Glycogen storage disease type III-hepatocellular carcinoma a long-term complication?

@article{Demo2007GlycogenSD,
  title={Glycogen storage disease type III-hepatocellular carcinoma a long-term complication?},
  author={Erin Demo and Donald R Frush and Marcia R. Gottfried and John P. Koepke and Anne Boney and Deeksha M. Bali and Yu T Chen and Priya Kishnani},
  journal={Journal of hepatology},
  year={2007},
  volume={46 3},
  pages={492-8}
}
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… CONTINUE READING
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