{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,2]],"date-time":"2026-03-02T16:55:46Z","timestamp":1772470546218,"version":"3.50.1"},"reference-count":20,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"12","license":[{"start":{"date-parts":[[2009,12,1]],"date-time":"2009-12-01T00:00:00Z","timestamp":1259625600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2009,11,24]],"date-time":"2009-11-24T00:00:00Z","timestamp":1259020800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"Scientific Research Fund of the Japanese Ministry of Education"},{"name":"Research Grant for Immunology, Allergy, and Organ Transplant"},{"name":"Japanese Ministry of Health, Labor, and Welfare","award":["17591358"],"award-info":[{"award-number":["17591358"]}]}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2009,12]]},"abstract":"<jats:sec>\n                    <jats:title\/>\n                    <jats:p>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. \u00a9 2009 AASLD.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/lt.21929","type":"journal-article","created":{"date-parts":[[2009,11,24]],"date-time":"2009-11-24T14:48:00Z","timestamp":1259074080000},"page":"1867-1871","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":32,"title":["Living Donor Liver Transplantation for Glycogen Storage Disease Type Ib"],"prefix":"10.1097","volume":"15","author":[{"given":"Mureo","family":"Kasahara","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Reiko","family":"Horikawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Seisuke","family":"Sakamoto","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takanobu","family":"Shigeta","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hideaki","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akinari","family":"Fukuda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kiyomi","family":"Abe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Keisuke","family":"Yoshii","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasuhiro","family":"Naiki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rika","family":"Kosaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atsuko","family":"Nakagawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"276","reference":[{"key":"R1-27-20260302","doi-asserted-by":"crossref","first-page":"661","DOI":"10.1016\/S0021-9258(18)38504-1","article-title":"Glucose-6-phosphatase in the liver in glycogen storage disease.","volume":"199","author":"Cori","year":"1992","journal-title":"J Biol Chem"},{"key":"R3-27-20260302","doi-asserted-by":"crossref","first-page":"426","DOI":"10.1006\/bbrc.1998.8985","article-title":"Molecular analysis of glycogen storage disease type 1b: identification of a prevalent mutation among Japanese patients and assignment of putative glucose-6-phosphate translocase gene to chromosome 11.","volume":"248","author":"Kure","year":"1998","journal-title":"Biochem Biophys Res Commun"},{"key":"R5-27-20260302","doi-asserted-by":"crossref","first-page":"S33","DOI":"10.1007\/BF02072085","article-title":"Defective neutrophil and monocyte functions in glycogen storage disease type 1b: a literature review.","volume":"152","author":"Gitzelmann","year":"1993","journal-title":"Eur J Pediatr"},{"key":"R6-27-20260302","doi-asserted-by":"crossref","first-page":"S20","DOI":"10.1007\/BF02679990","article-title":"Glycogen storage disease type 1: diagnosis, management, clinical course and outcome. Results of the European Study on Glycogen Storage Disease Type 1 (ESGSD1).","volume":"161","author":"Rake","year":"2002","journal-title":"Eur J Pediatr"},{"key":"R7-27-20260302","doi-asserted-by":"crossref","first-page":"1005","DOI":"10.1016\/S0022-3476(05)80403-2","article-title":"Treatment with lenograstim (glycosylated recombinant human granulocyte colony-stimulating factor) and orthotopic liver transplantation for glycogen storage disease type 1b.","volume":"123","author":"Lachaux","year":"1993","journal-title":"J Pediatr"},{"key":"R8-27-20260302","doi-asserted-by":"crossref","first-page":"419","DOI":"10.1002\/hep.1840150311","article-title":"Orthotopic liver transplantation for urea cycle enzyme deficiency.","volume":"15","author":"Todo","year":"1992","journal-title":"Hepatology"},{"key":"R9-27-20260302","doi-asserted-by":"crossref","first-page":"539","DOI":"10.1023\/B:BOLI.0000037400.49488.20","article-title":"The benefits of liver transplantation in glycogenosis type 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