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Failure of liver transplantation to diminish cardiac deposits of amylopectin and leukocyte inclusions in type iv glycogen storage disease
Author(s) -
Rosenthal Philip,
Podesta Luis,
Grier Robert,
Said Jonathan W.,
Sher Linda,
Cocjin Jose,
Watanabe Frederick,
Vasiliauskas Eric,
Van De Velde Robert,
Makowka Leonard
Publication year - 1995
Publication title -
liver transplantation and surgery
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1074-3022
DOI - 10.1002/lt.500010607
Subject(s) - transplantation , glycogen storage disease , glycogen , liver transplantation , amylopectin , medicine , liver disease , pathology , gastroenterology , biology , biochemistry , amylose , starch
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.