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PART XII The Fetus and the Neonatal Infant

QUESTIONS
ZUHAIR ALMUSAWI
1. Major cause of neonatal mortality in full-term newborn is
A. respiratory distress syndrome
B. necrotizing enterocolitis
C. bronchopulmonary dysplasia (BPD)
D. congenital anomalies
E. intraventricular hemorrhage

2. Generalized edema of the newborn may occur in the following conditions EXCEPT
A. prematurity
B. nonimmune hydrops
C. Turner syndrome
D. congenital nephrosis
E. Hurler syndrome

3. Soft areas in the occipital region suggest the irregular calcification and wormian bone
formation usually associated with the following conditions EXCEPT
A. osteogenesis imperfecta
B. craniosynostosis
C. cleidocranial dysostosis
D. cretinism
E. Down syndrome

4. The following disorders are associated with a large anterior fontanel EXCEPT
A. congenital rubella syndrome
B. hypophosphatasia
C. Apert syndrome
D. Russell-Silver syndrome
E. vitamin A deficiency

5. Leukokoria (white pupillary reflex) in newborn infant suggests the following


disorders EXCEPT
A. cataracts
B. tumor
C. congenital glaucoma
D. chorioretinitis
E. retinopathy of prematurity

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6. A single umbilical artery in a newborn infant increases the risk for
A. meningomyelocele
B. occult renal anomaly
C. omphalocele
D. gastroschisis
E. omphalitis

7. The following drugs can be given with caution to breast-feeding mother


A. psychotropic drugs
B. amphetamines
C. bromocriptine
D. chloramphenicol
E. methimazole

8. Of the following, the condition which is associated with polyhydramnios is


A. renal agenesis (Potter syndrome)
B. Prune-belly syndrome
C. pulmonary hypoplasia
D. intestinal pseudo-obstruction
E. diaphragmatic hernia

9. Low maternal serum α-fetoprotein (MSAFP) is associated with


A. open neural tube defects
B. trisomy 21
C. gastroschisis
D. omphalocele
E. congenital nephrosis

10. A delay in fetal pulmonary maturation may be associated with


A. hydrops fetalis
B. severe premature separation of the placenta
C. premature rupture of the fetal membranes
D. narcotic addiction
E. maternal hypertensive and renal vascular disease

11. True umbilical cord knots are seen in approximately 1% of births and are associated
with the following conditions EXCEPT
A. short cord
B. small fetal size
C. polyhydramnios
D. monoamniotic twinning
E. fetal demise

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12. Regarding multiple gestation pregnancies, the following are true EXCEPT
A. the reported incidence of spontaneous twinning is lowest in the Asian races
B. triplets are estimated to occur in 1 in 862 pregnancies in the USA
C. the incidence of monozygotic twins is unaffected by racial or familial factors
D. the overall incidence of multifetal gestation is unchanged
E. polyovular pregnancies are more frequent beyond the 2nd pregnancy

13. One of the following is a common neonatal respiratory problem associated with
premature infants
A. bronchopulmonary dysplasia
B. apnea
C. congenital pneumonia
D. pneumothorax
E. pneumomediastinum

14. One of the following drugs may cause pyloric stenosis if administered to a
premature infant
A. intravenous vitamin E
B. indomethacin
C. enteric gentamicin
D. prostaglandins
E. dexamethasone

15. The following factors pose a risk for poor academic performance of premature
infants EXCEPT
A. birthweight below 750 g
B. periventricular leukomalacia
C. antenatal exposure to magnesium sulfate
D. bronchopulmonary dysplasia
E. posthemorrhagic hydrocephalus

16. The incidence of all the following are increased in large for gestational age
newborn EXCEPT
A. cephalohematoma
B. hypocalcemia
C. hypoglycemia
D. congenital heart disease
E. developmental retardation

17. Sudden onset of hypotension in a very low birthweight (VLBW) infant suggests
A. pneumothorax
B. necrotizing enterocolitis

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C. bacterial sepsis
D. myocarditis
E. hypoglycemia

18. Seizures beginning in the delivery room or shortly thereafter may be due to
A. hypoxic-ischemic encephalopathy
B. unintentional injection of maternal local anesthetic into the fetus
C. intracranial hemorrhage
D. cerebral anomaly
E. hypoglycemia

19. After severe birth asphyxia, infants may have motor automatisms characterized by
A. abscence of oral-buccal-lingual movements
B. time-synchronized electroencephalographic discharges
C. significant cortical epileptic activity
D. good response to anticonvulsant therapy
E. a poor prognosis

20. Vomiting in the neonatal period is usually due to


A. pyloric stenosis
B. milk allergy
C. overfeeding
D. stress ulcer
E. an inborn error of metabolism

21. Regarding cephalohematoma, all the following are true EXCEPT


A. is a subperiosteal hemorrhage
B. it may extend across the midline and across suture lines
C. occur in 1-2% of live births
D. no discoloration of the overlying scalp
E. an underlying skull fracture may be associated with 10-25% of cases

22. Regarding intraventricular hemorrhage (IVH) of prematurity, the following are true
EXCEPT
A. it usually develops spontaneously
B. MRI is the preferred imaging technique for screening IVH
C. prophylactic administration of low-dose indomethacin reduces the incidence of
severe IVH
D. majority of patients with IVH have no clinical symptoms
E. it may rarely manifest at birth

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23. Early postnatal exposure to dexamethasone, within the 1st wk of life, in VLBW
infants, is associated with the following EXCEPT
A. metabolic derangements
B. poor growth
C. increased risk for sepsis
D. increased risk of spontaneous bowel perforation
E. cerebral palsy

24. The following are true regarding apnea EXCEPT


A. apnea is a common problem in preterm infants
B. in term infants, apnea is always worrisome
C. obstructive apnea is characterized by absence of airflow but persistent chest
wall motion
D. serious apnea is defined as cessation of breathing for longer than 20 sec
E. bradycardia follows the apnea by 1-2 sec in more than 95% of cases and is most
often nodal

25. The risk for development of respiratory distress syndrome RDS increases with the
following conditions EXCEPT
A. prolonged rupture of membranes
B. maternal diabetes
C. multiple births
D. precipitous delivery
E. cold stress

26. The following are true regarding respiratory distress syndrome (RDS) EXCEPT
A. signs of RDS usually appear within minutes of birth
B. in most cases, the symptoms and signs reach a peak within 7days
C. apnea and irregular respirations are ominous signs
D. improvement is often heralded by spontaneous diuresis
E. mixed respiratory-metabolic acidosis may be seen

27. One of the following conditions can mimic RDS both clinically and radiographically
A. persistent pulmonary hypertension
B. meconium aspiration syndrome
C. total anomalous pulmonary venous return
D. pulmonary lymphangiectasia
E. lobar emphysema

28. Administration of antenatal corticosteroids to women between 24 and 34 wk of


gestation significantly reduces the following EXCEPT
A. incidence and mortality of RDS

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B. postnatal growth
C. the overall neonatal mortality
D. need for and duration of ventilatory support
E. incidence of severe intraventricular hemorrhage

29. The basic defect requiring treatment in RDS is


A. metabolic acidosis
B. circulatory insufficiency
C. hypothermia
D. inadequate pulmonary exchange of oxygen and carbon dioxide
E. electrolytes disturbance

30. Regarding CPAP, the following are true EXCEPT


A. its prevents collapse of surfactant-deficient alveoli
B. its indicated, if oxygen saturation cannot be kept > 95%
C. its improves functional residual capacity FRC
D. its improves ventilation-perfusion matching
E. its reduces ventilatory needs

31. Prophylactic and rescue administrations of synthetic and natural surfactants have
the following advantages EXCEPT
A. reduces adverse outcomes
B. reduces neonatal mortality
C. decreses the risk for pneumothorax
D. decreses the risk for pulmonary interstitial emphysema
E. reduces bronco-pulmonary dysplasia BPD rates

32. Vitamin A supplementation given largely to infants < 1,000 g resulted in all the
following EXCEPT
A. a decrease in death
B. a decrease in bronco-pulmonary dysplasia BPD at 36 wk
C. less nosocomial sepsis
D. less retinopathy of prematurity
E. decreases the need for extracorporeal membrane oxygenation (ECMO)

33. Alkali therapy for the treatment of metabolic acidosis in RDS may result in all the
following EXCEPT
A. skin slough from infiltration
B. increased serum osmolarity
C. hypernatremia
D. hypocalcemia
E. hyperkalemia

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34. Contributary factors for the development of broncho-pulmonary dysplasia (BPD)
include the following EXCEPT
A. immaturity
B. dehydration during the 1st days of life
C. chorioamnionitis
D. symptomatic PDA
E. malnutrition

35. Methylxanthines for the treatment of broncho-pulmonary dysplasia (BPD) have the
following effects EXCEPT
A. increase respiratory drive
B. decrease apnea
C. improve diaphragmatic contractility
D. increase pulmonary vascular resistance PVR
E. diuretic effects

36. One of the following is FALSE in the chest radiograph of transient tachypnea of the
newborn (TTN)
A. prominent pulmonary vascular markings
B. fluid in the intralobar fissures
C. overaeration
D. flat diaphragms
E. diffuse reticulogranular pattern

37. The following are true regarding meconium aspiration syndrome (MAS) EXCEPT
A. (MAS) develops in 5% of meconium-stained infants
B. 30% require mechanical ventilation
C. 3-5% die
D. overdistention of the chest may be prominent
E. usually occurs in preterm or near-term infants

38. The following are predisposing factors for persistent pulmonary hypertension of the
newborn (PPHN) EXCEPT
A. anemia
B. meconium aspiration syndrome
C. early-onset sepsis
D. hypoglycemia
E. birth asphyxia

39. Associated anomalies have been reported in up to 30% of congenital diaphragmatic


hernia cases; these include the following EXCEPT
A. pulmonary hypoplasia

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B. CNS lesions
C. esophageal atresia
D. omphalocele
E. cardiovascular lesions

40. Congenital diaphragmatic hernia (CDH) can be diagnosed on prenatal


ultrasonography (between 16 and 24 wk of gestation) in > 50% of cases.
Findings on ultrasonography may include the following EXCEPT
A. chest mass
B. mediastinal shift
C. gastric bubble
D. olighydramnios
E. a liver in the thoracic cavity

41. A poor prognostic sign of congenital diaphragmatic hernia (CDH) is


A. grunting
B. use of accessory muscles
C. early respiratory distress, within 6 hr of life
D. cyanosis
E. scaphoid abdomen

42. Eventration of the diaphragm may be associated with the following EXCEPT
A. pulmonary hypoplasia
B. pulmonary sequestration
C. congenital heart disease
D. chromosomal trisomies
E. recurrent infections

43. All the following are true, regarding pathology of neonatal necrotizing enterocolitis
(NEC) EXCEPT
A. in fatal cases, gangrene may extend from the stomach to the rectum
B. the greatest risk factor for NEC is prematurity
C. in most situations, a pathogen is identified
D. NEC is much less common in infants fed human milk
E. NEC in term infants is often a “secondary” disease

44. Absolute indication for surgery in neonatal necrotizing enterocolitis (NEC) include
A. positive result of abdominal paracentesis
B. failure of medical management
C. a single fixed bowel loop on radiographs
D. abdominal wall erythema
E. a palpable mass

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45. The following factors are associated with decreased risk of significant jaundice
EXCEPT
A. gestational age ≥41 wk
B. male gender
C. exclusive bottle-feeding
D. black race
E. discharge from hospital after 72 hr

46. Jaundice, consisting of either indirect or direct bilirubin that is present at birth or
appears within the 1st 24 hr of life may be due to all the following EXCEPT
A. erythroblastosis fetalis
B. Crigler-Najjar syndrome
C. concealed hemorrhage
D. sepsis
E. congenital infections

47. A search to determine the cause of jaundice should be made in all the following
conditions EXCEPT
A. if it appears in the first 24-36 hr of life
B. if serum bilirubin is rising at a rate faster than 5 mg/dL/24 hr
C. if serum bilirubin is >12 mg/dL in a full-term infant
D. if jaundice persists after 10-14 days of life
E. if direct bilirubin fraction is >1 mg/dL at any time

48. Regarding breast milk jaundice, the following are true EXCEPT
A. it develops in an estimated 2% of breast-fed term infants
B. maximal unconjugated bilirubin concentrations as high as 10-30 mg/dL reached
during the 2nd-3rd week
C. jaundice may persist for 3-10 wk
D. phototherapy may be of benefit
E. kernicterus never occur

49. The therapeutic effect of phototherapy depends on the following factors EXCEPT
A. distance between the lights and the infant
B. skin color
C. surface area of exposed skin
D. rate of hemolysis
E. in vivo metabolism and excretion of bilirubin

50. Early ultrasonographic signs of hydrops include


A. double–bowel wall sign (bowel edema)
B. polyhydramnios

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C. ascites
D. pleural effusions
E. scalp edema

51. The blood smear of infant with hemolytic disease of the newborn (erythroblastosis
fetalis) typically shows the following EXCEPT
A. polychromasia
B. increasedreticulocyte count
C. a marked increase in nucleated RBCs
D. thrombocytopenia
E. low white blood cell count

52. One of the following is FALSE in hemolytic disease of the newborn caused by blood
group A and B incompatibility
A. ABO incompatibility occurs in 20-25% of pregnancies
B. A1 is more antigenic than A2
C. natural antibodies against A and B factors are usually IgM antibodies that do
not cross the placenta
D. isoimmune hemolytic disease may be found in first-born infants irrespective of
infant blood group
E. most cases are mild

53. One of the following is FALSE in hemolytic disease of the newborn caused by Rh
incompatibility
A. infant is Rh Positive (D, sometimes C)
B. never occur in first-born infant
C. maternal antibody titers can help predict the severity of fetal disease
D. severe anemia is frequent
E. stillbirth/hydrops is frequent

54. One of the following is FALSE in plethora in the newborn infant (polycythemia)
A. polycythemia is defined as a central Hct of 65% or higher
B. peripheral (heelstick) Hct and Coulter counter results values are higher than
central values
C. the incidence of neonatal polycythemia is 8% in small for gestational age (SGA)
infants
D. many affected infants are asymptomatic
E. neonatal RBCs have decreased deformability and filterability, which predispose
to stasis in the microcirculation

55. Regarding hemorrhagic disease of the newborn, all the following are true EXCEPT
A. age of onset is usually in 2-7 days old infants

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B. more frequent in breast-fed than in formula-fed infants
C. bleeding time is normal
D. oral vitamin K is as effective as intramuscular route in the prevention and
treatment of the disease
E. prothrombin time (PT), and partial thromboplastin time (PTT) are prolonged

56. Apt test is useful for diagnosis of


A. disseminated intravascular coagulopathy
B. swallowed blood syndrome
C. hemorrhagic disease of the newborn
D. neonatal thrombocytopenic purpura
E. congenital deficiency of factor VII

57. The normal cord at term is 55 cm long. Abnormally short cords are associated with
the following EXCEPT
A. fetal hypotonia
B. wrapping around fetal parts
C. uterine constraint
D. oligohydramnios
E. increased risk for complications of labor and delivery for both mother and
infant

58. Surgical repair of umbilical hernia is advised in the following conditions EXCEPT
A. if hernia persists to the age of 4-5 yr
B. causes symptoms
C. hernias that appear before the age of 6 mo
D. becomes strangulated
E. becomes progressively larger after the age of 1-2 yr

59. Generalized edema may be seen in the neonatal period with the following
conditions EXCEPT
A. congenital nephrosis
B. Turner syndrome
C. Hurler syndrome
D. hydrops fetalis
E. offspring of diabetic mothers

60. Diabetic mothers have a high incidence of all the following EXCEPT
A. oligohydramnios
B. preeclampsia
C. pyelonephritis
D. preterm labor

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E. chronic hypertension

61. In Infants of diabetic mothers, the following are true EXCEPT


A. hypoglycemia develops in about 25-50% of infants of diabetic mothers
B. increased weight of the placenta and all infant organs
C. heart failure occurs in 5-10% of infants of diabetic mothers
D. the incidence of congenital anomalies is increased threefold in infants of
diabetic mothers
E. lumbosacral agenesis is one of most common congenital anomalies

62. The following definitions are true EXCEPT


A. a syndrome is defined as a pattern of multiple abnormalities that are related by
pathophysiology and result from a common defined etiology
B. dysplasia sequence is a poor organization of cells into tissues or organs
C. an association refers to a nonrandom collection of malformations in which
there is an unclear relationship among the malformations
D. disruption sequence is a mechanical (uterine) forces that alter structure of
intrinsically normal tissue
E. malformation sequence is a single, local tissue morphogenesis abnormality that
produces a chain of subsequent defects

63. Chromosomal causes of congenital malformations include


A. Prader-Willi syndrome
B. X-linked hydrocephalus
C. Achondroplasia
D. Treacher Collins syndrome
E. Apert disease

64. One of the following definitions is FALSE


A. brachydactyly=A condition of having short digits
B. postaxial polydactyly=Extra finger or toe present on the medial side of the hand
or foot
C. clinodactyly=A medial or lateral curving of the fingers
D. camptodactyly=Permanent flexion of one or more fingers
E. syndactyly=Incomplete separation of the fingers

65. Intrauterine transplacental infections of significance to the fetus and/or newborn


include the following EXCEPT
A. hepatitis B virus (HBV)
B. rubella
C. toxoplasmosis
D. Parvovirus B19

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E. varicella

66. Agents that commonly cause nosocomialneonatal infection are


A. group B streptococci GBS
B. coagulase-negative staphylococci
C. proteus
D. gonococci
E. chlamydiae

67. One of the following is FALSE regarding intrapartum antibiotics


A. reduce vertical transmission of GBS
B. lessen neonatal morbidity after preterm rupture of membranes
C. prevent perinatal transmission of GBS
D. reduce the rates of late-onset GBS disease
E. has no effect on the rates of infection with non-GBS pathogens

68. In a cohort of 6,215 VLBW infants in the NICHD Neonatal Research Network, gram-
positive agents were associated with which percent of cases of late-onset sepsis
A. 90%
B. 70%
C. 50%
D. 30%
E. 10%

69. Osteitis may be a feature of one of the following transplacental infections


A. cytomegalovirus
B. herpes simplex virus
C. varicella-zoster virus
D. rubella
E. T. gondii

70. Intracranial calcification may be a feature of one of the following transplacental


infections
A. cytomegalovirus
B. herpes simplex virus
C. varicella-zoster virus
D. syphilis
E. rubella

71. Limb hypoplasia may be a feature of the following transplacental infections


A. cytomegalovirus
B. herpes simplex virus

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C. varicella-zoster virus
D. HIV
E. rubella

72. Common respiratory tract manifestations of neonatal bacterial infections is


A. ethmoiditis
B. otitis media
C. mastoiditis
D. retropharyngeal cellulitis
E. empyema

73. Common skin and soft tissue manifestations of neonatal bacterial infections are
A. facial cellulitis
B. scalp abscess
C. fasciitis
D. breast abscess
E. omphalitis

74. One of the following is not included in the definition of the systemic inflammatory
response (SIRS) in neonates and pediatric patients
A. temperature instability
B. abnormal white blood cell (WBC) count
C. respiratory dysfunction
D. cardiac dysfunction
E. perfusion abnormalities

75. Neonatal sepsis case fatality rate is highest for


A. Staphylococcus—coagulase negative
B. Group B streptococcus
C. Escherichia coli
D. Pseudomonas
E. Candida albicans

76. Persistence of the umbilical cord beyond which time should prompt consideration
of an underlying abnormality
A. 10 days
B. 20 days
C. 30 days
D. 40 days
E. 50 days

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77. The MOST important risk factor for necrotizing enterocolitis (NEC) in preterm
infants is
A. delivery by cesarean section
B. breast feeding
C. apgar score
D. exposure to glucocorticoids during the first week of life
E. gestational age and birth weight

78. Pneumatosis intestinalis is pathognomonic for


A. Hirschsprung's disease
B. necrotizing enterocolitis (NEC)
C. pseudomembranous enterocolitis
D. neonatal ulcerative colitis
E. meconium ileus

79. The following factors suggest hemolytic disease as a cause of jaundice in the
newborn EXCEPT
A. bilirubin rise of >0.5 mg/dL/h
B. reticulocytosis >5% at birth
C. onset of jaundice before 24 hours of age
D. significant decrease in hemoglobin
E. failure of phototherapy to lower serum bilirubin levels

80. Which common sugar does the clinitest screen not detect?
A. glucose
B. fructose
C. galactose
D. sucrose
E. lactose

81. The following are manifestations of hypocalcemia in the neonate EXCEPT


A. jitteriness
B. seizures
C. carpopedal spasm
D. high-pitched cry
E. laryngospasm

82. When screening for intraventricular hemorrhage IVH, the best time to perform an
ultrasound is
A. first day of life
B. second day of life
C. third day of life

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D. fourth day of life
E. fifth day of life

83. The antenatal administration of magnesium sulfate before preterm delivery has
been suggested to
A. reduce rates of necrotizing enterocolitis
B. decrease the risk of cerebral palsy in surviving infants
C. improve overall survival
D. decrease respiratory distress syndrome in premature infants
E. reduce rates of intraventricular hemorrhage

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PART XII The Fetus and the Neonatal Infant
ANSWERS
ZUHAIR ALMUSAWI
1.(D). Severe immaturity, respiratory distress syndrome, intraventricular hemorrhage,
infection, necrotizing enterocolitis, and bronchopulmonary dysplasia (BPD) are major
causes of mortality in preterms.
2.(C). Localized edema suggests a congenital malformation of the lymphatic system;
when confined to one or more extremities of a female infant; it may be the initial sign
of Turner syndrome.
3.(B). Persistently small fontanels suggest microcephaly, craniosynostosis, and
congenital hyperthyroidism.
4.(E). Vitamin D deficiency rickets is associated with a large anterior fontanel.
5.(C). A cornea >1cm in diameter in a term infant (with photophobia and tearing)
suggests congenital glaucoma and requires prompt ophthalmologic consultation.
6.(B). A single umbilical artery increases the risk for an occult renal anomaly.
7.(A). Amphetamines, bromocriptine, chloramphenicol, and methimazole are
contraindicated in breast-feeding mother.
8.(E). Renal agenesis (Potter syndrome), Prune-belly syndrome, pulmonaryhypoplasia,
and intestinal pseudo-obstruction are associated with oligohydramnios.
9.(B). Second-trimester screening (15-18 wk) of maternal serum α-fetoprotein (MSAFP)
values is used to screen for open neural tube defects. About 90% of affected
pregnancies can be detected by an elevated MSAFP value. Gastroschisis, omphalocele,
congenital nephrosis, twins, and other abnormal conditions can also be identified. Low
MSAFP is associated with incorrect gestational age estimates, trisomy 18 or 21, and
intrauterine growth restriction.
10.(A). Earlier lung maturation may occur in the presence of severe premature
separation of the placenta, premature rupture of the fetal membranes, narcotic
addiction, or maternal hypertensive and renal vascular disease. A delay in pulmonary
maturation may be associated with hydrops fetalis or maternal diabetes without
vascular disease.
11.(A). Short cords and noncoiled cords occur with chromosome abnormalities and
omphalocele.
12.(D). Although the incidence of spontaneous multifetal gestation has been stable
over the years, the overall incidence of multifetal gestation is increasing as a result of
treatment of infertility with ovarian stimulants (clomiphene, gonadotropins) and in
vitro fertilization. Twins account for about 2.5% of births but about 20% of very low
birthweight (VLBW) infants.
13.(B). Respiratory distress syndrome (hyaline membrane disease) and apnea are
common neonatal respiratory problem associated with premature infants.

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14.(D). Prostaglandins and erythromycin may cause pyloric stenosis if administered to a
premature infant.
15.(C). Antenatal exposure to magnesium sulfate may have neuroprotective effects and
may reduce the incidence of cerebral palsy in high-risk neonates.
16.(B). Large for gestational age LGA infants, regardless of their gestational age, have a
higher incidence of birth injuries, such as cervical and brachial plexus injuries, phrenic
nerve damage with paralysis of the diaphragm, fractured clavicles, cephalohematomas,
subdural hematomas, and ecchymoses of the head and face. LGA infants are also at
increased risk for hypoglycemia and polycythemia. The incidence of congenital
anomalies, particularly congenital heart disease, is also higher in LGA infants than in
term infants of normal weight. Intellectual and developmental retardation is
statistically more common in high birthweight term and preterm infants than in babies
of appropriate weight for gestational age
17.(A). Sudden onset of hypotension in a very low birthweight (VLBW) infant suggests
pneumothorax, intraventricular hemorrhage, or subcapsular hepatic hematoma.
18.(B). Seizures beginning in the delivery room or shortly thereafter may be due to the
unintentional injection of maternal local anesthetic into the fetus. Convulsions may
also result from hyponatremia and water intoxication in the infant after the
administration of large amounts of hypotonic fluid to the mother shortly before and
during delivery.
19.(E). After severe birth asphyxia, infants may have motor automatisms characterized
by oral-buccal-lingual movements, rotary limb activities (rowing, pedaling, and
swimming), tonic posturing, or myoclonus. These motor activities are not usually
accompanied by time-synchronized electroencephalographic discharges, may not
signify cortical epileptic activity, respond poorly to anticonvulsant therapy, and are
associated with a poor prognosis. Such automatisms may represent cortical depression
that produces a brainstem release phenomenon or subcortical seizures.
20.(C). Vomiting during the 1st day of life suggests obstruction in the upper digestive
tract or increased intracranial pressure. Roentgenographic studies are indicated when
obstruction is suspected. Vomiting may also be a nonspecific symptom of an illness
such as septicemia. It is a common manifestation of overfeeding, inexperienced feeding
technique, or normal reflux and is rarely due to pyloric stenosis, milk allergy, duodenal
ulcer, stress ulcer, an inborn error of metabolism (hyperammonemia, metabolic
acidosis), or adrenal insufficiency.
21.(B). Cephalohematoma is a subperiosteal hemorrhage, hence always limited to the
surface of one cranial bone, while caput succedaneum is a diffuse, sometimes
ecchymotic, edematous swelling of the soft tissues of the scalp involving the area
presenting during vertex delivery. It may extend across the midline and across suture
lines.
22.(B). Ultrasonography is the preferred imaging technique for screening because it is
noninvasive, portable, reproducible, and sensitive and specific for detection of IVH.

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23.(E). Infants exposed to postnatal steroids after the 1st wk of life have an increased
risk of cerebral palsy and developmental delay.
24.(E). Bradycardia follows the apnea by 1-2 sec in more than 95% of cases and is most
often sinus, but on occasion it can be nodal. Vagal responses and, rarely, heart block
are causes of bradycardia without apnea.
25.(A). The risk of RDS is reduced in pregnancies with chronic or pregnancy-associated
hypertension, maternal heroin use, prolonged rupture of membranes, and antenatal
corticosteroid prophylaxis.
26.(B). In most cases, the symptoms and signs reach a peak within 3 days, after which
improvement is gradual.
27.(C). In the differential diagnosis, early-onset sepsis may be indistinguishable from
RDS. In pneumonia manifested at birth, the chest roentgenogram may be identical to
that for RDS. Maternal group B streptococcal colonization, identification of organisms
on gram staining of gastric or tracheal aspirates or a buffy coat smear, and/or the
presence of marked neutropenia may suggest the diagnosis of early-onset sepsis.
Cyanotic heart disease (total anomalous pulmonary venous return) can also mimic RDS
both clinically and radiographically. Echocardiography with color-flow imaging should
be performed in infants who show no response to surfactant replacement, to rule out
cyanotic congenital heart disease as well as ascertain patency of the ductus arteriosus
and assess pulmonary vascular resistance (PVR).
28.(B). Postnatal growth is not adversely affected. Antenatal steroids do not increase
the risk of maternal death, chorioamnionitis, or puerperal sepsis.
29.(D). The basic defect requiring treatment in RDS is inadequate pulmonary exchange
of oxygen and carbon dioxide; metabolic acidosis and circulatory insufficiency are
secondary manifestations.
30.(B). Warm humidified oxygen should be provided at a concentration initially
sufficient to keep arterial oxygen pressure between 50 and 70 mm Hg (91-95%
saturation) in order to maintain normal tissue oxygenation while minimizing the risk of
oxygen toxicity. If oxygen saturation cannot be kept >90% at inspired oxygen
concentrations of 40-70% or greater, applying CPAP at a pressure of 5-10 cm H2O via
nasal prongs is indicated and usually produces a rapid improvement in oxygenation.
CPAP reduces collapse of surfactant-deficient alveoli and improves both FRC and
ventilation–perfusion matching.
31.(E). The lack of reduction in BPD rates following surfactant replacement is probably,
in part, due to the survival of infants with severe RDS who would have died without
surfactant administration.
32.(E). Inhaled nitric oxide (iNO) decreases the need for extracorporeal membrane
oxygenation (ECMO) in term and near-term infants with hypoxic respiratory failure or
persistent pulmonary hypertension of the neonate.
33.(E). Alkali therapy may result in skin slough from infiltration, increased serum
osmolarity, hypernatremia, hypocalcemia, hypokalemia, and liver injury when

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concentrated solutions are administered rapidly through an umbilical vein catheter
wedged in the liver.
34.(B). Overhydration during the 1st days of life may also contribute to the
development of BPD. Vitamin A supplementation (5,000 IU intramuscularly 3 times/wk
for 4 wk) in VLBW infants reduces the risk of BPD.
35.(D). Methylxanthines may also decrease PVR and increase lung compliance in infants
with BPD, probably through direct smooth muscle relaxation.
36.(E). The distinctive features of transient tachypnea are rapid recovery of the infant
and the absence of radiographic findings for RDS (hypoaeration, diffuse
reticulogranular pattern, air bronchograms) and other lung disorders.
37.(E). Meconium-stained amniotic fluid is found in 10-15% of births and usually occurs
in term or post-term infants.
38.(A). Persistent pulmonary hypertension of the newborn (PPHN) occurs in term and
post-term infants. Predisposing factors include birth asphyxia, MAS, early-onset sepsis,
RDS, hypoglycemia, polycythemia, maternal use of nonsteroidal anti-inflammatory
drugs with in utero constriction of the ductus arteriosus, maternal late trimester use of
selective serotonin reuptake inhibitors, and pulmonary hypoplasia due to
diaphragmatic hernia, amniotic fluid leak, oligohydramnios, or pleural effusions. PPHN
is often idiopathic.
39.(A). Pulmonary hypoplasia and malrotation of the intestine are part of the lesion,
not associated anomalies.
40.(D). Polyhydramnios
41.(C). Early respiratory distress, within 6 hr of life, is thought to be a poor prognostic
sign.
42.(A). Congenital eventration may affect lung development, but it has not been
associated with pulmonary hypoplasia.
43.(C). Clustering of cases suggests a primary role for an infectious agent. Various
bacterial and viral agents, including Escherichia coli, Klebsiella, Clostridium perfringens,
Staphylococcus epidermidis, astrovirus, norovirus, and rotavirus, have been recovered
from cultures. Nonetheless, in most situations, no pathogen is identified.
44.(A). A surgeon should be consulted early in the course of treatment. Indications for
surgery include evidence of perforation on abdominal roentgenograms
(pneumoperitoneum) or positive result of abdominal paracentesis (stool or organism
on Gram stain preparation from peritoneal fluid). Failure of medical management, a
single fixed bowel loop on radiographs, abdominal wall erythema, and a palpable mass
are relative indications for exploratory laparotomy.
45.(B). Male gender is a minor risk factor.
46.(B). Jaundice that first appears on the 2nd or 3rd day is usually physiologic but may
represent a more severe form. Familial nonhemolytic icterus (Crigler-Najjar syndrome)
and early-onset breast-feeding jaundice are seen initially on the 2nd or 3rd day.
47.(E). Direct bilirubin fraction is >2 mg/dL at any time.

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48.(E). Although uncommon, kernicterus can occur in patients with breast milk
jaundice.
49.(B). Dark skin does not reduce the efficacy of phototherapy.
50.(A). Early ultrasonographic signs of hydrops include organomegaly (liver, spleen,
heart), the double–bowel wall sign (bowel edema), and placental thickening.
Progression to polyhydramnios, ascites, pleural or pericardial effusions, and skin or
scalp edema may then follow.
51.(E). The white blood cell count is usually normal but may be elevated.
52.(D). Although antibodies against A and B factors occur without previous
immunization (“natural” antibodies), they are usually IgM antibodies that do not cross
the placenta. However, IgG antibodies to A antigen may be present and these do cross
the placenta, so A-O isoimmune hemolytic disease may be found in first-born infants.
53.(B). 5% of first-born infants may be affected.
54.(B). Peripheral (heelstick) Hct values are higher than central values, whereas Coulter
counter results are lower than Hct values determined by microcentrifugation.
55.(D). Although oral vitamin K (birth, discharge, 3-4 wk: 1-2 mg) has been suggested as
an alternative, oral vitamin K is less effective in preventing the late onset of bleeding
due to vitamin K deficiency and thus cannot be recommended for routine therapy. The
intramuscular route remains the method of choice.
56.(B). Apt devised the following test for differentiation of fetal from maternal blood:
(1) Rinse a blood-stained diaper or some grossly bloody (red) stool with a suitable
amount of water to obtain a distinctly pink supernatant hemoglobin solution; (2)
centrifuge the mixture and decant the supernatant solution; (3) add 1 part of 0.25 N
(1%) sodium hydroxide to 5 parts of the supernatant fluid. Within 1-2 min, a color
reaction takes place: A yellow-brown color indicates that the blood is maternal in
origin; a persistent pink indicates that it is from the infant. A control test with known
adult or infant blood, or both, is advisable.
57.(B). Long cords (>70 cm) increase risk for true knots, wrapping around fetal parts
(neck, arm), and/or prolapse. Straight untwisted cords are associated with fetal
distress, anomalies, and intrauterine fetal demise.
58.(C). Most umbilical hernias that appear before the age of 6 mo disappear
spontaneously by 1 yr of age. Even large hernias (5-6 cm in all dimensions) have been
known to disappear spontaneously by 5-6 yr of age.
59.(B). Persistent edema of 1 or more extremities may represent congenital
lymphedema (Milroy disease) or, in females, Turner syndrome.
60.(A). Diabetic mothers have a high incidence of polyhydramnios, preeclampsia,
pyelonephritis, preterm labor, and chronic hypertension; their fetal mortality rate is
greater than that of nondiabetic mothers, especially after 32 wk of gestation. Fetal loss
throughout pregnancy is associated with poorly controlled maternal diabetes
(especially ketoacidosis) and congenital anomalies.
61.(B). The probable pathogenic sequence is that maternal hyperglycemia causes fetal
hyperglycemia, and the fetal pancreatic response leads to fetal hyperinsulinemia; fetal

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hyperinsulinemia and hyperglycemia then cause increased hepatic glucose uptake and
glycogen synthesis, accelerated lipogenesis, and augmented protein synthesis. Related
pathologic findings are hypertrophy and hyperplasia of the pancreatic islet β cells,
increased weight of the placenta and infant organs except for the brain, myocardial
hypertrophy, increased amount of cytoplasm in liver cells, and extramedullary
hematopoiesis. The incidence of congenital anomaliesis increased 3-fold in infants of
diabetic mothers; cardiac malformations (ventricular or atrial septal defect,
transposition of the great vessels, truncus arteriosus, doubleoutlet right ventricle,
tricuspid atresia, coarctation of the aorta) and lumbosacral agenesis are most
common.
62.(D). Disruption sequence is in utero tissue destruction after a period of normal
morphogenesis while deformation sequence is a mechanical (uterine) force that alters
structure of intrinsically normal tissue.
63.(A). X-linked hydrocephalus, Achondroplasia, Treacher Collins syndrome, and Apert
disease are monogenic causes of congenital malformations.
64.(B). Postaxial polydactyly= Extra finger or toe present on the lateral side of the hand
or foot. Preaxial polydactyly=Extra finger or toe present on the medial side of the hand
or foot.
65.(A). Although HSV, HIV, hepatitis B virus (HBV), hepatitis C virus, and tuberculosis
(TB) can each result in transplacental infection, the most common mode of
transmission for these agents is intrapartum, during labor and delivery with passage
through an infected birth canal (HIV, HSV, HBV), or postpartum, from contact with an
infected mother or caretaker (TB) or with infected breast milk (HIV).
66.(B). Agents that commonly cause nosocomial infection are coagulase-negative
staphylococci, gram-negative bacilli (E. coli, Klebsiella pneumoniae, Salmonella,
Enterobacter, Citrobacter, Pseudomonas aeruginosa, Serratia), enterococci, S. aureus,
and Candida. Viruses contributing to nosocomial neonatal infection include
enteroviruses, CMV, hepatitis A, adenoviruses, influenza, respiratory syncytial virus
(RSV), rhinovirus, parainfluenza, HSV, and rotavirus. Community-acquired pathogens
such as Streptococcus pneumoniae may also cause infection in newborn infants after
discharge from the hospital.
67.(D). Intrapartum chemoprophylaxis does not reduce the rates of late-onset GBS
disease and has no effect on the rates of infection with non-GBS pathogens. Of concern
is a possible increase in gram-negative infections (especially E. coli) in VLBW and
possibly term infants in spite of a reduction in early GBS sepsis by intrapartum
antibiotics.
68.(B). In a cohort of 6,215 VLBW infants in the NICHD Neonatal Research Network,
gram-positive agents were associated with 70%, gram-negative with 18%, and fungi
with 12% of cases of late-onset sepsis.
69.(D). Osteitis may be a feature of rubella and T. pallidum.
70.(A). Intracranial calcification may be a feature of CMV, HIV, toxoplasmosis, and T.
cruzi.

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71.(C).
72.(E). Pneumonia and empyema are common respiratory tract manifestations of
neonatal bacterial infections.
73.(D). Breast abscess and impetigo are common skin and soft tissue manifestations of
neonatal bacterial infections.
74.(B). Abnormal white blood cell (WBC) count or an increase in immature forms is
included in the definition of the systemic inflammatory response SIRS in adults.
75.(D). Staphylococcus—coagulase negative (9.1%), Group B streptococcus (21.9%),
Escherichia coli (34.0%), Pseudomonas (74.4%), and Candida albicans (43.9%).
76.(C). The umbilical cord generally dries up and sloughs by 2 weeks of life. Delayed
separation can be normal up to 45 days. However, because neutrophilic and/or
monocytic infiltration appears to play a major role in autodigestion, persistence of the
cord beyond 30 days should prompt consideration of an underlying functional
abnormality of neutrophils (leukocyte adhesion deficiency) or neutropenia.
77.(E). In an analysis of 15,072 neonates born at 98 centers over a 2-year period, the
most important variables associated with NEC were gestational age and birth weight.
78.(E). Pneumatosis intestinalis can be seen in various other conditions, including
Hirschsprung's disease, pseudomembranous enterocolitis, neonatal ulcerative colitis,
and ischemic bowel disease. However, it is a characteristic finding in 85% of patients
with NEC. Dark, concentric rings within the bowel wall represent hydrogen as a
byproduct of bacterial metabolism
79.(B). Reticulocytosis (>8% at birth, >5% during first 2-3 days, >2% after first week).
80.(D). Reducing sugars (e.g., glucose, fructose, galactose, pentoses, and lactose) are
detected, but sucrose is not a reducing sugar.
81.(C). Chvostek's sign (facial muscle twitching on tapping), and Trousseau's sign
(carpopedal spasm) may be present, but more commonly these are absent during the
neonatal period.
82.(D). In a series of infants studied by ultrasonography, approximately 50% had the
onset of hemorrhage on the first day of life, 25% on the second day, and 15% on the
third day. Thus, a single scan on the fourth day of life would be expected to detect
>90% of IVHs.
83.(B). The antenatal administration of magnesium sulfate before preterm delivery has
been suggested to decrease the risk of cerebral palsy in surviving infants but not
improve overall survival. Prospective multicenter randomized trials are currently
examining this potential benefit.

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