Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis |
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Authors: | E M Sokal F Van Hoof D Alberti J de Ville de Goyet T de Barsy J B Otte |
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Institution: | (1) Department of Paediatrics, Université Catholique de Louvain, Hôpital St. Luc 1301, 10 av Hippocrate, B-1200 Brussels, Belgium;(2) Laboratory of Physiological Chemistry and International Institute of Cellular and Molecular Pathology, Université Catholique de Louvain, Hôpital St. Luc 1301, 10 av Hippocrate, B-1200 Brussels, Belgium;(3) Department of Paediatric Surgery, Université Catholique de Louvain, Hôpital St. Luc 1301, 10 av Hippocrate, B-1200 Brussels, Belgium |
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Abstract: | Orthotopic liver transplantation (OLT) has been proposed to treat patients with type IV glycogenosis because of early progressive cirrhosis. Reports have shown absence of disease progression in other organs after OLT and even regression of cardiac amylopectin infiltration in one case. We describe a 15-month-old child in whom a liver transplant was performed for type IV glycogenosis. There were no clinical signs of extrahepatic disease before OLT. Nine months later, the patient developed progressive cardiac insufficiency and died from cardiac failure. Because of massive amylopectin deposits, decreased myofibrils in cardiac cells, and exclusion of other causes of cardiac failure, death was attributed to amylopectinosis. Our observation contrasts with the Pittsburgh experience and suggests that cardiac amylopectionosis may progress after OLT. |
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Keywords: | Type IV Glycogenosis Orthotopic liver transplantation Metabolic diseases Liver cirrhosis Inborn error metabolism |
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