LIVER NEOPLASM IN PATIENTS WITH GLYCOGEN- STORAGE DISEASES - REPORT OF A FAMILY WITH THE DISEASE

In a family composed by eight brothers, five had a type I glycogen sto rage disease and three presented with liver tumors complicated with he morrhage or malignant transformation, during the follow up. The period ic ultrasonographic control allowed the early diagnosis of these neopl asms. We describe the clinical picture and treatment of two patients. Metabolic alterations were corrected during the preoperative period wi th parenteral nutrition. Resection was successful in both cases. It is concluded that a close follow up and early treatment of these lesions is effective and avoids complications. In patients with multiple lesi ons and severe metabolic alterations, liver transplantation is the tre atment of choice.