R. Rossi et al., LIVER NEOPLASM IN PATIENTS WITH GLYCOGEN- STORAGE DISEASES - REPORT OF A FAMILY WITH THE DISEASE, Revista Medica de Chile, 124(2), 1996, pp. 225-230
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.