Glycogen storage disease type III-hepatocellular carcinoma a long-term complication?
E Demo, D Frush, M Gottfried, J Koepke, A Boney… - Journal of …, 2007 - Elsevier
BACKGROUND/AIMS: Glycogen storage disease III (GSD III) is caused by a deficiency of
glycogen-debranching enzyme which causes an incomplete glycogenolysis resulting in
glycogen accumulation with abnormal structure (short outer chains resembling limit dextrin)
in liver and muscle. Hepatic involvement is considered mild, self-limiting and improves with
age. With increased survival, a few cases of liver cirrhosis and hepatocellular carcinoma
(HCC) have been reported. METHODS: A systematic review of 45 cases of GSD III at our …
glycogen-debranching enzyme which causes an incomplete glycogenolysis resulting in
glycogen accumulation with abnormal structure (short outer chains resembling limit dextrin)
in liver and muscle. Hepatic involvement is considered mild, self-limiting and improves with
age. With increased survival, a few cases of liver cirrhosis and hepatocellular carcinoma
(HCC) have been reported. METHODS: A systematic review of 45 cases of GSD III at our …
