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

Transplantation. 2001 Jul 27;72(2):343-4. doi: 10.1097/00007890-200107270-00033.


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

Publication types

  • Case Reports

MeSH terms

  • Adenoma / diagnosis*
  • Adenoma / diagnostic imaging
  • Adenoma / surgery
  • Adult
  • Carcinoma, Hepatocellular / diagnosis*
  • Carcinoma, Hepatocellular / diagnostic imaging
  • Carcinoma, Hepatocellular / surgery
  • Fatal Outcome
  • Female
  • Glycogen Storage Disease Type I / complications*
  • Humans
  • Kidney Failure, Chronic / complications
  • Kidney Failure, Chronic / surgery*
  • Kidney Transplantation*
  • Liver Neoplasms / diagnosis*
  • Liver Neoplasms / diagnostic imaging
  • Liver Neoplasms / surgery
  • Male
  • Postoperative Complications
  • Ultrasonography