[Surgical excision of a hepatoma complicating chronic tyrosinemia (author's transl)].
A girl with chronic hereditary tyrosinemia is described in whom the diet caused an immediate resolution of tubular defect and rapid healing of the rickets. However cirrhosis was not prevented and at the age of 8 she developed a hepatoma. Complete surgical excision was possible and she remained well 15 months later. The value of blood alpha-fetoprotein levels and liver echotomography to monitor the course of the disease is emphasized. The apparent ineffectiveness of the low phenylalanine and tyrosine diet in preventing hepatic complications is discussed.
D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications*; D006528:Carcinoma, Hepatocellular / Q000150:complications / Q000601:surgery*; D002648:Child; D005260:Female; D006801:Humans; D008113:Liver Neoplasms / Q000150:complications / Q000601:surgery*; D014443:Tyrosine / Q000097:blood*
D014443:Tyrosine
Hépatome compliquant une forme chronique de tyrosinose. Evolution favorable quinze mois après l'exérèse chirurgicale.