A patient is investigated for renal failure. Which of the following condition is associated with boiled cabbage or rancid butter smelling urine in this patient?
Phenylketonuria
Tyrosinemia Type 1
Diabetic Ketoacidosis
Multiple carboxylase deficiency
Boiled cabbage or rancid butter smelling urine is seen in Tyrosinemia Type 1. Boiled cabbage smell occur due to substance 2 hydroxy butyric acid. Rancid butter smell occur due to 2 Keto 4 methiolbutyric acid. Tyrosinaemia type I is an inborn error of metabolism caused by deficiency of fumaryl acetoacetase which is the last enzyme in tyrosine degradation pathway. Accumulation of fumarylacetoacetate, maleylacetoacetate and succinylacetone causes liver and kidney damage.