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Liver cirrhosis in glycogen storage disease Ib |
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| Authors: | Fabian Baertling Ertan Mayatepek Patrick Gerner Hideo A Baba Julia Franzel Andrea Schlune Thomas Meissner |
| Institution: | 1. Department of General Pediatrics, Neonatology and Pediatric Cardiology, University Children''s Hospital Duesseldorf, Heinrich Heine University, Moorenstr. 5, D-40225 Duesseldorf, Germany;2. Department of Pediatric Gastroenterology, Transplant Medicine, Nephrology and Endocrinology, University Hospital Essen, Hufelandstr. 55, D-45122 Essen, Germany;3. Department of Pathology and Neuropathology, University Hospital Essen, Hufelandstr. 55, D-45122 Essen, Germany |
| Abstract: | Glycogen storage disease Ib is an inborn error of carbohydrate metabolism leading to impaired glycogenolysis and gluconeogenesis. Cardinal symptoms include fasting hypoglycemia, lactic acidosis and hepatomegaly as well as neutropenia. We report for the first time on the development of liver cirrhosis in a nine-year-old boy in the course of glycogen storage disease Ib and discuss possible underlying pathomechanisms. |
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