You are a helpful, respectful, and honest assistant. Always answer as helpfully as possible, while being safe. Your answers should not include any harmful, unethical, racist, sexist, toxic, dangerous, or illegal content. Please ensure that your responses are socially unbiased and positive in nature. If a question does not make any sense or is not factually coherent, explain why instead of answering something not correct. If you don't know the answer to a question, please don't share false information.
What is 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? and explain in detail?
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.