Which of the following inborn error of metabolism has porphyria like clinical features?: (A) Phenylketonuria, (B) Tyrosinemia type I, (C) Alkaptonuria, (D) Metachromatic leukodystrophy
Answer is B. Succinylacetone accumulates in hereditary tyrosinemia type I (fumarylacetoacetate hydrolase deficiency). Succinylacetone is structurally similar to ALA and inhibits ALA dehydratase leading to porphyria like features.