title: 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.affiliation: Liver Transplant Service, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
authors.forename: P
authors.initials: P
authors.lastname: Rosenthal
authors.forename: L
authors.initials: L
authors.lastname: Podesta
authors.forename: R
authors.initials: R
authors.lastname: Grier
authors.forename: J W
authors.initials: JW
authors.lastname: Said
authors.forename: L
authors.initials: L
authors.lastname: Sher
authors.forename: J
authors.initials: J
authors.lastname: Cocjin
authors.forename: F
authors.initials: F
authors.lastname: Watanabe
authors.forename: E
authors.initials: E
authors.lastname: Vasiliauskas
authors.forename: R
authors.initials: R
authors.lastname: van de Velde
authors.forename: L
authors.initials: L
authors.lastname: Makowka
mesh_terms: D000687:Amylopectin / Q000378:metabolism*; D001706:Biopsy; D017809:Fatal Outcome; D005500:Follow-Up Studies; D006011:Glycogen Storage Disease Type IV / Q000378:metabolism* / Q000473:pathology / Q000601:surgery; D006331:Heart Diseases / Q000378:metabolism*; D006801:Humans; D007223:Infant; D016031:Liver Transplantation; D008297:Male; D009206:Myocardium / Q000378:metabolism* / Q000648:ultrastructure; D009504:Neutrophils / Q000473:pathology*; D014057:Tomography, X-Ray Computed
chemical_list: D000687:Amylopectin