ATI RN
Pediatric Endocrine Exam Questions Questions
Question 1 of 5
Matching: Giant granules in granulocytes
Correct Answer: A
Rationale: Chédiak-Higashi syndrome features giant granules in neutrophils.
Question 2 of 5
A 10-day-old manifests profound coma one day after an illness characterized by emesis, poor oral intake, and hypotonia. Laboratory findings reveal 4+ ketonuria, an arterial blood gas of 6.9, PCO2 19, PO2 95, an anion gap of 37, and an absolute neutrophil count of 400. The most likely diagnosis is
Correct Answer: C
Rationale: Methylmalonic acidemia causes severe acidosis, ketonuria, and neutropenia during metabolic crises.
Question 3 of 5
During acute metabolic decompensation, all of the following are appropriate therapy EXCEPT
Correct Answer: B
Rationale: Albumin infusion is not standard for urea cycle defect crises; others help remove ammonia or provide energy.
Question 4 of 5
Matching: Disorder of peroxisomal import
Correct Answer: B
Rationale: Zellweger syndrome involves peroxisomal import defects.
Question 5 of 5
The best diagnostic test for the patient in the previous question is
Correct Answer: C
Rationale: Elevated VLCFAs confirm X-linked adrenoleukodystrophy.