Robert Grier is now with Integrated Genetics, Santa Fe, NM.
Failure of liver transplantation to diminish cardiac deposits of amylopectin and leukocyte inclusions in type iv glycogen storage disease
Article first published online: 15 DEC 2005
Copyright © 1995 American Association for the Study of Liver Diseases
Liver Transplantation and Surgery
Volume 1, Issue 6, pages 373–376, November 1995
How to Cite
Rosenthal, P., Podesta, L., Grier, R., Said, J. W., Sher, L., Cocjin, J., Watanabe, F., Vasiliauskas, E., Van De Velde, R. and Makowka, L. (1995), Failure of liver transplantation to diminish cardiac deposits of amylopectin and leukocyte inclusions in type iv glycogen storage disease. Liver Transpl, 1: 373–376. doi: 10.1002/lt.500010607
- Issue published online: 15 DEC 2005
- Article first published online: 15 DEC 2005
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.