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What is A 2 month old infant presented with hepatic crisis, peripheral neuropathy & fanconi like syndrome. Lab investigations showed elevated transaminase, low coagulation factors and elevated succinyl acetone in serum & urine. The most likely diagnosis : and explain in detail?
Tyrosine is derived from ingested proteins or is synthesized endogenously from phenylalanine. It is used for protein synthesis and is a precursor of dopamine, norepinephrine, epinephrine, melanin, and thyroxine. Tyrosinemia type I or Hereditary tyrosinemia or Hepatorenal tyrosinemia Caused by a deficiency of the enzyme fumarylacetoacetate hydrolase. Hepatic disease, peripheral neuropathy and renal involvement are hallmark of disease. The presence of elevated levels of succinylacetone in serum and urine is diagnostic