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Tyrosenemia (Tyrosinosis)

Fig: Biochemical pathway of metabolism of phenylalanine and tyrosine


  1. Deficiency of the enzyme fumarylaceacetate Hydrolase. Q
  2. An acute hepatic crisis :Hepatomegaly, Jaundice, elevated hepatocedlular carcinoma
  3. Acute peripheral neuropathy(40%) characterized be severe pain with hypertonia, marked weakness and paralysis.
  4. Renal involvement is manifested as a fanconi- like syndrome.
  5. Urine have boiled cabbage or rotten cabbage like odour. Q
  6. Diagnosis is established be measurement FAH activity in liver biopsy.
  7. A diet low in tyrosine, phenylalanine and methionine is advised.
  8. Definitive treatment : Liver transplantation. Q

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