An adolescent female develops fever, palpitation and disorientation. Examination shows exophthalmos, goiter, tachycardia and elevated blood pressure. Lab tests reveal elevated T4 and T3 with low TSH. Of the following, the BEST initial treatment is

Correct Answer: A

Rationale: Thyroid storm requires immediate symptom control; beta blockers (e.g., propranolol) rapidly manage tachycardia and palpitations, making them the best initial treatment before antithyroid drugs (B, C), radioiodine (D), or surgery (E).

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.

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.

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.

Correct Answer: C

Rationale: Cystinuria impairs reabsorption of cystine, lysine, arginine, and ornithine (dibasic amino acids), but tryptophan, a neutral amino acid, is not affected.