1. An infant has the following findings at 5 minutes of life: pulse 130 bpm, cyanotic hands and feet, good muscle tone, and a strong cry and grimace. This infant Apgar score is:

A. 6B. 7C. 8D. 9E. 10

2. Jaundice is most likely to be physiologic in a term infant in which of the following situations:

A. Jaundice at 12 hours of ageB. Serum bilirubin level increasing less than 5 mg/dL/24 hours in the first 2-4

daysC. Direct serum bilirubin greater than 2 mg/dLD. Jaundice at 13 days of ageE. Serum bilirubin level 15 mg/dL at 7 days of age

3. Which of the following is most appropriate for treating hyperbilirubinemia (11.2 mg/dL) in a 3-week-old, breast-fed infant with normal growth and development?

A. PhototherapyB. Exchange transfusionC. PhenobarbitalD. Replace breast milk by formula for 48 hoursE. None of the above

4. Newborns are obligate nasal breathers for the first few months after birth. Of the following, the most common cause of neonatal nasal obstruction is:

A. Choanal atresiaB. Craniofacial malformationsC. Intranasal encephaloceleD. Lacrymal cystE. Mucosal inflammation

5. A term newborn appears dusky at rest, but becomes pink with crying. Attempts to pass a catheter through each nostril have failed. Physical examination of the infant reveals other congenital anomalies. Of the following, the most likely clinical syndrome in this infant is:A. Apert syndromeB. CHARGE associationC. Crouzon syndromeD. Pfeiffer syndromeE. Treacher-Collins syndrome

6. You are called to the delivery of a boy at 42 weeks’ GA with thick meconium-stained fluid and type II decelerations. The obstetrician rapidly delivers the baby and hands him to you. The boy hypotonic, cyanotic, apneic, and bradycardic. The most appropriate action is to

A. Stimulate the infant to breathB. Administer epinephrineC. Provide positive-pressure bag-and-mask ventilationD. Intubate and provide positive-pressure ventilationE. Intubate and apply negative-pressure suction

7. Normal findings in a newborn baby includes all of the following ExceptA. A mongolian blue spotB. A strawberry nevusC. Vaginal blood lossD. Lanugo hairE. Erythema toxicum

8. A term male newborn has severe intrauterine growth restriction, triangular face with broad forehead and pointed chin, leg length asymmetry, fifth finger clinodactyly, and cryptorchidism. Of the following, the most likely genomic imprinting disorder in this infant is:

A. Albright hereditary osteodystrophyB. Angelman syndromeC. Beckwith-Wiedemann syndromeD. Prader-Willi syndromeE. Silver-Russell syndrome

9. Regarding the head of a newborn infant (multiple choices)A. A cephalohematoma will resolve within the first 24 hours of lifeB. Caput is due to edema of the presenting part of the headC. A cephalohematoma is due to bleeding into the skinD. Overlapping of the skull bones is a normal findingE. A cephalohematoma should be drained

10. In contrast to “classic” bronchopulmonary dysplasia (BPD), the “new” BPD in the more contemporary clinical setting is characterized by different histopathologic findings on examination of the lung tissue. The most striking abnormality in the lungs of infants who have new BPD is A. Decrease in alveolar septationB. Diffuse leukocytic infiltrationC. Epithelial squamous metaplasiaD. Hypertrophy of airway smooth muscleE. Lung parenchymal fibrosis

11.A 1700-g infant is born at 36 weeks’ gestation complicated by severe oligohydramnios. The Apgar scores are 3 and 5 at 1 and 5 min respectively.

The baby required intubation as part of the resuscitation and continued MV to improve the ABGs. At 1 hour of age, the baby showed acute deterioration with cyanosis, bradycardia, and hypotension. The most likely diagnosis of this acute change is:

A. Patent ductus arteriosusB. Intraventricular hemorrhageC. HypoglycemiaD. PneumothoraxE. Severe respiratory distress syndrome

12.A 14-day-old preterm infant, whose birthweight was 980 g at an estimated gestational age of 27 weeks, is receiving full enteral feedings of fortified human milk. The infant is breathing spontaneously in room air and has no evidence of cardiac, renal, or intracranial abnormalities. Of the following, the most desirable goal of enteral nutrition in this infant is to achieve:

A. Fetal rate of body weight gainB. Fetal rate of crown-heel length gainC. Fetal rate of head circumference gainD. Intrauterine body compositionE. Normal blood urea concentration

13.A 3600-g, breast-fed female, 42 weeks’ GA, is noted to have persistent hyperbilirubinemia at 2 weeks of age. On examination, the infant has not gained weight since birth and has decreased tone, an umbilical hernia, and an anterior fontanel measuring 4 x 6 cm. The most likely diagnosis is:

A. Crigler-Najjar syndromeB. Gilbert diseaseC. Biliary atresiaD. HypothyroidismE. Galactosemia

14.Approximately 10% of infants born with congenital diaphragmatic hernia (CDH) have significant heart defects. The most common congenital heart defect associated with CDH is:

A. Aortic arch obstructionB. Tetralogy of FallotC. Total anomalous pulmonary venous returnD. Transposition of the great arteriesE. Ventricular septal defect

15.Contemporary management of gastroschisis is associated with a 10% to 25% incidence of postoperative intestinal and related complications. The most common postoperative complication of surgical repair of gastroschisis is:

A. Abdominal compartment syndrome

B. Enterocutaneous fistulaC. Necrotizing enterocolitisD. Renal ischemiaE. Short bowel syndrome

16.The following predispose a baby to be small for gestational age (multiple choices):

A. Placental insufficiencyB. Twin pregnancyC. Congenital infectionD. Maternal diabetesE. Alcohol consumption

17.The twin-twin transfusion is unique to twins that are:A. Dizygotic, dichorionicB. Dizygotic, monoamnionicC. Dizygotic, monochorionicD. Monozygotic, dichorionicE. Monozygotic, monochorionic

18.Respiratory distress syndrome is exacerbated by the following ExceptA. HypothermiaB. AcidosisC. HypoxiaD. Meconium aspirationE. Withholding enteral feeds

19.The most important determinant of endotracheal tube resistance is:A. Airflow velocityB. Biofilm coatingC. Gas densityD. Tube curvatureE. Tube diameter

20.Bilirubin conjugated in the liver and secreted into bile can be deconjugated in the gut, then reabsorbed into the bloodstream, resulting in enterohepatic bilirubin circulation. The enzyme most responsible for contributing to enterohepatic bilirubin circulation is:

A. Beta-glucuronidaseB. Bilirubin hydrogenaseC. Bilirubin oxidaseD. Biliverdin reductaseE. Uridine diphosphoglucuronate glucuronosyltransferase

21. Transient tachypnea of the newborn (multiple choices)A. occurs in 15% of full-term babies

B. can be diagnosed if onset occurs up to 48 hours post deliveryC. is caused by delayed absorption of fetal lung fluidD. is more common in babies delivered by cesarean sectionE. cannot be clearly differentiated from early pneumonia

21.Polycythemia in the neonatal period is associated with the following ExceptA. Trisomy 21B. The donor twin in a twin-twin transfusionC. Maternal diabetesD. Intrauterine growth restrictionE. Respiratory distress

22.When a newborn fails to achieve or sustain the normal decrease in pulmonary vascular resistance at birth, the result is hypoxemic respiratory failure or persistent pulmonary hypertension of the newborn (PPHN). Of the following, the mediator most responsible for normal pulmonary vascular transition at birth is

A. Asymmetric dimethylarginine.B. Endothelin-1C. LeukotrieneD. Nitric oxideE. Thromboxane

23.Harlequin color changes is a sign of:A. Congenital ichthyosisB. TORCH infectionC. ErythrodermaD. Normal physiologyE. Spinal cord trauma

24.The risk of developing necrotizing enterocolitis is increased in (multiple choices)

A. Breast-fed babiesB. Asphyxiated babiesC. Premature babiesD. Infants who have the umbilical artery catheterizedE. Infants who have had no milk feeds

25.A baby with Apgar scores of 1 and 2 at 1 and 5 minutes, respectively, appears hyperalert and has hyperactive deep tendon reflexes and mydriasis. The most likely diagnosis is:

A. Stage I hypoxic-ischemic encephalopathyB. Stage II hypoxic-ischemic encephalopathyC. Stage III hypoxic-ischemic encephalopathyD. KernicterusE. Intraventricular hemorrhage

26.The ventilator variable most influential in avoiding atelectasis in mechanically ventilated neonates is:

A. Inspiratory timeB. Peak inspiratory pressureC. Positive end-expiratory pressureD. Tidal volumeE. Ventilator rate

27.The most critical determinant of ventilator-induced lung injury in preterm neonates is excessive:

A. Airway pressureB. Gas flowC. Oxygen concentrationD. Tidal volumeE. Time constant

28.The most critical factor in the development of respiratory distress syndrome in the preterm neonate is:

A. Immature composition and biophysical function of surfactantB. Leaking epithelium/endothelium barrier from lung injuryC. Low lung gas volume with susceptibility to overdistentionD. Low surfactant lipid pool sizeE. Saccular versus alveolar stage of lung development

29.A severely hydropic infant is about to be delivered. Complications to be prepared for include all of the following Except

A. Pulmonary hypoplasiaB. Abdominal ascitesC. PolycythemiaD. Heart failureE. Laryngeal edema

30.The distribution of surfactant in the lungs is most efficient when surfactant is administered:

A. After a period of mechanical ventilationB. As an aerosolized preparationC. At a slow rate of infusionD. At birth in the presence of fetal lung fluidE. Using a smaller volume of the drug31.Neural tube defects:A. Occurs with frequency of 2 per 10,000 birthsB. Have a genetic predispositionC. Result from abnormal development of the neural tube at 3-4 months’

gestationD. Can be detected antenatally by low maternal serum alpha-fetoprotein levels

E. Are treated with folic acid

32. Neonatal hypercalcemia is associated with all of the following Except:A. Williams syndromeB. Subcutaneous fat necrosisC. Blue diaper syndromeD. Familial hypercalciuric hypercalcemiaE. Primary hyperparathyroidism

33 The single greatest risk factor for necrotizing enterocolitis is:A. PrematurityB. Patent ductus arteriosusC. Indomethacin therapyD. PolycythemiaE. Hypoxic-ischemic insult

34. Incidence of respiratory distress syndrome increases with all of the following ExceptA. PrematurityB. Maternal diabetesC. Black raceD. Male genderE. Cesarean section delivery

35. The most prevalent clinical manifestation of late-onset group B Streptococcus disease is:A. ArthritisB. CellulitisC. MeningitisD. OsteomyelitisE. Pneumonia