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Living donor liver transplantation for glycogen storage disease type Ib
Author(s) -
Kasahara Mureo,
Horikawa Reiko,
Sakamoto Seisuke,
Shigeta Takanobu,
Tanaka Hideaki,
Fukuda Akinari,
Abe Kiyomi,
Yoshii Keisuke,
Naiki Yasuhiro,
Kosaki Rika,
Nakagawa Atsuko
Publication year - 2009
Publication title -
liver transplantation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.814
H-Index - 150
eISSN - 1527-6473
pISSN - 1527-6465
DOI - 10.1002/lt.21929
Subject(s) - medicine , glycogen storage disease type i , glycogen storage disease , liver transplantation , neutropenia , transplantation , gastroenterology , type 1 diabetes , surgery , diabetes mellitus , glycogen , endocrinology , toxicity
Glycogen storage disease type 1b (GSD‐1b) is due to an autosomal recessive inborn error of carbohydrate metabolism caused by defects in glucose‐6‐phosphatase translocase. Patients with GSD‐1b have severe hypoglycemia with several clinical manifestations of hepatomegaly, obesity, a doll‐like face, and neutropenia. Liver transplantation has been indicated for severe glucose intolerance. This study retrospectively reviewed 4 children with a diagnosis of GSD‐1b who underwent living‐donor liver transplantation (LDLT). Between November 2005 and June 2008, 96 children underwent LDLT with overall patient and graft survival of 92.3%. Of these, 4 (4.2%) were indicated for GSD‐1b. All patients are doing well with an excellent quality of life because of the stabilization of glucose intolerance, decreased hospital admission, and normalized neutrophil count. LDLT appears to be a feasible option and is associated with a better quality of life for patients with GSD‐1b. Long‐term observation may be necessary to collect sufficient data to confirm the efficacy of this treatment modality. Liver Transpl 15:1867–1871, 2009. © 2009 AASLD.