Hepatocellular carcinoma in type I glycogen storage disease - PubMed (original) (raw)

Case Reports

. 1988 May-Jun;8(3):531-7.

doi: 10.1002/hep.1840080317.

Affiliations

Case Reports

Hepatocellular carcinoma in type I glycogen storage disease

J Limmer et al. Hepatology. 1988 May-Jun.

Abstract

Patients suffering from Type I glycogen storage disease frequently develop hepatic tumors. Some of these were classified as carcinoma, with the majority of tumors representing benign adenomata. However, no evidence exists of malignant transformation of adenomata in these patients. Here, we describe the occurrence of a hepatocellular carcinoma in the adenomata-bearing liver of the elder of two sisters suffering from Type I glycogen storage disease at the age of 20 years, 6 years after the diagnosis had been made. Surprisingly, alpha-fetoprotein levels were normal throughout the entire course of this patient, whereas the younger sister had elevated levels despite the absence of malignant lesions. Thus, the clinical significance of alpha-fetoprotein remains unclear in both cases. Nocturnal feeding, although performed continuously over the 6 years after the diagnosis, had obviously failed to prevent the development of hepatic tumors in both patients.

PubMed Disclaimer

Publication types

MeSH terms

Substances

LinkOut - more resources