Cushing syndrome in children has many causes, either exogenous or endogenous. Of the following, the LEAST likely endogenous cause in children is

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Endocrine System Pediatrics Questions

Question 1 of 5

Cushing syndrome in children has many causes, either exogenous or endogenous. Of the following, the LEAST likely endogenous cause in children is

Correct Answer: C

Rationale: Nodular adrenal hyperplasia is rare in children as an endogenous cause of Cushing syndrome, unlike adrenal tumors (A, B), pituitary microadenomas (D), or ectopic ACTH tumors (E), which are more common.

Question 2 of 5

A 5-month-old infant presented with symmetrical profound muscle weakness, tachypnea, tachycardia, and poor feeding. His chest x-ray showed cardiomegaly and his ECG revealed short PR interval. Of the following, the MOST likely glycogen storage disease is

Correct Answer: C

Rationale: Type II (Pompe) causes profound muscle weakness, cardiomyopathy (cardiomegaly), and respiratory issues due to glycogen accumulation in muscles and heart, with short PR interval on ECG, matching this presentation.

Question 3 of 5

A constant threat of homocystinuria is

Correct Answer: D

Rationale: Homocystinuria’s constant threat is arterial thromboses due to homocysteine’s vascular toxicity, a life-threatening complication outweighing skeletal (B, C), ocular (A), or cognitive (D) issues.

Question 4 of 5

Sudden infant death syndrome is reported in infants with

Correct Answer: C

Rationale: Medium-chain acyl-CoA dehydrogenase (MCAD) deficiency is a known cause of SIDS due to hypoketotic hypoglycemia during fasting stress, more commonly than other fatty acid oxidation disorders.

Question 5 of 5

Lipidoses affect many solid organs, cherry-red spots is seen in

Correct Answer: D

Rationale: GM2 gangliosidosis (Tay-Sachs) classically features cherry-red spots in the macula from lipid accumulation in retinal ganglion cells, a hallmark finding.

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