Failure of liver transplantation to diminish cardiac deposits of amylopectin and leukocyte inclusions in type IV glycogen storage disease.

Abstract

Orthotopic liver transplantation has been used to treat glycogen storage disease type IV. Most long-term surviving patients who have undergone liver transplantation have been free of neuromuscular and cardiac morbidity, and regression of cardiac amylopectin infiltration has been reported after liver transplantation. Leukocyte inclusions in glycogen storage disease type IV have also been reported. We present the case of a child who underwent orthotopic liver transplantation for glycogen storage disease type IV. In contrast to previous reports, at autopsy 2 1/2 years after transplantation, there was massive amylopectin deposits in his heart. Further, peripheral leukocytes never showed loss of amylopectin inclusions after transplantation. Orthotopic liver transplantation for type IV glycogen storage disease may not, in all cases, result in improvement in other affected organs. Consideration of multiorgan transplantation appears warranted.

• Authors: P Rosenthal, L Podesta, R Grier, J W Said, L Sher, J Cocjin, F Watanabe, E Vasiliauskas, R van de Velde, L Makowka
• Journal: Liver transplantation and surgery : official publication of the American Association for the Study of Liver Diseases and the International Liver Transplantation Society (Liver Transpl Surg) Vol. 1 Issue 6 Pg. 373-6 (Nov 1995) ISSN: 1074-3022 [Print] United States
• PMID: 9346615 (Publication Type: Case Reports, Journal Article)

Chemical References

• Amylopectin

Topics

• Amylopectin (metabolism)
• Biopsy
• Fatal Outcome
• Follow-Up Studies
• Glycogen Storage Disease Type IV (metabolism, pathology, surgery)
• Heart Diseases (metabolism)
• Humans
• Infant
• Liver Transplantation
• Male
• Myocardium (metabolism, ultrastructure)
• Neutrophils (pathology)
• Tomography, X-Ray Computed