CHAPTER 3

The Neonate
Eugenia K. Pallotto, MD

The neonatal period is defined as the period of life from birth until 4 weeks (28 days) of age. This is
a vulnerable period of time in the life of a human since the transition from fetal to extrauterine life involves
many complex biochemical and physiologic processes. The newborn infant must adapt from dependence on
the maternal placental circulation to self-sufficient functioning of all physiologic systems.
There are many unique disease processes that present during this time period along with a unique set of
normal laboratory and physiologic parameters. Many problems of the newborn infant are a result of abnormalities
in the transition to extrauterine life either due to prematurity, the effects of an immature immunological system,
congenital anomalies, or adverse effects of the events surrounding delivery. Understanding of the unique
physiology and pathophysiology associated with this period of life is imperative for the physician to be able
to appropriately diagnose and treat the newborn infant.

47
 Questions

DIRECTIONS (Questions 1 through 88): For each (A) hyperbilirubinemia

of the multiple choice questions in this section (B) congenital infection
select the one lettered answer that is the best (C) aminoglycoside therapy
response in each case.
(D) neuroblastoma
(E) late-onset group B streptococcal meningitis
1. An infant is diagnosed with a given disorder
below. Which of these poses the greatest recur- 4. The pediatric surgeon is requesting an echocar-
rence risk for this patient’s future siblings? diogram on a hospitalized newborn with a con-
(A) Hirschsprung disease genital defect of the gastrointestinal tract.
Which of the following defects has the highest
(B) cystic fibrosis
incidence of associated cardiac defects?
(C) ventricular septal defect
(D) trisomy 21 (A) omphalocele
(E) trisomy 13 (B) congenital volvulus
(C) Hirschsprung disease
2. A term infant with microcephaly, jaundice, and (D) gastroschisis
thrombocytopenia is thought to have congeni- (E) pyloric stenosis
tal CMV infection. Your attending physician
notes though that 1.5% of all newborns may 5. You are a practicing pediatrician in a state
have asymptomatic congenital cytomegalovirus where cystic fibrosis is not a routine part of
infection. Which of the following is the most newborn screening. Which of the following
commonly reported sequelae of such infections? symptoms in a newborn infant would prompt
(A) chorioretinitis you to test for cystic fibrosis?
(B) sensorineural hearing loss (A) pneumonia
(C) thrombocytopenia (B) intrauterine growth retardation
(D) poor growth (C) meconium ileus
(E) liver failure (D) wheezing
(E) hypochloremic alkalosis
3. A 2-year-old infant has acquired sensorineural
hearing loss. His mother is asking what the 6. An infant is born precipitously to a mother
most likely cause could be. What is the least without prenatal care and you are requested to
likely etiology of acquired sensorineural hear- determine the infant’s gestational age. Of the
ing loss in a toddler? following, which physical finding is most
indicative of a full-term infant?

48
 Questions: 1–12 49

(A) veins and tributaries are seen over the (D) reincorporation into hemoglobin
abdomen (E) hepatic secretion and storage in the
(B) long lanugo is present on the back intestinal lumen
(C) palpable breast tissue of less than 1 cm
(D) pitting edema over the tibia 10. You are the attending physician for a newborn
infant with hemolytic jaundice. The mother did
(E) soft ear pinnae, easily folded
not receive prenatal care with this pregnancy or
her prior pregnancy. The direct Coombs test is
7. A 1-week-old term newborn is in your office for
positive. The mother’s blood type is A- and the
a well-child assessment, physical examination
baby’s blood type is O+. Her first baby did
findings are consistent with oral candidiasis
not have hemolytic jaundice. What is the most
(thrush). Which of the following is a correct
likely cause of the hemolytic jaundice?
statement regarding thrush in a term newborn?
(A) ABO incompatibility
(A) responds well to topical therapy with
nystatin (B) Toxoplasmosis
(B) requires systemic therapy with (C) Rh incompatibility
amphotericin B (D) rubella
(C) requires both topical (nystatin) and sys- (E) hereditary spherocytosis
temic (amphotericin) therapy
(D) requires investigation to rule out 22q11 11. The blood bank has received an order for an
deletion syndrome intrauterine transfusion. A fetus with which of
the following would most likely require trans-
(E) requires no treatment
fusion prior to birth?
8. During the delivery room resuscitation of a (A) erythroblastosis fetalis
vigorous term newborn, which of the following (B) sickle cell anemia
should be performed first? (C) spherocytosis
(A) Verify the airway is clear, dry, and stim- (D) fetal distress and bradycardia
ulate the infant. (E) congenital heart disease
(B) The heart rate should be auscultated.
(C) Breath sounds should be auscultated. 12. The obstetrician performing the intrauterine
(D) The mouth and trachea should be transfusion is counseling the mother regard-
suctioned. ing complications of this procedure. Which of
the following is the most common complication
(E) Assess color and administer oxygen if
of intrauterine transfusion?
necessary.
(A) a transfusion reaction (mismatch)
9. A low-risk newborn infant has pathologic (B) graft-versus-host reaction
unconjugated hyperbilirubinemia, which is (C) premature onset of labor
appopriately diagnosed within the first few
(D) acquired immunodeficiency syndrome
hours of life due to the astute observation of
(AIDS)
rapid, progressive jaundice by the nurse. She is
asking why the infant was not jaundiced imme- (E) renal failure
diately after birth. Which of the following best
describes the major route for excretion of biliru-
bin in the fetus in utero?
(A) via the kidney
(B) transplacental passage
(C) degradation to biliverdin
50 3: The Neonate
13. During a routine prenatal visit a mother states
she has been reading about kernicterus. She is
very concerned that her infant will develop
kernicterus if she provides breast milk for the
infant since she has also read that some breast-
fed infants have high bilirubin levels. Which of
the following is most predictive for the devel-
opment of kernicterus?
(A) hyperbilirubinemia within the first
24 hours of life
(B) peak conjugated bilirubin level
(C) peak unconjugated bilirubin level
(D) duration of hyperbilirubinemia
(E) hemoglobin level immediately after birth
14. A medical student in your office is assessing a
1-week-old term infant. The mother has been
exclusively breast-feeding and the student is
not sure how to interpret the change in the
infant’s weight compared to the birth weight.
Which of the following is the expected weight
flux for an infant in the first week of life?
(A) gain approximately 30 g/day
(B)gain approximately 60 g/day
(C) neither gain nor lose weight
(D) lose approximately 5%–10% of its birth
weight
(E) lose approximately 15% of its birth weight
15. A 3-day-old infant is requiring phototherapy
for hyperbilirubinemia. Which of the following
risk factors best predicts the occurrence of ABO
isoimmune hemolytic disease in a newborn?
(A) first pregnancy
(B)more than four pregnancies
(C) prior Rh disease
(D) maternal blood type is O and infant is
type A
(E) preexisting maternal anemia
16. The patient in Question 15 was hospitalized
for 2 weeks, treated with phototherapy for
12 days, and required a red cell transfusion
during the hospitalization. Which of the following
is the most common serious late clinical mani-
festation of ABO disease?
(A) kernicterus
(B) congestive heart failure
(C) gallstones
(D) bilirubinuria
(E) iron deficiency
17. Prevention of bilirubin encephalopathy or ker-
nicterus is one of the goals for the appropriate
diagnosis and treatment of hyperbilirubine-
mia. Which of the following mechanisms has a
role in preventing these adverse outcomes?
(A) Unconjugated bilirubin is not lipid soluble.
(B) Unconjugated bilirubin is tightly bound
to albumin.
(C) Unconjugated bilirubin is tightly bound
to hemoglobin.
(D) The blood–brain barrier is impermeable
to unconjugated bilirubin.
(E) Unconjugated bilirubin is rapidly
metabolized by cerebrospinal fluid.
18. You are formulating a differential diagnosis for
a newborn infant with respiratory distress.
Which of the following is most closely associ-
ated with the development of neonatal respi-
ratory distress syndrome (hyaline membrane
disease)?
(A) gestational age
(B) birth weight
(C) cesarean section delivery
(D) maternal diabetes
(E) meconium in the amniotic fluid
19. Which of the following statements is true
regarding infants of comparable weight and
gestational age (> 1500 g) in the United States
regarding mortality rate?
(A) There is no difference in mortality rates
between males and females and African
Americans and Caucasians.
(B) Males have a higher mortality rate than
females, and African Americans have a
higher mortality rate than Caucasians.
(C) Males have a lower mortality rate than
females, and African Americans have a
lower mortality rate than Caucasians.

(D) Males have a higher mortality rate than
females, and African Americans have a
lower mortality rate than Caucasians.
(E) Males have a lower mortality rate than
females, and African Americans have a
higher mortality rate than Caucasians.
20. A 29-week-gestation infant is being resusci-
tated in the delivery room. Surfactant is given
through the endotracheal tube. Which of the
following is the most physiologically active
component of surfactant?
(A) surfactant protein A
(B) surfactant protein B
(C) neutral lipid
(D) water
(E) phospholipid
21. A25-week-gestation infant is born to a 25-year-old
primigravida, who has had preeclampsia.
Which of the following is a true statement
describing the neonatal mortality rate for this
infant?
(A) It decreases with increasing gestational
age from 30 weeks through 43 completed
weeks of gestation.
(B) It is not related to birth weight.
(C) It is not related to race.
(D) It is higher than the mortality rates of
adolescents.
(E) It has not changed significantly since
1980.
22. During the resuscitation of a preterm infant, a
medical student in the delivery room is asking
why surfactant is so important to neonatal lung
physiology. Which of the following is true of
surfactant production as it relates to respiratory
distress syndrome in a premature infant?
(A) Surfactant is synthesized and stored in
the type I alveolar cells.
(B) Surfactant is synthesized and stored in
the type II alveolar cells.
(C) Surfactant is produced by the pulmonary
alveolar macrophages.
Questions: 13–25 51
(D) Surfactant is stored in the interstitial
spaces in the lungs.
(E) Surfactant is not produced until after
labor ensues.
23. A mother presents in active labor, she did not
receive prenatal care and is unsure when her
last menstrual period was. Using ultrasound,
the estimated gestational age is 30 weeks.
Which of the following best describes the aver-
age birth weight of a 30-week gestation infant?
(A) 500 g
(B) 1000 g
(C) 1500 g
(D) 2000 g
(E) 2500 g
24. A mother presents for prenatal care with a com-
plicated medical history as listed in the following
choices. Which of the conditions most predis-
poses her fetus to congenital heart disease?
(A) hypertension
(B) diabetes mellitus
(C) atherosclerotic coronary vascular disease
(D) anemia
(E) rheumatoid arthritis
25. On a discharge examination you hear a heart
murmer and consult the cardiologist. An
echocardiogram was notable for a small ven-
tricular septal defect and a patent foramen
ovale. You are notifying the parents of the
results and explain to them the role of the fora-
men ovale in fetal life. Which of the following
statements are true?
(A) Blood flows through the foramen ovale
from the right ventricle to the left ventricle.
(B) Blood flows through the foramen ovale
from the left ventricle to the right ventricle.
(C) Blood flows through the foramen ovale
from the left atrium to the right atrium.
(D) Blood flows through the foramen ovale
from the right atrium to the left atrium.
(E) Blood must pass through the foramen
ovale for blood to enter the right atrium
from the umbilical vein.
52 3: The Neonate
26. A full-term newborn has a diffuse rash on the
day of anticipated discharge. You diagnose ery-
thema toxicum and must discuss this finding
with the parents. Which of the following is true?
(A) more common among term than prema-
ture infants
(B) usually associated with fever and a gen-
eral toxic state
(C) uncommon before the fifth day of life
(D) usually associated with an elevated
peripheral white blood cell count
(E) manifested in less than 10% of newborns
27. A mother is hospitalized with high blood pressue
at 35 weeks gestation. The perinatologist recom-
mends obtaining a lecithin–sphingomyelin ratio
of the amniotic fluid to aid in the decision for
delivery. For which of the following systems
does the lecithin–sphingomeylin ratio indicate
maturity?
(A) central nervous system
(B) lungs
(C) liver
(D) kidneys
(E) immunologic system
28. A hospitalized neonate is given the diagnosis of
bronchopulmonary dysplasia (BPD). Which of
the following best describes the pathophysiol-
ogy of BPD?
(A) An inflammatory insult to the lungs late
in fetal development.
(B) Failure of development of pulmonary
arterioles during early fetal life.
(C) Failure of development of the bronchial
buds during early fetal life.
(D) Intrauterine viral infection.
(E) The use of oxygen and positive-pressure
breathing in the treatment of respiratory
distress syndrome.
29. A 32-week-gestation infant is now 24 hours
old and has had progressive respiratory dis-
tress. Given the infant’s clinical course and the
radiographic appearance of the lungs the deci-
sion has been made, in consultation with the
neonatologist, to give surfactant replacement
therapy for respiratory distress syndrome.
Which of the following is true?
(A) is considered experimental
(B) is only useful in infants with birth
weight less than 1500 g
(C) has no known complications
(D) has not been shown to reduce mortality
in very low birth weight infants
(E) requires tracheal intubation to administer
30. You are examining the chest x-ray of a 4-hour-
old infant born at 30 weeks gestation. The
infant is breathing 100 times per minute while
breathing 100% oxygen and intubation is immi-
nent. You suspect respiratory distress syndrome.
Which of the following is the characteristic
roentgenographic findings of the infant with
respiratory distress syndrome?
(A) lobar atelectasis and interstitial edema
(B) bilateral patchy densities and pneu-
mothorax
(C) diffuse reticulogranular changes and air
bronchograms
(D) diffuse hyperaeration and cardiomegaly
(E) cardiomegaly and interstitial edema
31. After surfactant therapy, the infant with respi-
ratory distress syndrome is treated with con-
tinuous positive airway pressure. Which of the
following is the major goal of continuous pos-
itive airway pressure?
(A) prevent infection
(B) prevent pneumothorax
(C) improve cardiac output
(D) raise arterial Po2
(E) raise arterial Pco2
32. A 30-week-gestation infant with respiratory
distress syndrome is weaning from the venti-
lator. He currently has an oxygen saturation of
100% while breathing 50% oxygen on minimal
ventilator settings. In a premature infant with
respiratory distress syndrome, which of the fol-
lowing may be an adverse effect of supple-
mental oxygen therapy?
 Questions: 26–38 53

(A) alveolar proteinosis 36. A mother has brought her infant into your
(B) atelectasis office for the first newborn visit. She is very
(C) fire or explosion concerned about a pigmented skin lesion that
was not discussed with her in the hospital.
(D) kernicterus
Which of the following is true of pigmented
(E) retinopathy of prematurity lesions known as slate gray spots?
33. The father of a 26-week-gestation infant, who (A) They never occur in white infants.
required intubation at birth, is asking why the (B) They are identified in over 40% of
infant’s lungs were not mature. Which of the African American infants.
following is the pathophysiology mechanism (C) They consist of small, well-demarcated
of respiratory distress syndrome in the prema- lesions approximately 2 mm in diameter.
ture infant? (D) Malignant degeneration is common.
(A) increased production of pulmonary (E) The most common site of occurrence is
surfactant the nape of the neck.
(B) decreased production of pulmonary
surfactant 37. A newborn infant presents with cyanosis and
(C) increased metabolism of pulmonary mild tachypnea at about 6 hours of life. The
surfactant infant is placed in 95% oxyhood and satura-
tions normalize. Which of the following is the
(D) decreased metabolism of pulmonary
most likely diagnosis in this infant?
surfactant
(E) rerouting of pulmonary surfactant to the (A) cyanotic congenital heart disease
systemic circulation (B) lung disease
(C) central nervous system disease
34. A healthy term infant is circumcised and expe- (D) liver disease
riences excessive blood loss eventually requir-
(E) methemoglobinemia
ing transfusion. The most likely diagnosis is
which of the following?
38. A 35-week-gestation infant is delivered weighing
(A) factor IX deficiency 3.9 kg, with an omphalocele and a large tongue.
(B) factor VIII deficiency No other abnormalities are detected. Which of
(C) von Willebrand disease the following is the most likely diagnosis?
(D) disseminated intravascular coagulopathy (A) congenital hypothyroidism
(E) protein C deficiency (B) trisomy 18
(C) trisomy 13
35. An irregular red reflex is noted on the initial (D) fetal alcohol syndrome
examination of an infant. The infant is referred to
(E) Beckwith-Wiedemann syndrome
the opthalmologist for evaluation of a cataract.
Which of the following is most likely to be asso-
ciated with a cataract in the newborn?
(A) maple syrup urine disease
(B) glucose-6-phosphate dehydrogenase
deficiency
(C) phenylketonuria
(D) galactosemia
(E) propionic acidemia
54 3: The Neonate

39. A 3-week-old infant is noted to have micro- (A) hypopigmented patch

cephaly, cerebral calcifications on skull x-ray, (B) harlequin color change
and blindness. Which of the following is the (C) salmon patch on the nasal glabella
most likely cause of these findings?
(D) hemangioma of the thigh
(E) pustular melanosis

42. A term, otherwise healthy, neonate has isolated

premature synostosis of the sagittal suture.
Which of the following is the most likely asso-
ciated condition?
(A) scaphocephaly
(B) increased intracranial pressure
(C) microcephaly
(D) hydrocephalus
(E) subdural effusions

43. A healthy newborn is noted to have numerous

Figure 3-1 3 mm vesicles on the chest and neck, along
(Courtesy of Angela Myers, MD ) with several similar-sized hyperpigmented
macules in the same distribution. Which of the
following is the most likely diagnosis?
(A) bilateral subdural hemorrhages
(A) mucocutaneous herpes simplex infection
(B) cerebral agenesis
(B) acne neonatorum
(C) cytomegalovirus infection
(C) erythema toxicum
(D) erythroblastosis
(D) incontinentia pigmenti
(E) primary microcephaly
(E) transient neonatal pustular melanosis
40. A 7-day-old infant develops white, cheesy
patches on the tongue and buccal mucosa with 44. A newborn infant gags and chokes with feed-
mild inflammation of the mucosa. Which of ings. He has just had an apneic spell during a
the following organisms is most likely the feed resulting in significant cyanosis. Which of
cause of these oral lesions? the following is the most likely diagnosis?

(A) Candida albicans (A) proximal esophageal atresia

(B) Listeria monocytogenes (B) hypoplastic left heart syndrome
(C) Escherichia coli (C) group B streptococcal sepsis
(D) group A streptococcus (D) neonatal herpes simplex infection
(E) group B streptococcus (E) pyloric stenosis

41. You are interviewing a family with a newborn
infant. They are new to your practice and you
elicit a family history of seizures in both the
mother and maternal grandfather. During
examination of the infant you note a skin find-
ing that may be associated with the family his-
tory of seizures. Which of the following is the
most likely skin finding in this infant?
45. As a medical student in the newborn nursery,
you correctly identify a scalp swelling as a
cephalohemtoma. Your attending physician then
asks you to describe how you would differenti-
ate this from caput succedaneum. Which of the
following best describes the way to differentiate
a cephalohematoma from caput succedaneum?
 Questions: 39–51 55

(A) absence of a history of prolonged or dif- 49. A nurse working in the newborn nursery calls
ficult labor your office because she is concerned about the
(B) limitation of swelling to the area over appearance of the feet of a newborn infant just
one bone delivered. Based on her description, the infant
(C) a normal neurologic examination has congenital clubfoot. Which of the following
systems is most commonly also involved with
(D) a prolonged prothrombin time
this disorder?
(E) a normal lumbar puncture
(A) the central nervous system
46. A 40-year-old couple is in your office for (B) the hematopoietic system
genetic counseling prior to having their first (C) the gastrointestinal system
child. They are very concerned about advanc- (D) the cardiovascular system
ing maternal age and the possible genetic prob-
(E) the respiratory system
lems it could cause for their child. Which of
the following is the most likely disorder asso-
50. A newborn infant has micrognathia, glossop-
ciated with advancing maternal age?
tosis, and cleft soft palate. These findings are
(A) autosomal recessive disorders consistent with Pierre Robin sequence. Which
(B) nondisjunction chromosome disorders of the following life-threatening events is asso-
(C) autosomal dominant disorders ciated with these findings?
(D) X-linked disorders (A) heart failure
(E) inborn errors of metabolism (B) seizures
(C) intestinal obstruction
47. The same couple in Question 46 also is inquiring (D) metabolic acidosis
about the genetic affects of the father’s age. Which
(E) upper airway obstruction
of the following is the most important disorder
associated with advancing paternal age?
51. A consultant in the neonatal intensive care unit
(A) autosomal recessive disorders is recommending a trial of pyridoxine for a
(B) nondisjunction chromosome disorders patient. Which of the following problems in a
(C) autosomal dominant disorders newborn infant might respond to a pharmaco-
logic dose of pyridoxine?
(D) X-linked disorders
(E) inborn errors of metabolism (A) blindness
(B) seizures
48. A normal newborn is screened for hypo- (C) jaundice
glycemia after birth as per the normal nursery (D) rash
protocol at your hospital. In the asymptomatic
(E) urinary retention
term neonate, evaluation and treatment of
hypoglycemia should be initiated when the
glucose level is at or below which of the fol-
lowing levels?
(A) 10 mg/dL
(B) 20 mg/dL
(C) 40 mg/dL
(D) 60 mg/dL
(E) 80 mg/dL
56 3: The Neonate
52. The geneticist is evaluating a patient in the
newborn intensive care unit. The possibility of
a disorder with a mitochondrial inheritance
pattern has been discussed with the family.
Which of the following is true of mitochondrial
inheritance?
(A) These disorders commonly follow a
paternal line of inheritance.
(B) These disorders have not been identified
in humans thus far.
(C) Only females are affected.
(D) Organ systems with low energy
demands are the most affected.
(E) These disorders commonly follow a
maternal line of inheritance.
53. A family with an infant in the neonatal inten-
sive care unit is very concerned that their child
will have long-term neurologic abnormalities.
Of the following, which correlates best with
subsequent neurologic abnormalities?
(A) fetal bradycardia
(B) failure to breathe at birth
(C) a low 1-minute Apgar score
(D) a low 5-minute Apgar score
(E) seizures in the first 36 hours of life
54. An 8-day-old infant develops inflammatory
papules and pustules on the forehead, nose,
and malar areas of the face. The child is other-
wise well, and the remainder of the physical
examination is normal. Which of the follow-
ing is the most likely diagnosis?
(A) congenital syphilis
(B) impetigo
(C) neonatal acne
(D) staphylococcal pustulosis
(E) tuberous sclerosis
55. You are counseling a mother with an abnor-
mal serum alpha-fetoprotein test during a
follow-up prenatal visit. The maternal serum
alpha-fetoprotein test is most useful in diag-
nosing which of the following?
(A) duodenal atresia
(B) clubfoot
(C) cleft lip and palate
(D) myelomeningocele
(E) alveolar proteinosis
56. A newborn with trisomy 21 develops bilious
emesis on the first day of life. An abdominal
x-ray reveals a “double bubble sign.” Which of
the following is the most likely diagnosis?
(A) annular pancreas
(B) duodenal atresia
(C) gastric volvulus
(D) pyloric stenosis
(E) Hirschsprung disease
57. A 1700-g infant was asphyxiated at birth, after
a successful resuscitation, the infant had
numerous apneic episodes. On the third day of
life, the infant began to vomit. Abdominal dis-
tention and bloody stools were noted. Which of
the following is the most likely diagnosis?
(A) congenital aganglionic megacolon
(B) intussusception
(C) necrotizing enterocolitis
(D) Shigella enteritis
(E) volvulus
58. A 2-month-old infant is in your office for a
well-child examination. The mother is asking
you about a red macule on the infant’s cheek.
She describes it as getting larger over the past
few weeks. You suspect this is a capillary hema-
gioma. The natural history of an elevated capil-
lary or cavernous hemangioma is best described
by which of the following statements?
(A) no significant change in size after birth.
(B) an increase in size during the first few
years after birth and then regression.
(C) an increase in size during the first decade
of life and then no further change.
(D) a slow but progressive increase in size
throughout life.
(E) a slow but progressive decrease in size
starting shortly after birth.
 Questions: 52–65 57

59. A full-term, ill appearing, infant presents with 63. A newborn infant is being evaluated for
vesicles and large bullae shortly at birth. Which ambiguous genitalia. While awaiting results of
of the following is most likely? chromosome analysis, the infant develops
hyponatremia, hypoglycemia, and low blood
(A) herpes simplex virus
pressure. What is the most likely diagnosis?
(B) gram-negative infection
(C) epidermolysis bullosa (A) 21-hydroxylase deficiency
(D) Staphylococcus aureus (B) severe hypospadius
(E) maternal diabetes (C) 3-beta-hydroxysteroid dehydrogenase
deficiency
60. A newborn infant presents with skin lesions (D) 11-beta-hydroxylase deficiency
typical of erythema toxicum and you wish to (E) 17-alpha-hydroxylase deficiency
confirm the diagnosis. Wright stain of the con-
tents from a lesion of erythema toxicum usually 64. You are evaluating a small-for-gestational-age
will reveal which of the following cells? infant for tachycardia. There is no prenatal care
history available, but based on the infant’s
(A) basophils
signs and symptoms and the obstetrician’s
(B) eosinophils description of the mother you suspect neonatal
(C) lymphocytes thyrotoxicosis. Thyrotoxicosis in the first day of
(D) immature lymphocytes life most likely occurs in an infant born to a
(E) polymorphonuclear leukocytes mother with which of the following diagnoses?
(A) with untreated hypothyroidism
61. A mother with hyperparathyroidism just deliv-
(B) with untreated Graves disease
ered a term infant who appears healthy. Which
of the following problems is likely to develop (C) with Graves disease being treated with
in her infant? antithyroid medications
(D) with euthyroid goiter
(A) hypercalcemia
(E) receiving iodides as therapy for chronic
(B) hypocalcemia bronchitis
(C) parathyroid carcinoma
(D) hyperthyroidism 65. During a prenatal care visit, a mother notifies
(E) hyperparathyroidism you that her partner has been diagnosed with
herpes simplex virus (HSV). She is asking
62. A screening bilirubin test is sent on a term new- about risks for the infant if she has HSV. Which
born who appears jaundiced at 72 hours of life. of the following is the most important risk fac-
You unexpectedly identify conjugated hyper- tors for neonatal HSV infection?
bilirubinemia. Which of the following is asso- (A) prematurity
ciated with conjugated hyperbilirubinemia?
(B) cold sore on mother’s lip at time of
(A) sequestrated blood delivery
(B) tyrosinemia (C) primary maternal genital infection dur-
(C) hereditary spherocytosis ing pregnancy
(D) Rh incompatibility (D) fetal scalp monitoring
(E) physiologic jaundice (E) rupture of membranes greater than
4–6 hours
58 3: The Neonate
66. As a medical student in the neonatal intensive
care unit, you report a calcium level in a 1-day-
old term newborn that was flagged as abnor-
mal by the laboratory. Which of the following
is true of serum calcium levels in the newborn?
(A) High parathyroid hormone (PTH) levels
suppress the serum calcium.
(B) The serum calcium promptly decreases
after delivery.
(C) The serum calcium remains essentially
unchanged over the first 3 days.
(D) The serum calcium is usually higher on
day 2 than day 1.
(E) Infants of diabetic mothers have an
accentuated PTH response resulting in
hypercalcemia.
67. A mother of a 3-day-old newborn, born at
home with a midwife, is calling your office
because she is concerned about constipation.
You are deciding whether to evaluate this
infant for Hirschsprung disease and ask her
about meconium passage. Meconium passage
delayed beyond which of the following time
points to the diagnosis of Hirshsprung disease?
(A) during the process of birth
(B) within a few minutes of birth
(C) 2–4 hours of life
(D) 12–18 hours of life
(E) 24–36 hours of life
68. On the fifth day of life, an infant is noted to
have a violaceous, circumscribed, subcutaneous
nodule immediately beneath fading forceps
marks on one cheek. The infant is otherwise
well appearing. Which of the following is the
most likely diagnosis?
(A) an abscess
(B)a hemangioma
(C) a pericytoma
(D) periorbital cellulitis caused by
Haemophilus influenzae
(E) subcutaneous fat necrosis
69. Your office is notified by the state screening
program that a newborn in your practice is pre-
sumped positive for hypothyroidism. Which
of the following is true of congenital hypothy-
roidism?
(A) Affected infants are usually clinically
apparent by the third day of life.
(B) Affected infants have unusually small
fontanelles.
(C) Affected infants appear thin.
(D) Prolonged hyperbilirubinemia is common.
(E) Affected infants usually have a goiter
present at birth.
70. Iron deficiency is the leading cause of anemia in
infancy and childhood. Which of the following
infants is at greatest risk of this early on?
(A) a premature infant
(B) an infant with ABO incompatibility
(C) an infant with physiologic hyperbiliru-
binemia
(D) a postmature infant
(E) an infant with polycythemia
71. A term infant has respiratory distress and a
blood count reveals a hematocrit of 65%. Your
resident states that fetal hemoglobin has an
increased affinity for oxygen as compared with
adult hemoglobin. What effect might this
result in?
(A) an increased mass of fetal hemoglobin
(B) thin red cell membrane in the fetus
(C) the presence of unconjugated bilirubin
in the red cell
(D) decreased binding with 2,3-diphospho-
glycerate (2,3-DPG)
(E) intracellular alkalosis
72. A term infant was born with the anomaly
shown in Figure 3-2. Which of the following is
the most likely cause of this infant’s mass
lesion?
 Questions: 66–75 59

(A) vitamin K deficiency

(B) factor VIII deficiency
(C) thrombocytopenia
(D) disseminated intravascular coagulation
(E) factor IX deficiency

74. You are a medical student evaluating a jaun-

diced infant in clinic. The infant appears
healthy and has been entirely breast-fed. The
total bilirubin level is 19 mg/dL, direct biliru-
bin is 0.2 mg/dL, and the hemoglobin is
normal. You have diagnosed “breast milk jaun-
Figure 3-2 dice.” Which of the following is the most typical
(Courtesy of Clarence Greene Jr., MD ) feature of breast milk jaundice?
(A) It is an unconjugated hyperbilirubinemia.
(B) It usually appears within the first 12 hours
(A) scalp abscess
of life.
(B) posterior hydrocephalus
(C) The elevated levels may persist for more
(C) encephalocele than 60 days.
(D) myelomeningocele (D) It is generally associated with moderate
(E) hydrocele anemia and reticulocytosis.
(E) It is uncommon in Caucasian infants.
73. An infant born with malformed forearms is
noted to develop severe hemorrhagic manifes- 75. You are counseling a diabetic teenager who
tations. Which of the following would be the states she is trying to get pregnant. She has not
most likely cause of the bleeding problem? been compliant with her diabetes management
over the past few years. You are trying to stress
the importance of glucose control prior to con-
ception and during pregnancy by explaining
the possible complications associated with
infants born to diabetic mothers. Which of the
following is the most common problem in
infants of diabetic mothers?
(A) birth asphyxia
(B) sacral agenesis
(C) hyponatremia
(D) hypoglycemia
(E) cardiac septal hypertrophy

Figure 3-3
(Courtesy of Doug C. Rivard, DO )
60 3: The Neonate
76. A 12-month-old infant with a cloudy cornea is
diagnosed with primary infantile glaucoma, a
rare disorder occurring in 1:10,000 live births.
You explain to the parents that it can be a major
cause of blindness. Which of the following state-
ments regarding infantile glaucoma is true?
(A) It is best treated with medical
management.
(B) It is frequently seen with trisomy 21.
(C) It presents with the white pupil sign.
(D) It is rarely diagnosed before 6 months of
age.
(E) It is associated with congenital rubella
and neurofibromatosis.
77. A premature infant is now 4 hours old and you
suspect respiratory distress syndrome (hyaline
membrane disease). Which of the following is
the least likely first clinical presentation of res-
piratory distress syndrome?
(A) tachypnea
(B) delayed capillary refill
(C) cyanosis
(D) wheezing
(E) pallor
78. Fetal heart rate monitoring is being used during
the labor of a 40-year-old primagravida who is
now 1 week post dates. Which of the following
would most commonly be seen in a healthy baby?
(A) heart rate 60–80 beats per minute
(B) accelerations of the heart rate
(C) heart rate below 60 beats per minute
(D) decreased beat-to-beat variability in
heart rate
(E) fixed heart rate
79. You are the resident on call and are called
urgently to the bedside of a newborn. The nurse
is concerned that the infant has developed a
tension pneumothorax. Which of the following
clinical signs most likely represent those seen in
a newborn with tension pneumothorax?
(A) cyanosis, apnea, and tachycardia
(B) apnea, hypertension, and bradycardia
(C) tachypnea, cyanosis, and bradycardia
(D) wheezing, tachycardia, and hypertension
(E) wheezing, hypotension, and apnea
80. You are evaluating an infant born at 39 weeks
gestation weighing 2000 g. Which of the fol-
lowing would be the appropriate classification
of this infant?
(A) low birth weight
(B) premature
(C) small for gestational age
(D) low birth weight and small for gesta-
tional age
(E) premature and small for gestational age
81. A term infant is now 10 hours of age and has a
seizure. Which of the following diagnoses is
more common among term rather than pre-
mature infants?
(A) intraventricular hemorrhage
(B) hemorrhagic disease of the newborn
(C) sepsis
(D) subdural hemorrhage
(E) congenital infection
82. A term neonate begins vomiting during the
first few days of life, and develops a distended
abdomen. The family history is positive for
cystic fibrosis. Which of the following condi-
tions, if found, would most likely be related to
the family history?
(A) annular pancreas
(B) duodenal atresia
(C) hypertrophic pyloric stenosis
(D) meconium ileus
(E) volvulus
83. An infant delivered precipitously to a mother
who had not received prenatal care. The infant
has finding suggestive of congenital syphilis.
Maternal and infant testing confirm this diag-
nosis. Which of the following is most sugges-
tive of early congenital syphilis?
(A) disseminated intravascular coagulation
(B) bullous lesions of the palms and soles
(C) hepatitis
 Questions: 76–93 61

(D) dermal erythropoiesis (A) bilateral renal agenesis

(E) pneumonia (B) atrioventricular canal defect
(C) esophageal atresia
84. Prior to the newborn screening program in (D) group B streptococcal infection
your state, you are evaluating an infant with a
(E) transposition of the great vessels
skeletal survey due to concerns of trauma.
Which of the following roentgenographic find-
88. Hydrocephalus, chorioretinitis, and diffuse cere-
ings in a newborn infant is most suggestive of
bral calcifications are present in a newborn male.
undiagosed hypothyroidism?
Of the following pets, which is most likely to be
(A) epiphyseal dysgenesis the source of this zoonotic congenital infection?
(B) absence of ossification of the hamate bone (A) dog
(C) prominent thymic shadow (B) cat
(D) osteoporosis (C) horse
(E) cardiomegaly (D) rabbit
(E) gerbil
85. A 5-day-old appropriate-for-gestational age
male presents with tachypnea, poor feeding,
DIRECTIONS (Questions 89 through 93): The
and lethargy. On examination, the neonate
following group of questions is preceded by a list
appears in shock with hypotension, pallor, and
of lettered answer options. For each question,
poor capillary refill. Among the following,
match the one lettered option that is most closely
which is the most likely diagnosis?
associated with the question. Each lettered option
(A) tetrology of Fallot may be selected once, multiple times, or not at all.
(B) tricuspid atresia
(C) transposition of the great vessels (A) trisomy 13 (Patau syndrome)
(D) truncus arteriosus (B) trisomy 18 (Edwards syndrome)
(E) hypoplastic left heart syndrome (C) 5p deletion (cri-du-chat syndrome)
(D) trisomy 21 (Down syndrome)
86. Incontinentia pigmenti (IP) is an X-linked (E) 45,X or XO (Turner syndrome)
hereditary disorder and is typically lethal in
males. Skin lesions may be present at birth. 89. Low-set ears, nail hypoplasia, “rocker-bottom”
Which of the following best describe the initial feet, growth retardation, severe mental retardation
lesions of incontinentia pigmenti?
(A) hypopigmented 90. Holoprosencephaly, microphthalmia or anoph-
(B) scaly thalmia, cleft lip and palate, polydactyly, severe
mental retardation
(C) waxy papules
(D) inflammatory bullae 91. Hypotonia and hyperextensible joints, clin-
(E) small vesicles odactyly, Brushfield spots, duodenal atresia,
flattening of the occiput
87. A newborn infant is noted to have a peculiar
face with low-set ears and widely spaced eyes 92. Microcephaly, mental retardation, hyper-
with epicanthic folds. The chin is receeding, and telorism, and cat-like (mewing) cry in
the nose is broad and flat. Oligohydramnios was infancy
noted prior to delivery. Examination of the pla-
cental membranes reveals amnion nodosum. 93. Redundant skin at nape of neck, low posterior
Which of the following diagnoses should be hairline, edema of dorsum of feet in newborn
suspected? period, cardiac and renal anomalies
62 3: The Neonate
DIRECTIONS (Questions 94 through 104): For each
of the multiple choice questions in this section select
the one lettered answer that is the best response in
each case.
94. A 42-week gestation neonate is born by
cesarean delivery following repeated fetal heart
rate decelerations. At rupture of membranes,
thick meconium was noted. The infant has no
respiratory effort but slight movement and a
heart rate of 90 beats per minute. Of the fol-
lowing, which is the first intervention?
(A) pass an umbilical artery catheter to
measure pH and Po2
(B) start bag-and-mask ventilation with
100% oxygen
(C) suction the mouth and trachea to
remove the meconium
(D) intubate the trachea and begin ventila-
tion with 100% oxygen
(E) establish monitoring with ECG and
pulse oximeter
95. An ultrasound at 33 weeks gestation reveals a
small-for-gestational-age fetus with no other
abnormalities. At birth at 39 weeks, the 1.6-kg
neonate requires no resuscitation. On examina-
tion, the infant is noted to be microcephalic and
have a generalized rash consisting of dozens of
blue-purple macules. His abdominal examina-
tion reveals hepatosplenomegaly. Which of the
following is the most likely diagnosis?
(A) group B streptococcal septicemia
(B) isoimmune thrombocytopenia
(C) congenital cytomegalovirus infection
(D) congenital toxoplasmosis
(E) congenital L monocytogenes infection
96. A term infant at 12 hours of age is normal
except for jaundice. Initial laboratory values
reveal a total bilirubin of 12 mg/dL and several
spherocytes on the peripheral blood smear.
Among the following, which is the most likely
diagnosis?
(A) hereditary elliptocytosis
(B) hereditary spherocytosis
(C) glucose-6-phosphate dehydrogenase
deficiency
(D) disseminated intravascular coagulopathy
(E) ABO isoimmune hemolytic disease
97. A 3.5-kg term male delivered by uncomplicated
vaginal delivery has a normal physical exami-
nation. On the day of discharge the mother tells
you that an older sibling has just broken out with
chicken pox. Which of the following is true?
(A) All newborns are very susceptible to
varicella upon exposure.
(B) This infant is likely already infected
because of the maternal exposure to the
sick sibling.
(C) Varicella immune globulin should
be administered to all infants in this
situation.
(D) If the mother had varicella in the past,
the infant is likely protected with pas-
sively acquired antibody from the
mother.
(E) The infant will not develop a typical
varicella infection, but will likely
develop varicella zoster.
98. A previously healthy term infant is brought to
clinic by his mother at 2 weeks of age because
of rapid breathing, poor breastfeeding, and
excessive sleeping. He is afebrile. Which of the
following is the most common arrhythmia at
this age?
(A) supraventricular tachycardia
(B) atrial flutter
(C) atrial fibrillation
(D) complete atrioventricular block
(E) ventricular tachycardia
99. A 1-hour-old, 33-week gestation male neonate
develops tachypnea, grunting respirations, and
deep intercostal retractions. A chest x-ray
reveals a homogeneously grainy appearance
to both lung fields. An arterial blood gas would
most likely reveal which of the following?
(A) respiratory alkalosis and hypoxemia
(B) respiratory acidosis and metabolic
alkalosis

(C) respiratory acidosis and hypoxemia
(D) respiratory and metabolic alkalosis
(E) metabolic acidosis and respiratory
alkalosis
100. A college student delivers an infant in the
campus housing area after denying her preg-
nancy and experiencing 6 hours of abdominal
pain. Paramedics arrive and find an infant they
estimate to be 4–5 lb in weight, crying but slightly
dusky. They administer oxygen. The newborn’s
temperature is 94°F. Hypothermia in this infant
would produce which of the following?
(A) decreased oxygen consumption
(B) hypertriglyceridemia
(C) hypercalcemia
(D) hypoglycemia
(E) metabolic alkalosis
101. A 30-day-old, former 24-weeks gestation, 600 g
neonate had a difficult initial respiratory course
complicated by a tension pneumothorax. She
had serial head ultrasound evaluations during
the first weeks of life. All previous studies
revealed a normal immature brain. Now, the
head ultrasound reveals an abnormality. Among
the following, which is most likely?
(A) grade II intraventricular hemorrhage
(B) grade IV intraventricular hemorrhage
(C) aqueductal stenosis
(D) periventricular leukomalacia
(E) vein of Galen aneurysm
102. A newborn infant has signs of congestive heart
failure. Physical examination does not reveal a
significant cardiac murmur. Auscultation of the
head reveals a loud cranial bruit. Which of the
following is the most likely diagnosis?
(A) polycythemia
(B) hyperthyroidism
(C) ruptured cerebral aneurysm
(D) transposition of the great vessels
(E) arteriovenous malformation of the great
vein of Galen
Questions: 94–104 63
103. A term 4.3-kg infant is delivered vaginally to a
33-year-old woman with juvenile-onset dia-
betes. The delivery was complicated by severe
shoulder dystocia and the infant experienced a
brachial plexus injury with limited movement
of the right arm. At 72 hours of age, the infant
is noted to be tachypneic but he is pink and
well perfused. Which of the following is the
most likely explanation for his tachypnea?
(A) respiratory distress syndrome (RDS)
(B) diaphragmatic paralysis
(C) pulmonary hemorrhage
(D) pneumothorax
(E) cystic adenomatoid malformation of the
lung
104. A term infant is delivered vaginally to an
18-year-old primagravida after an uncompli-
cated pregnancy. Mother presented with rup-
ture of membranes and a low-grade fever.
Labor and delivery proceeded rapidly, maternal
antibiotics were not administered prior to
delivery of the infant. Maternal screening
during pregnancy for group B streptococcus
was negative. At 30 hours of age the infant is
mottled, not feeding well, and cries when
handled. A lumbar puncture is performed and
the spinal fluid reveals 660 WBC/mm3 and a
CSF protein of 290 mg/dL. Which of the fol-
lowing is the most likely agent causing this
infant’s infection?
(A) group B streptococcus
(B) Escherichia coli
(C) Listeria monocytogenes
(D) Haemophilus influenzae
(E) Streptococcus pneumoniae