ATI RN
Endocrine System Pediatric Questions
Question 1 of 5
A 15-year-old boy is brought to clinic by his parents because of delayed puberty. His growth chart reveals a height just below but parallel to the 5th centile. Examination is unremarkable. Bone age is consistent with a 12 year chronological age. Lab investigations including hormonal study are normal. Of the following, the MOST likely diagnosis is
Correct Answer: D
Rationale: Constitutional growth delay involves delayed puberty and growth with normal labs and a delayed bone age, matching this case. Hypothyroidism (A), Klinefelter (B), hypogonadism (C), and craniopharyngioma (D) typically show abnormal labs or physical findings.
Question 2 of 5
A 3-week-old neonate develops repeated vomiting and dehydration. Examination reveals ambiguous genitalia with fusion of the anterior portion of the labioscrotal folds. Ultrasonography reveals presence of uterus, ovaries, and fallopian tubes. Of the following, the MOST valuable test to establish the diagnosis is
Correct Answer: A
Rationale: Elevated 17-hydroxyprogesterone confirms congenital adrenal hyperplasia (e.g., 21-hydroxylase deficiency), common in 46,XX neonates with ambiguous genitalia and salt-wasting, as seen here. Other tests (B, C, D, E) are less diagnostic initially.
Question 3 of 5
Protein intake is typically restricted in which of the following populations?
Correct Answer: B
Rationale: Protein intake is typically not restricted in pediatric CKD (A) to support growth, but is restricted in adult CKD (B) to reduce uremic toxins. VUR (C) and ARPKD (D) do not typically require protein restriction.
Question 4 of 5
Any organ or system can be injured by toxic accumulation of any of the metabolites involved in inborn errors. Of the following, the metabolic disease causing hepatic failure is
Correct Answer: C
Rationale: Glycogen storage disease IV (Andersen) causes hepatic failure due to amylopectin-like polysaccharide accumulation leading to cirrhosis. GSD I (A) and III (B) affect liver function but rarely cause failure, while MPS I (D) and II (E) primarily affect other systems.
Question 5 of 5
Individuals homozygous for galactokinase deficiency usually develop
Correct Answer: B
Rationale: Galactokinase deficiency leads to galactose accumulation forming galactitol, causing cataracts after the neonatal period. It lacks the severe systemic effects (A, D, E) or cognitive impact (C) of classic galactosemia.