their metabolic rate; ventilation, in turn, must increase propor¬tionally to ensure an adequate oxygen supply. Because a preterm infant is likely to have respiratory problems and be unable to oxygenate adequately, lactate can accumulate and lead to a metabolic acidosis. Infants rarely shiver in response to a need to increase heat production. If the tachypnea persists after warming the infant, sepsis, pneumonia, and primary surfac-tant deficiency are all possible; thus, several of the alternative answers may then be appropriate.

The answer is b. If a mother is positive and her baby is A positive, the baby has a chance of developing hemolytic disease. Hemolytic disease and jaun¬dice caused by a major blood-group incompatibility are usually less severe than with Rh incompatibility. Although the hematocrit of affected infants usually is normal, elevation of the reticulocyte count and the presence of nucleated red blood cells and micro spherocytes in the blood smear provide evidence of hemolysis. In comparison with hemolytic disease caused by Rh incompatibility, where it is usually strongly positive, major blood-group incompatibility is often associated with a direct Coombs test that is fre¬quently weakly positive. Petechiae are usually associated with decreased number of platelets. Crescent-shaped (sickled) red blood cells are found with sickle cell disease.

The answer is a. Small-for-dates infants are subject to a differ¬ent set of complications than preterm infants whose size is appropriate for gestational age. The small-for-dates infants have a higher incidence of major congenital anomalies and are at increased risk for future growth retardation, especially if length and head circumference as well as weight are small for gestational age. Also more common are neonatal asphyxia and the meconium aspiration syndrome, which can lead to pneumothorax, pneumomediastinum. or pulmonary hemorrhage. These, rather than hya¬line membrane disease, are the major pulmonary problems in SGA infants. Because neonatal symptomatic hypoglycemia is more commonly found in small-for-dates infants, careful blood glucose monitoring and early feeding are appropriate precautions. Normal or elevated hematocrit is also more common in these infants.

The answer is a. Apneic episodes are characterized by an absence of respi¬rations for more than 20 s and may be accompanied by bradycardia and cyanosis. A large number of conditions can cause apnea. Periods of apnea are generally thought to be secondary to an incompletely developed respi¬ratory center, particularly when they are seen, as is common, associated with prematurity. Although seizures, hypoglycemia, and pulmonary dis¬ease accompanied by hypoxia can lead to apnea, these causes are less likely in the infant described, given that no unusual movements occur during the apneic spells, that the blood sugar level is more than 40 mg/dL, and that the child appears well between episodes. Other, less common, explanations for central apnea include congenital central hypoventilation syndrome (formerly known as Ondine’s curse), Arnold-Chiari malformations, and congenital infections. Periodic breathing, a common pattern of respi¬ration in low-birth-weight babies, is characterized by recurrent breathing pauses of 3 to 10 s.

The answer is a. most developed countries, screen in the neonatal period infants for congenital hypothyroidism. Identified and treated early, most affected children will have normal growth and intelligence. Untreated, the outcome of congeni¬tal hypothyroidism is mental retardation and poor growth (cretinism). Early treatment is essential and should not be delayed while arranging fur¬ther diagnostic evaluation. Approximately 85% of all cases of congenital hypothyroidism are a result of thyroid dysgenesis, generally a sporadic event. A sodium iodine uptake scan can help identify the underlying cause, but treatment should not be withheld pending the outcome of this or any other study after initial confirmation.

The answer is b. The clavicle is the most commonly fractured bone in the delivery process. While some fractures are identified at birth, others may not be identified until callus formation is noted at about a week of age. Clavicular fracture may happen in any delivery, although there is higher risk with large-for-gestational-age infants. Initial presentation of a fractured clavicle may include a pseudoparalysis, in which the infant refuses to move the ipsilateral arm, mimicking an Erb-Duchenne paralysis.

The answer is c. In a difficult delivery in which traction is applied to the head and neck, several injuries, including all those listed in the question, may occur. Erb-Duchenne paralysis affects the fifth and sixth cervical nerves; the affected arm cannot be abducted or externally rotated at the shoulder, and the forearm cannot be supinated. Injury to the seventh and eighth cervical and first thoracic nerves (Klumpke paralysis) results in palsy of the hand and also can produce Horners syndrome. Fractures in the upper limb are not associated with a characteristic posture, and passive movement usually elicits pain. Spinal injury causes complete paralysis below the level of injury. When paralysis of an upper extremity from injury to the brachial plexus is found in a neonate, injury to the phrenic nerve should also be suspected because the nerve roots are close together and can be injured concurrently. The paralyzed diaphragm can be noted to remain elevated on a chest x-ray taken during deep inspiration when it will con¬trast with the opposite normal diaphragm in its lower normal position; on expiration, this asymmetry cannot be seen. On inspiration, not only is breathing impaired since the paralyzed diaphragm does not contract, but also the negative pressure generated by the intact diaphragm pulls the mediastinum toward the normal side, impairing ventilation further. The diagnosis can easily be made by fluoroscopy, where these characteristic movements on inspiration and expiration can be seen. Rarely, both diaphragms can be paralyzed, producing much more severe ventilatory impairment. Fortunately, these injuries frequently improve spontaneously.

The answer is a. A subgaleal or subaponeurotic hemorrhage can be life threat¬ening; infants may lose a third or more of their blood volume into this potential space, leading to hypovolemic shock. The other two more com¬mon findings on a newborns head, caput succedaneum and cephalohematoma, may be distinguished by examination. Caput is edema and will feel firm and nonfluctuant; it may cross the midline. Cephalohematoma is limited by its location under the periosteum of the individual bones of the skull, so the lesion stops at suture lines and does not cross the midline. Cephalohematomas will typically feel fluid-filled or fluctuant. A subgaleal hemorrhage will feel like a cephalohematoma that crosses the midline. In a situation as described in the question, fluid resuscitation may be required, as may be transfusion. Careful monitoring is essential. Trying to identify the source of bleeding is rarely successful, so a CT is of limited benefit. Observation alone in this case could lead to shock and death. Surgical drainage is contraindicated except in cases of infected hematoma.

The answer is a. An infant of 2100 g at 38 weeks would be considered small for gestational age (SGA), a not uncommon consequence of maternal toxemia. Pregnancy induced hypertension can produce a decrease in uteroplacental blood flow and areas of placental infarction. This can result in fetal nutritional deprivation and intermittent fetal hypoxemia, with a decrease in glycogen storage and a relative erythrocytosis, respec-tively. Hence, neonatal hypoglycemia and polycythemia are common clini¬cal findings in these infants. A blood glucose level of 30 mg/dL in a full-term infant, however, is probably normal during the first postnatal day, and an infant is very unlikely to have a convulsion as a result of a level of 41 mg. Serum calcium levels usually decline during the first 2 to 3 postna¬tal days, but will only be considered abnormally low in a term infant when they fall below 7.5 to 8 mg/dL. Neonatal hypermagnesemia is common in an infant whose mother has received MgSO4 therapy, but is usually asymp¬tomatic or produces decreased muscle tone or floppiness. A persistent venous hematocrit of greater than 65% in a neonate is regarded as polycythemia and will be accompanied by an increase in blood viscosity.
Manifestations of the “hyperviscosity syndrome” include tremulousness or jitteriness that can progress to seizure activity because of sludging of blood in the cerebral microcirculation or frank thrombus formation, renal vein thrombosis, necrotizing enterocolitis, and tachypnea. Therapy by partial exchange transfusion with saline or lactated Ringers solution is preferred, and may be more likely to be useful if per¬formed prophylactically before significant symptoms have developed, but literature looking at outcomes in these infants is lacking.

The answer is b. For the child described in the question, prematurity; and the clinical picture presented make the diagnosis of hyaline membrane disease (infant respiratory distress syndrome) likely. HMD is caused by surfactant deficiency, and the incidence is increased with decreasing gestational age and birth weight. In this disease, lung compliance is reduced; lung volume is also reduced, and a significant right-to-left shunt of blood, can occur. Some of the shunt can result from a patent ductus arteriosus or foramen ovale, and some can be due to shunting in the lung. Minute ventilation is higher than normal, and affected infants must work harder in order to sustain adequate respiration.

The answer is c. The transmission of HIV from mother to infant has decreased in recent years, due in large part to perinatal administration of antiretroviral medications to the mother and a course of zidovudine to the exposed infant. Studies suggest that a better than 50% decrease in transmission can be seen with appropriate medications as outlined.
An ELISA is an antibody test and will be positive in the infant born to an HIV-infected mother due to maternal antibodies that are passed through the placenta; it is not a useful test in the newborn infant to determine neonatal infection. IVIG has not been shown to have a role in decreasing perinatal transmission. Healthy asymptomatic term infants born to HIV-infected mothers do not need special monitoring, nor do they need routine radiographs.

The answer is c. Turner syndrome is a genetic disorder; the 45, X karyotype is the most common. At birth, affected infants have low weights, short stature, edema over the dorsum of the hands and feet, and loose skin folds at the nape of the neck. Some other findings with this syndrome include sexual infantilism, streak gonads, typical faces, shield chest, low hairline, coarctation of the aorta, hypertension, bicuspid aortic valve, high palate, and horseshoe kidney. Coarse, tremulous movements accompanied by ankle clonus; vascular instability as evidenced, for example, by a harlequin color change (a transient, longitudinal division of a body into red and pale halves); softness of parietal bones at the vertex (craniotabes); and a liver that is palpable down to 2 cm below the costal margin are all findings often demonstrated by normal infants and are of no diagnostic significance in the clinical situation presented.

The answer is d. Abdominal distention, choking, drooling, and coughing associated with feedings are symptoms of esophageal anomalies. The anomaly illustrated by D is the most common: that A can be diag¬nosed after repeated episodes of pneumonia. The anomalies in E and C are associated with all the same symptoms except abdominal distention, which cannot develop because air cannot enter the gastrointestinal tract. B and C are the least common; in these, the upper esophageal segment is connected directly to the trachea, and massive entry of fluid into the lungs occurs.
VATER association, a complex of cardiovascular malformations, skele¬tal malformations, and renal abnormalities, has tracheoesophageal fistula as a common finding.

The answer is b. Failure to give vitamin K prophylactically to newborn infants is associated with a decline in the levels of vitamin K-dependent coagulation factors. In less than 1% of infants (but especially those fed human breast milk), the levels reached are low enough to pro¬duce classic hemorrhagic manifestations on the second to seventh day of life. These manifestations include melena, hematuria, and bleeding from the circumcision; intracranial hemorrhage and hypovolemic shock are seri¬ous complications. Diagnosis of this condition is indicated by a prolonged prothrombin time, which reflects inadequate concentrations of factors II, VII, IX, and X. Bleeding time, fibrinogen, and platelets are normal.

The answer is d. The infant of a mother who is a carrier of hepatitis B surface antigen has a significant risk of acquiring infection. This usually occurs at the time of delivery, but infection can also be acquired dur¬ing pregnancy and postnatally. A small percentage of infected neonates develop acute icteric hepatitis, but the majority remain asymptomatic. Of these infected asymptomatic infants, 80% or more will develop chronic infection, the long-term consequences of which are chronic liver disease and, possibly, hepatocellular carcinoma. Combined passive-active immunoprophylaxis in the form of hepatitis B immune globulin and hepatitis B vac¬cine affords protection not only from immediate perinatal infection but also from infection that may be acquired as a result of continued exposure in the household of a chronic carrier.
Immunization in this infant is indicated regardless of the presence of hepatitis B “e” antigen (HBeAg) in the mother. Although the presence of HBeAg, especially in the absence of antibody to HBeAg, is associated with high rates of transmission to neonates, any woman positive for hepatitis B surface antigen (HBsAg) is potentially infectious. It is not necessary to iso¬late infants born to carriers of HBsAg, and screening of neonates for HBsAg is not indicated. Testing for HBsAg and anti-HBsAg at least 1 month after the third dose of hepatitis B vaccine will determine the efficacy of these measures.

The answer is a. The effect of a drug on the fetus is determined by the nature of the drug and by the timing and degree of exposure. Heparin does not cross the placental barrier and does not appear to directly affect the fetus once pregnancy is well established. Phenytoin may cause birth defects when given during the first trimester. Penicillin and aluminum hydroxide have not been found to affect the fetus. Propranolol, which may cause growth retardation when given throughout pregnancy, diminishes the ability of an asphyxiated infant to increase heart rate and cardiac output. It has also been associated with hypoglycemia and apnea.

The answer is b. Goat’s milk, by itself, is not an ideal source of infant nutrition, as it contains inadequate folate and iron, potentially contributing to anemia. Caloric content is actually more dense than cow’s milk-based formulas (about 30 kcal/oz, compared to 20 kcal/oz for for¬mula and breast milk). Casein and whey are the protein sources in goat’s milk, as they are for many formulas. Some manufacturers in different coun¬tries produce a goat milk-based formula that contains supplemental vita¬mins and iron. Unpasteurized goat’s milk should never be used as an infant formula because of the risk of brucellosis.

The answer is b. The development of jaundice in a healthy full-term baby may be considered the result of a normal physiologic process if the time of onset and duration of the jaundice and the pattern of serially deter¬mined serum concentrations of bilirubin are in conformity with currently-accepted safe criteria. Physiologic jaundice becomes apparent on the sec¬ond or third day of life, peaks to levels no higher than about 12 mg/dL on the fourth or fifth day and disappears by the end of the week. The rate of rise is less than 5 mg/dL per 24 h and levels of conjugated bilirubin do not exceed about 1 mg/dL. Concern about neonatal jaundice relates to the risk of the neurotoxic effects of unconjugated bilirubin.
The precise level and duration of exposure necessary to produce toxic effects are not known, but bilirubin encephalopathy, or kernicterus, is rare in term infants whose bilirubin level is kept below 18 to 20 mg/dL. Certain risk factors affecting premature or sick newborns increase their susceptibil¬ity to kernicterus at much lower levels of bilirubin. The diagnosis of phys¬iologic jaundice is made by excluding other causes of hyperbilirubinemia by means of history, physical examination, and laboratory determinations.
Jaundice appearing in the first 24 h is usually a feature of hemolytic states and is accompanied by an indirect hyperbilirubinemia, reticulocytosis, and evidence of red-cell destruction on smear. In the absence of blood group or Rh incompatibility, congenital hemolytic states (e.g., spherocytic anemia) or G6PD deficiency should be considered. With infection, hemo¬lytic and hepatotoxic factors are reflected in the increased levels of both direct and indirect bilirubin. Studies should include maternal and infant Rh types and blood groups and Coombs tests to detect blood-group or Rh incompatibility and sensitization.
Measurements of total and direct bilirubin concentrations help to determine the level of production of bilirubin and the presence of conju¬gated hyperbilirubinemia. Hematocrit and reticulocyte count provide in¬formation about the degree of hemolysis and anemia, and a complete blood count screens for the possibility of sepsis and the need for cultures.
examination of the blood smear is useful in differentiating common hemolytic disorders. Except for determinations of total and direct biliru¬bin, tests of liver function are not particularly helpful in establishing the cause of early-onset jaundice. Transient elevations of transaminases (AST and ALT) related to the trauma of delivery and to hypoxia have been noted.
Biliary atresia and neonatal hepatitis can be accompanied by elevated levels of transaminase but characteristically present as chronic cholestatic jaundice with mixed hyperbilirubinemia after the first week of life.

The answer is a. The clinical findings of congenital hypothyroidism are subtle, and may not be present at all at birth; this is thought to the result of passage of some T4 transplacentally. Infants with examination findings will usually have an umbilical hernia and a distended abdomen. The head may be large, and the fontanelles will be large as well. The child may be hypothermic and have feeding difficulties; constipation and jaun¬dice may be persistent. Skin may be cold and mottled, and edema may be found in the genitals and extremities. The heart rate may be slow, and ane¬mia may develop. As these findings may be subtle or nonexistent, neonatal screening programs are extremely important for early diagnosis of these infants.
Sepsis can cause hypothermia and poor feeding, but the two-week time makes this choice unlikely. Hirschsprung disease may cause chronic constipation and abdominal distension, but not the other findings. Botu¬lism can cause a flaccid paralysis and poor feeding, but the large fontanelles and umbilical hernia are not caused by this infection.

The answer is e. Transient tachypnea of the newborn is usually seen after a nor¬mal vaginal or, especially, after a cesarean delivery. The condition is a result of retained fetal lung fluid. These patients have tachypnea, retractions, grunting, and sometimes cyanosis. The chest examination is usually nor¬mal; the chest radiograph demonstrates prominent pulmonary vascular markings with fluid in the fissures and hyperexpansion (flat diaphragms), Therapy is supportive, with maintenance of normal oxygen saturation. Resolution usually occurs in the first 3 days of life.

The answer is d. Diseases that are due to defects in a sin¬gle gene are designated as autosomal or X-linked, depending on whether the affected gene is located on an autosome or an X chromosome. Geneti¬cally determined diseases that are multifactorial in origin (i.e., neural tube defects) do not conform to the Mendelian pattern of inheritance but exhibit a variable outcome that reflects the interaction between a particular geno¬type and an environment. The relatives of persons with diseases of multi-factorial origin have an increased risk of having similar abnormalities.
The recurrence risk for most single primary defects of multifactorial inheritance (e.g., neural tube defects) is increased with each child affected. This increased risk forms the basis for assuming that genetic factors play a role in the occurrence of these abnormalities. Other factors, such as race, sex, and ethnic and racial background, influence the frequency with which an abnormality of multifactorial inheritance occurs in relatives. The prena¬tal diagnosis of neural tube defects (anencephaly and meningomyelocele) can be made by the detection of elevated levels of a-fetoprotein in the amniotic fluid. To reduce the risk of neural tube defects, it is now recom¬mended that all women capable of becoming pregnant take 400 meg of folic acid daily.

The answer is b. Idiopathic apnea is common in prema¬ture infants but is not expected in the full-term newborn. When apnea occurs in the term infant, there is almost always an identifiable cause. Sep¬sis, gastroesophageal reflux, congenital heart disease, seizures, hypoglycemia, and airway obstruction can cause apnea in term newborns. Harlequin syndrome is a transient change in the skin color of the otherwise asymptomatic newborn (usually preterm) in which the dependent side of the entire body turns red while the upper side remains pale.

The answer is d. Neonatal sepsis, a clinical syndrome of systemic illness accompanied by bacteremia, often results in spread of infection to the meninges and other distant sites. The diagnosis of serious infection,, including meningitis, in a neonate is difficult because the signs and symp¬toms are subtle and nonspecific. They include lethargy; feeding problems including abdominal distention, vomiting, and diarrhea; temperature instability; respiratory distress or apnea; and jaundice. Nuchal rigidity and Kernig and Brudzinski signs are frequently not present in the neonate with meningitis.

The answer is b. Twin-to-twin transfusions occur in about 15% of monochorionic twins and commonly cause intrauterine death. This dis¬order should be suspected when the hematocrits of twins differ by more than 15 mg/dL. The donor twin is likely to have oligohydramnios, anemia, and hypovolemia with evidence of shock if the hematocrit is significantly reduced; the recipient twin is likely to have hydramnios and plethora and to be larger than the donor twin. A 20 percent difference in body weight may result. As the central venous hematocrit rises above 65%, infants can develop hyperviscosity, respiratory distress, hyperbilirubinemia, hypocalcemia, renal vein thrombosis, congestive heart failure, and convulsions.

The answer is b. There is loss of body weight of 1.5 to 2% per day for the first 5 days of life for a nor¬mal newborn infant as excessive fluid is excreted. One might think this would tend to cause hemoconcentration and produce an increase in hema¬tocrit, but, to the contrary, the hematocrit falls as an adaptation to an envi¬ronment of higher oxygen. Infants usually have several meconium stools during the first day or two of life, changing to soft yellow stools at 1 to 2 days of life. As the hematocrit falls, there is a corresponding increase in serum bilirubin that peaks around 3 to 5 days of life. Temperature should not change; temperature instability in a term infant is frequently a sign of serious infection

The answer is c. It is generally presumed that duodenal atresia and tracheoesophageal fistula lead to hydramnios (polyhydramnios) by interference with reabsorption of swallowed amniotic fluid. Hydramnios is also associated with approximately 80% of infants who have trisomy 18. Approximately 50% of women with anencephalic fetuses have polyhy¬dramnios. Oligohydramnios occurs in association with congenital abnor-malities of the fetal kidneys or other parts of the genitourinary tract, such as renal agenesis or obstruction, that impede normal formation or excre¬tion of fetal urine.

Answer is a
Sudden infant death syndrome (SIDS) usually does not occur in the first month of life. SIDS should not be confused with apnea of prematurity, because one is not related to the other. Apnea of prematurity occurs in the first month of life but does not contribute to mortality.

The answer is b
Indeed the low-birthweight rate has increased during this interval. This can be explained only in part by a rising number of multiple births following reproductive technology advances.

The answer is d
Premature rupture of membranes (PROM) is associated with risks of early-onset sepsis and premature birth. Both should contribute to neonatal, not postnatal, mortality.

The answer is c
These are the most ideal ages for safe maternal reproduction. Adolescents and women over the age of 40 yr are considered high risk.

The answer is c: Acrocyanosis is normal at this time, and if the physical examination is also normal there is no risk of a serious underlying disorder. Warming is all that may be needed.

The answer is b
This is the classic presentation of this benign neonatal rash. The flaccid vesicles contain neutrophils, which is in contrast to the eosinophils seen in erythemia toxicum.

The answer is a:
Vertex positioning with possible pressure of the developing skull by the mother’s bony pelvis contributes to this deformation

The answer is e
Child abuse is often associated with bilateral retinal hemorrhages, especially in what is called the “shaken baby syndrome.” Retinal hemorrhages may be noted immediately after birth. Nonetheless, they should all resolve by 1 mo of age.

The answer is c
Grunting is common, particularly after a cesarean section without prior labor. If grunting persists beyond 30 min or if there are other signs of distress, the child should be evaluated for sepsis, respiratory distress syndrome (RDS), or congenital heart disease.

Answer is b
The Apgar score helps to rapidly assess the need to resuscitate neonates after birth. Although it has some value in predicting neonatal mortality and cerebral palsy, it has a poor positive predictive value. Most children with cerebral palsy have had normal Apgar scores, while neonates with low Apgar scores do not universally get cerebral palsy.

Answer is b
Group B streptococci are not increased in these pregnancies. In addition to the risks of low birthweight (LBW) and very low birthweight (VLBW), as well as multiple-fetus
pregnancies, there is an increased risk of cerebral palsy, related in part to preterm birth.

Answer is e
Although monitoring for the rate of spontaneous uterine contractions before the onset of labor is popular, it has a poor sensitivity and a low positive predictive value for premature birth.

Answer is a
Esophageal atresia is most often associated with polyhydramnios, as are other upper intestinal obstructive lesions or disorders of fetal swallowing.

Answer is c
The lecithin-sphingomyelin ratio (L/S) predicts pulmonary maturity and is a functional test that is based on maturation of the fetal type II pulmonary alveolar cells. Fetal ultrasonography may estimate fetal gestational age, and thus the potential for lung maturity, but it cannot ascertain the actual L/S ratio.

Answer is a
Maternal lupus may affect the fetal cardiac conduction system, and it produces bradycardia by the
development of an antibody immune-related process.

Answer is e
It is interesting that B might be a choice. However, splenectomy reduces the destruction of maternal platelet antibody complexes by the spleen, thus increasing the mother’s platelet count. Splenectomy does not reduce the IgG antiplatelet antibodies, which can readily cross the placenta.
21. An important

Answer is c
Polyhydric aromatic hydrocarbons may alter fetal growth. It is very possible that enzymes that metabolize these to either toxic or-more likely-nontoxic metabolites modify the impact of the fetal growth restriction due to cigarette smoking.

Answer is c
This is future shock! Although one case of fetal valvotomy has been reported, it was not performed via PUBS but via fetal cardiac puncture.

Answer is b
Fetal-placental amniotic Candida infection may demonstrate nodules on the umbilical cord and a neonatal macular-papular, erythematous, disseminated rash.

Answer is a
Monozygotic twinning has little or no genetic determination. Many twins detected at 12 wk of gestation are singletons at 40 wk owing to fetal twin demise. This process
may affect the surviving twin (e.g., incidence of cerebral palsy
is higher in the surviving twin).

The answer is b
Some VLBW infants may have growth restriction, but almost all infants weighing <1500 g are premature.

The answer is b
Both bronchopulmonary dysplasia (BPD) and a patent ductus arteriosus (PDA) are associated with excessive intravenous fluids in premature infants. Answers A, C, D, or E are some reasons why fluid intakes may be increased, but at the same time, the infant is placed at risk for BPD or a PDA.

The answer is e
Answers A to D, other congenital heart defects, postnatal hemorrhage (CNS, liver, spleen), and sepsis can all produce hypotension.

The answer is b
Many studies have demonstrated the value of administering hydrocortisone (Solu-Cortef) to VLBW infants who respond poorly to fluid boluses and dopamine.

The answer is e
This is a classic problem in infants of diabetic mothers (IDMs). All jittery children should be examined carefully and concern for a seizure considered. If the child appears normal and has no abnormal eye movements, and if the motion is sensory-dependent, is only present during active wakefulness, and is stopped by simple pressure, it is probably not a seizure. Nonetheless, it could be associated with a serious problem such as hypocalcemia, hypoglycemia, or opiate withdrawal. IDMs are often more jittery than other infants but are also at risk for hypoglycemia and hypocalcemia.

The answer is d
Phototherapy is clearly indicated. Aspiration or incision and drainage (I + D) should not be done to manage a cephalohematoma.

The answer is a
In addition, nonroutine ultrasonography should be performed for symptoms of intraventricular hemorrhage (IVH) and for the follow-up of abnormalities noted on the first ultrasound.

The answer is e
Although repeat lumbar puncture (LP) is often done, most physicians do not believe that they avoid the need for a ventricular peritoneal (VP) shunt. Most cases of dilated ventricles after IVH do not necessitate later placement of a shunt.

The answer is a
Most Erb palsies resolve rapidly with immobilization, rehabilitation, and positioning. If there is no improvement between 3-6 mo, a referral for surgical evaluation
is indicated.

The answer is b
Apnea from any cause is treated with securing a patent airway and instituting ventilation. If bag mask ventilation is ineffective, endotracheal intubation should be performed.
The existing evidence from randomised controlled trials is insufficient to determine whether naloxone confers any important benefits to newborn infants with cardiorespiratory or neurological depression that may be due to intrauterine exposure to opioid. Given concerns about the safety of naloxone in this context, it may be appropriate to limit its use to randomised controlled trials that aim to resolve these uncertainties

The answer is a
Indeed, one expects that with a successful intubation and ventilation, the end-tidal (exhaled) CO2 will rise dramatically. This is now used in many neonatal resuscitations

The answer is b
A previously healthy “gainer and groaner” should not develop apnea of prematurity at 3 wk of age and at a corrected age of 35 wk. Sudden onset and repeated episodes strongly suggest an underlying illness. The abdominal distention also suggests an ileus or necrotizing enterocolitis.

The answer is c
Nasal CPAP is often quite effective when larger premature infants develop respiratory distress syndrome

The answer is a
In a neonate with no signs of intraventricular hemorrhage (IVH) and on the first day of life, one does not need a head ultrasound. The others (B-E) are important to look for other causes of respiratory distress and prepare to treat empirically for the possibility of early-onset group B
streptococcal pneumonia.

Answer is a
The possibility of a duct dependent cyanotic heart disease is unlikely, he mostly has PDA, chest infection is a possibility

The answer is d
Indomethacin, and perhaps ibuprofen when approved by the FDA, inhibits prostaglandin synthesis,
facilitating medical closure of a PDA. If medical treatment is unsuccessful, surgical ligation is performed.

The answer is e
Systemic hypertension may be a complication of treating persistent pulmonary hypertension of the newborn (PPHN) by ECMO or from umbilical artery catheterization.

The answer is e
Systemic hypertension may be a complication of treating persistent pulmonary hypertension of the newborn (PPHN) by ECMO or from umbilical artery catheterization.

The answer is e
Although prematurity may cause delayed passage of meconium (NPO, immature colonic function), it is not associated with a higher incidence of meconium plugs.

The answer is d
Surgeons should be involved early to help in the management and to discuss indications for surgery, including evidence of perforation and failure to respond to medical
management. Placement of an abdominal drain with peritoneal lavage may also help in the management of a sick VLBW infant.

The answer is e
Postmature infants have a lower incidence of jaundice unless polycythemia is present. Other risk factors for jaundice include hemolysis, Gilbert disease, breast-feeding, prematurity, diabetic mother, bruising, intestinal obstruction, hypothyroidism, and diseases producing cholestatic disorders.

The answer is b
Until this test is done, it is unknown whether the infant has cholestatic or indirect hyperbilirubinemia. In this child, the total bilirubin was 20 mg/dL and the direct was 10
mg/dL. She had biliary atresia.

The answer is c
The Kleihauer-Betke test is performed on maternal blood and tests for the presence of fetal hemoglobin containing erythrocytes from a fetal-to-maternal transfusion. A low bilirubin suggests that there is no hemolysis, and a normal examination, except for tachycardia, suggests no internal blood loss. Fetal-to-maternal bleeding can be chronic or acute.

The answer is e
Recombinant human erythropoietin (rHuEpo) has not consistently demonstrated benefit. Indeed, in older patients, antibodies to erythropoietin may develop, which will bind to endogenous erythropoietin, thus producing a severe anemia. It may increase risk of retinopathy of prematurity

C. Both

C. Both

B. Rh

C. Both

D. Neither

B. Rh

B. Rh

D. Neither

Explanation: B. Rh

Explanation: B. Rh

Answer is b
The recipient twin develops polycythemia.
Additional risks for polycythemia include intrauterine growth retardation, postmature infants, Beckwith-Wiedemann syndrome, and being born at high altitude.

Answer is a
Because fluid intake and urine flow may be reduced in the first day or two after birth, an obstruction may be missed if only one ultrasound is performed. Therefore, it is recommended to repeat the ultrasound examination at 1 mo of age.

Answer is e
This infant has late neonatal onset tetany, which is usually due to ingesting cow’s milk with a very high phosphate content. However, some infants breast-fed by a mother at nutritional risk may develop hypocalcemia or, later in infancy, rickets. The patient responded to intravenous calcium
and was sent home on supplemental vitamin D. Infants with hypocalcemia often have only peripheral neuromuscular signs and appear alert during what appears to be a seizure.

Answer is b
Meconium drug testing has become the standard and reflects long-term drug misuse at a site of drug excretion and storage. Urine test results may vary, depending on the timing between drug intake and testing and the dose.

Answer is e
Periconception control and control during the early period of organogenesis help reduce the risk of
congenital malformations. Common anomalies affect the heart and musculoskeletal system.

Answer is c
This infant has cardiomyopathy associated with infants of diabetic mothers. This is a form of ventricular septal hypertrophy that can obstruct the left ventricular outflow tract and cause heart failure and poor cardiac output. Inotropic agents that increase the force of ventricular muscle contraction exacerbate the obstruction and are contraindicated

Answer is c
This constellation of findings suggests a chromosomal abnormality. Multiple malformations however may be of unknown etiology. Metabolic disorders rarely cause multiple malformations but may result in normal formation with subsequent changes, such as coarsening of the facial features
or microcephaly or macrocephaly.

Answer is d
Recurrence risks of what is traditionally thought to be a polygenetic disorder are between 3-5%. This is seen in congenital heart disease and other single-organ malformations without a positive family history.

Answer is a
Polyhydramnios suggests obstruction of the gastrointestinal tract or disorders of swallowing due to poor CNS function in utero. Contractions suggest poor motor activity
in the fetus or being fixed in one position (this is unlikely in the presence of polyhydramnios).

Answer is a
Most studies suggest that screening for group B Streptococcus in the mother and then selectively treating affected women will reduce the risk of neonatal sepsis.

Answer is c
Premature infants with respiratory distress syndrome (RDS) may look indistinguishable from infants with early-onset sepsis. Waiting for culture results in this situation would be dangerous, as the infant will die without therapy before the culture is reported positive. A lumbar puncture is not wise in this case, as the infant is hypotensive and hypoxic; the blood culture is the only needed initial culture. If the blood culture is positive, when the patient’s condition becomes more
stable, a lumbar puncture can be performed.

A. Rubinstein-Taybi syndrome
B. Trisomy 21
C. Williams syndrome
D. Pierre Robin syndrome
E. Prader-Willi syndrome
F. VATER syndrome
G. None of the above

Answer is : C. Williams syndrome

Answer is A. Rubinstein-Taybi syndrome

Answer is: E. Prader-Willi syndrome

Answer is: B. Trisomy 21

Answer is: D. Pierre Robin syndrome

Answer is: F. VATER syndrome

Answer is: F. VATER syndrome

Answer is: F. VATER syndrome

Answer is c:
One point in the Apgar score is taken off for color.

Answer is d
No treatment is necessary for the infant described in the question, assuming normal growth and
development.

Answer is e
Lumbar puncture is contraindicated in a child with intracranial hemorrhage

The answer is e

Answer is c

The answer is A
This infant has a case of severe vitamin K deficiency-hemorrhagic disease of the newborn. The next most likely diagnosis is child abuse; most infants in coma with retinal
hemorrhages have been shaken, and a skeletal survey thus is appropriate. The combination of home delivery (no vitamin K administered), breast-feeding (low vitamin K content), and the amoxicillin treatment that eliminated normal intestinal bacterial synthesis of vitamin K led to the tragic demise of an otherwise normal infant. Of all possible preventive measures, administration of vitamin K at birth would have been the most effective.

The answer is d
Hypothyroidism was confirmed by the late arrival of the newborn screening results, indicating high TSH and low T4 levels. Treatment with thyroxine improved the jaundice and the other signs. The hyperbilirubinemia is indirect (unconjugated). Crigler-Najjar syndrome is a possibility and is
either autosomal dominant or recessive (check the family history). However, there are signs other than jaundice that suggest another disease. Biliary atresia is always a concern in infants with delayed clearance of jaundice or worsening jaundice after 2 wk of life. The hyperbilirubinemia is
predominantly direct (conjugated). Galactosemia should be considered, especially in the presence of hypoglycemia, direct reacting jaundice, hepatomegaly, or ascites

The answer is e
Jaundice usually resolves in all infants with hyperbilirubinemia due to ABO incompatibility in the first week of life. Nonetheless, the hemolysis continues without evidence
of jaundice because the liver can now excrete the bilirubin load. Late-onset anemia must be watched for and treated with a packed red blood cell transfusion if the infant is symptomatic.
Hereditary spherocytosis is a possibility but is relatively rare. A thorough family history and examination of the child’s and parents’ blood smear are helpful (because most cases of
spherocytosis are inherited as an autosomal dominant trait). Sickle cell anemia hemolytic crisis is not encountered this early in life because a considerable amount of fetal hemoglobin remains; thus, there are few -chains to sickle.

A. CMV
B. Varicella
C. Enteroviruses
D. Rubella
E. Parvovirus
F. Toxoplasmosis
G. Trypanosoma cruzi

Answer: G. Trypanosoma cruzi

Answer: D. Rubella

Answer: A. CMV

Answer: B. Varicella

Answer: C. Enteroviruses

Answer: E. Parvovirus

Answer:F. Toxoplasmosis, (

A. Neonatal heart failure
B. Oligohydramnios
C. Spina bifida
D. Arthrogryposis
E. Facial-ear anomalies
F. Goiter

answer: E. Facial-ear anomalies

answer: A. Neonatal heart failure

answer: F. Goiter

answer: B. Oligohydramnios

answer: C. Spina bifida

answer: D. Arthrogryposis,

Answer is d
Klumpke paralysis involves injury to the 7th and 8th cervical nerves and the 1st thoracic nerve. It is usually unilateral, due to traction injury of the brachial plexus.

The answer is c
Transection of the spinal cord may occur in vertex and breech positions and may be noted with normal vertebral body anatomy. It would manifest as in this patient, and also with shock, hypothermia, and bowel and bladder dysfunction. With time, hypotonia resolves into hypertonia and hyperreflexia.

The answer is d
Infants of diabetic mothers (IDM) often experience hypocalcemia within 24-48 hr after birth, due in part to an attenuated parathyroid gland response to reduced calcium levels. The IDM also develops hypomagnesemia, which, if uncorrected, results in persistent hypocalcemia due to
the dependency of parathyroid function on magnesium.