Tomcat urine odor is seen in:: (A) Multiple carboxylase deficiency, (B) Phenylketonuria, (C) Hawkinuria, (D) Maple syrup disease
Answer is A. MULTIPLE CARBOXLASE DEFICIENCY - BIOTIN is coenzyme in carboxylase reaction. Defects in the ability to add or remove biotin from carboxylases result in multiple carboxylase deficiency. It is autosomal recessive Developmental delay Treatment is biotin supplement Tomcat Urine odour