Multiple adenomas and hepatocellular carcinoma in a renal transplant patient with glycogen storage disease type 1A (von Gierke disease)

We report on a 42-year-old female patient with glycogen storage disease typ e la (von Gierke disease, GSD 1a) who developed hepatic adenomas and finall y a hepatocellular carcinoma 10 years after renal transplantation. The tumo r was resected; however, the patient died 6 months later as a result of ful minant carcinoma recurrence. In patients who have GSD la with terminal rena l failure, combined liver and. kidney transplantation may be considered at an early stage of the disease.