Progressive cardiac failure following orthotopic liver transplantation for type IV glycogenosis

E. M. Sokal, F. Van Hoof, D. Alberti, J. de Ville de Goyet, T. de Barsy, J. B. Otte

Research output: Contribution to journalArticlepeer-review

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.

Original languageEnglish
Pages (from-to)200-203
Number of pages4
JournalEuropean Journal of Pediatrics
Volume151
Issue number3
DOIs
Publication statusPublished - Mar 1992

Keywords

  • Inborn error metabolism
  • Liver cirrhosis
  • Metabolic diseases
  • Orthotopic liver transplantation
  • Type IV Glycogenosis

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health

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