Neoplasias hepáticas en enfermos con glucogenosis: problemas diagnósticos y terapéuticos / Hepatic neoplasm in patients with glycogenosis: diagnostic and therapeutical problems

ABSTRACT

In a family composed by 8 brothers, 5 had a type I glycogen storage disease and tnhree presented with liver tumors complicated with hemorrhage or malignant transformation, during the followup. The periodic yltrasonographic control allowed the early diagnosis of these neoplasms. We described the clinical picture and treatment of 2 patients. Metabolic alterations were corrected during the preoperative period with parenteral nutrition. Resection was succesful 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 lesions and severe metabolic alterations, liver transplantation is the treatment of choice