Q 1:
A
full-term male newborn is admitted to the regular nursery after an uneventful
delivery. Apgar scores are 8 at 1 minute and 9 at 5 minutes. He feeds well
initially, but 32 hours after birth you are called by the nurse because he is
feeding poorly and seems “lethargic.” You arrange for transfer of the newborn
to the intensive care nursery, where the resident on duty obtains a blood
culture and provides appropriate antibiotic coverage.
Of
the following, if sepsis is ruled out, the test MOST likely to be diagnostic in
this newborn is
A. a complete blood cell count with differential
B. an electroencephalogram
C. head ultrasonography
D. liver function tests
E. serum ammonia and urine organic
acids
Answer:
E
The child in this vignette is a healthy, full-term newborn who has developed mental status changes within 48 hours after birth. Although sepsis is the most likely diagnosis in this scenario, the newborn’s presentation is also typical of an inborn error of metabolism, such as a urea cycle defect or organic acidemia. Therefore, serum ammonia and urine organic acid testing should be considered and done in parallel with blood cultures and antibiotic administration. Early identification of an inborn error with immediate treatment may minimize potential neurologic sequelae from complications such as severe metabolic acidosis or coma secondary to hyperammonemia.
--------------
Q 2
An infant who was born at 26 weeks’ gestation, weighing 700 g, is nearing 37 weeks corrected
age. He is receiving nasal cannula oxygen (0.2 L/min) and being treated with diuretics for
chronic lung disease. He has no intracranial hemorrhage and is growing well on enteral feedings
via a nasogastric tube. Attempts at oral feeding have been unsuccessful because of frequent
oxygen desaturation, bradycardia, and concerns over the inability to coordinate suckingswallowing.
The mother asks why you are continuing to feed her son via a feeding tube.
Of the following, the condition that BEST explains why her son has feeding problems is
A. apnea of prematurity
B. chronic lung disease
C. gastroesophageal reflux
D. necrotizing enterocolitis
E. tracheoesophageal fistula
Answer
B
The infant described in the vignette has CLD and demonstrates problems in coordinating breathing with suckling and swallowing. Accordingly, he continues to be assisted with nasogastric tube feedings despite reaching a postconceptive age of 37 weeks.
Apnea of prematurity is not present; the infant has passed the gestational age equivalent of prematurity.
Gastroesophageal reflux does not occur during a feeding but typically presents with postprandial
mesis.
Necrotizing enterocolitis is a gastrointestinal disease associated with systemic decompensation and an acute abdomen. Tracheoesophageal fistula (TEF) is associated with respiratory distress and secretion control problems during intervals between feeding; the rare H type fistula with an intact esophagus (accounting for <5% of all TEFs) typically does not present in the newborn period.
-----------------
Q 3
You admitted a 2,000-g term newborn to the neonatal intensive care unit. Her Apgar scores
were 4 and 6 at 1 and 5 minutes, respectively. She exhibits seizure activity at 24 hours of age.
Physical examination reveals microcephaly , respiratory distress requiring assisted
ventilation, hepatosplenomegaly, cutaneous petechiae, and lethargy. Laboratory tests
demonstrate thrombocytopenia, anemia, elevated liver transaminases, and hyperbilirubinemia.
Of the following, the viral agent MOST likely to cause these findings is
A. adenovirus
B. Coxsackievirus B
C. cytomegalovirus
D. hepatitis C virus
E. herpes simplex virus
Answer:
C
The infant described in the vignette has findings consistent with symptomatic congenital
CMV infection: intrauterine growth restriction; seizures, which are common in association with
microcephaly; and intracranial calcifications. Respiratory distress may be due to
pneumonitis, and hepatopathy may include elevated serum transaminase concentrations,
coagulopathy, and persistent jaundice. Bone marrow suppression is not uncommon, with
thrombocytopenia resulting in petechiae . If anemia is profound, extramedullary
hematopoiesis may manifest with hepatomegaly, splenomegaly , and cutaneous
nodules (“blueberry muffin” spots).
---
Q 4
You are evaluating a newborn who has complete heart block and several 1.5-cm erythematous
macules and annuli located on the forehead, behind the ears, and in the scalp.
Of the following, the test MOST likely to confirm the infant’s diagnosis is
A. anti-Ro (SSA) antibodies
B. creatine kinase
C. hepatic function profile
D. platelet count
E. urinalysis
Answer
A
The infant described in the vignette has complete heart block and erythematous annular plaques
on sun-exposed areas, suggesting a diagnosis of neonatal lupus erythematosus (NLE). NLE is
a rare disorder caused by transplacental passage of maternal autoantibodies. In the majority of
cases (95%), the antibodies responsible are anti-Ro (SSA) antibodies alone or in association
with anti-La (SSB) antibodies. A small proportion of patients and their mothers exhibit anti-
U1RNP antibodies. Performance of these antibody studies on the infant and mother (if she has
not been evaluated previously) help confirm the diagnosis.
Answer:
E
The child in this vignette is a healthy, full-term newborn who has developed mental status changes within 48 hours after birth. Although sepsis is the most likely diagnosis in this scenario, the newborn’s presentation is also typical of an inborn error of metabolism, such as a urea cycle defect or organic acidemia. Therefore, serum ammonia and urine organic acid testing should be considered and done in parallel with blood cultures and antibiotic administration. Early identification of an inborn error with immediate treatment may minimize potential neurologic sequelae from complications such as severe metabolic acidosis or coma secondary to hyperammonemia.
--------------
Q 2
An infant who was born at 26 weeks’ gestation, weighing 700 g, is nearing 37 weeks corrected
age. He is receiving nasal cannula oxygen (0.2 L/min) and being treated with diuretics for
chronic lung disease. He has no intracranial hemorrhage and is growing well on enteral feedings
via a nasogastric tube. Attempts at oral feeding have been unsuccessful because of frequent
oxygen desaturation, bradycardia, and concerns over the inability to coordinate suckingswallowing.
The mother asks why you are continuing to feed her son via a feeding tube.
Of the following, the condition that BEST explains why her son has feeding problems is
A. apnea of prematurity
B. chronic lung disease
C. gastroesophageal reflux
D. necrotizing enterocolitis
E. tracheoesophageal fistula
Answer
B
The infant described in the vignette has CLD and demonstrates problems in coordinating breathing with suckling and swallowing. Accordingly, he continues to be assisted with nasogastric tube feedings despite reaching a postconceptive age of 37 weeks.
Apnea of prematurity is not present; the infant has passed the gestational age equivalent of prematurity.
Gastroesophageal reflux does not occur during a feeding but typically presents with postprandial
mesis.
Necrotizing enterocolitis is a gastrointestinal disease associated with systemic decompensation and an acute abdomen. Tracheoesophageal fistula (TEF) is associated with respiratory distress and secretion control problems during intervals between feeding; the rare H type fistula with an intact esophagus (accounting for <5% of all TEFs) typically does not present in the newborn period.
-----------------
Q 3
You admitted a 2,000-g term newborn to the neonatal intensive care unit. Her Apgar scores
were 4 and 6 at 1 and 5 minutes, respectively. She exhibits seizure activity at 24 hours of age.
Physical examination reveals microcephaly , respiratory distress requiring assisted
ventilation, hepatosplenomegaly, cutaneous petechiae, and lethargy. Laboratory tests
demonstrate thrombocytopenia, anemia, elevated liver transaminases, and hyperbilirubinemia.
Of the following, the viral agent MOST likely to cause these findings is
A. adenovirus
B. Coxsackievirus B
C. cytomegalovirus
D. hepatitis C virus
E. herpes simplex virus
Answer:
C
The infant described in the vignette has findings consistent with symptomatic congenital
CMV infection: intrauterine growth restriction; seizures, which are common in association with
microcephaly; and intracranial calcifications. Respiratory distress may be due to
pneumonitis, and hepatopathy may include elevated serum transaminase concentrations,
coagulopathy, and persistent jaundice. Bone marrow suppression is not uncommon, with
thrombocytopenia resulting in petechiae . If anemia is profound, extramedullary
hematopoiesis may manifest with hepatomegaly, splenomegaly , and cutaneous
nodules (“blueberry muffin” spots).
---
Q 4
You are evaluating a newborn who has complete heart block and several 1.5-cm erythematous
macules and annuli located on the forehead, behind the ears, and in the scalp.
Of the following, the test MOST likely to confirm the infant’s diagnosis is
A. anti-Ro (SSA) antibodies
B. creatine kinase
C. hepatic function profile
D. platelet count
E. urinalysis
Answer
A
The infant described in the vignette has complete heart block and erythematous annular plaques
on sun-exposed areas, suggesting a diagnosis of neonatal lupus erythematosus (NLE). NLE is
a rare disorder caused by transplacental passage of maternal autoantibodies. In the majority of
cases (95%), the antibodies responsible are anti-Ro (SSA) antibodies alone or in association
with anti-La (SSB) antibodies. A small proportion of patients and their mothers exhibit anti-
U1RNP antibodies. Performance of these antibody studies on the infant and mother (if she has
not been evaluated previously) help confirm the diagnosis.
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