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.

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.

Correct Answer: C

Rationale: MPS III (Sanfilippo) typically lacks hepatosplenomegaly, focusing on neurological deterioration, unlike other MPS types where organomegaly is common.

Correct Answer: B

Rationale: In Edwards syndrome, you find rocker-bottom feet, hypoplastic nails, and characteristic clenching of fists (second and fifth digits overlap third and fourth), distinguishing B as a key feature.

Correct Answer: A

Rationale: Methionine malabsorption (oasthouse syndrome) produces a cabbage-like or malty odor from unmetabolized methionine.