A 15-month-old boy had recurrent hospital admissions because of fever, jaundice, subcutaneous bleeding, and hypoglycemic fist; on examination, there is hepatomegaly

(D). Tyrocinemia type 1 is a severe disease of the liver, kidney, and peripheral nerves are caused by a deficiency of the enzyme fumarylacetoacetate hydrolase. An acute hepatic crisis commonly heralds the onset of the disease and is usually precipitated by an intercurrent illness that produces a catabolic state. Episodes of acute peripheral neuropathy occur in approximately 40% of affected children. Renal nvolvement is manifested as a Fanconi-like syndrome