Hepatocellular carcinoma in an adult patient with type IV glycogen storage disease
| Journal | Volume 72 - 2009 |
| Issue | Fasc.3 - Letters |
| Author(s) | Ibrahim Koral Onal, Nesrin Turhan, Erkin Oztas, Mehmet Arhan, Zuhal Akcoren, Perihan Oguz, Meral Akdogan, Eda Demir Ona, Sabite Kacar, Mevlut Kurt, Nurgul Sasmaz |
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| (1) Department of Gastroenterology, (2) Department of Pathology, (4) Department of Gastroenterology, Turkiye Yuksek Ihtisas Education and Research Hospital ; (3) Department of Pediatrics, Pediatric Pathology Unit, (5) Department of Internal Medicine, Hacettepe University School of Medicine, Ankara, Turkey. |
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Type IV glycogen storage disease (type IV GSD), also known as Andersen disease or amylopectinosis, is a rare autosomal-recessive disorder caused by deficiency of the glycogen branching enzyme (1). The classic form is characterized by progressive hepatic fibrosis resulting in hepatosplenomegaly and failure to thrive and death by the age of 5 years. Few patients do not develop hepatic failure and survive to adulthood (2). Here we report a case of hepatocellular carcinoma (HCC) in a young adult with GSD IV and chronic liver disease following a 20 year period with no apparent disease progression. |
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© Acta Gastro-Enterologica Belgica. PMID 19902877 |
