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 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.
Gossmann
J
J
Medizinische Klinik II, Zentrum der Inneren Medizin, Klinikum der Johann Wolfgang Goethe-Universität, Theodor-Stern-Kai 7, 60590 Frankfurt a.M., Germany.
Scheuermann
E H
EH
Frilling
A
A
Geiger
H
H
Dietrich
C F
CF
D000236:Adenoma / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D000328:Adult; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D017809:Fatal Outcome; D005260:Female; D005953:Glycogen Storage Disease Type I / Q000150:complications*; D006801:Humans; D007676:Kidney Failure, Chronic / Q000150:complications / Q000601:surgery*; D016030:Kidney Transplantation; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000000981:diagnostic imaging / Q000601:surgery; D008297:Male; D011183:Postoperative Complications; D014463:Ultrasonography