{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T01:20:27Z","timestamp":1787016027121,"version":"build-2736575974"},"reference-count":9,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2020,10,12]],"date-time":"2020-10-12T00:00:00Z","timestamp":1602460800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2021,3]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Patients with GSD type 1 (von Gierke disease) are initially managed medically to maintain normoglycemia. However, if they do not achieve good metabolic control, LT is then considered. We describe the long\u2010term outcome of 6 children with GSD type 1 who underwent LT. Retrospective chart review of the data of 6 children with GSD type 1 who underwent LT at National University Hospital, Singapore, from May 1998 to October 2018, was performed. The median (IQR) age at diagnosis of the GSD was 1\u00a0year (0.92\u20105.50) and at transplant was 13.88\u00a0years (11.46\u201016.38). All of the patients had elevated liver enzymes, hypercholesterolemia, hypertriglyceridemia, and hyperlactatemia prior to transplant. All of the patients are alive at the time of analysis and follow\u2010up. None of them required a re\u2010transplant. For the three patients who had hypoglycemia pretransplant, there was no recurrence post\u2010transplant. All of the patients had normalization of liver enzymes by 1\u00a0year post\u2010transplant. Long\u2010term outcome of patients with GSD who underwent LT has been positive with improvement in metabolic control for most patients. We report the unusual finding of two siblings with persistent hyperuricemia post\u2010transplant requiring allopurinol.<\/jats:p>","DOI":"10.1111\/petr.13872","type":"journal-article","created":{"date-parts":[[2020,10,12]],"date-time":"2020-10-12T16:12:40Z","timestamp":1602519160000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Long\u2010term outcome after liver transplantation in children with type 1 glycogen storage disease"],"prefix":"10.1111","volume":"25","author":[{"given":"Wing Yan","family":"Yuen","sequence":"first","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Seng Hock","family":"Quak","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System Singapore Singapore"},{"name":"Department of Paediatrics Yong Loo Lin School of Medicine National University of Singapore Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marion M.","family":"Aw","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System Singapore Singapore"},{"name":"Department of Paediatrics Yong Loo Lin School of Medicine National University of Singapore Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2887-8248","authenticated-orcid":false,"given":"Sivaramakrishnan Venkatesh","family":"Karthik","sequence":"additional","affiliation":[{"name":"Khoo Teck Puat\u2010National University Children's Medical Institute National University Health System Singapore Singapore"},{"name":"Department of Paediatrics Yong Loo Lin School of Medicine National University of Singapore Singapore Singapore"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2020,10,12]]},"reference":[{"key":"e_1_2_10_2_1","first-page":"1521","volume-title":"The Metabolic and Molecular Bases of Inherited Disease","author":"Chen Y","year":"2001"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02679990"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1186\/1750-1172-9-47"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.21151"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(91)90483-2"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/PL00014320"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0041-1345(02)03951-9"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/lt.25649"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1023\/A:1005544117285"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.13872","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/petr.13872","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.13872","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,2]],"date-time":"2023-09-02T09:43:37Z","timestamp":1693647817000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.13872"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,10,12]]},"references-count":9,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2021,3]]}},"alternative-id":["10.1111\/petr.13872"],"URL":"https:\/\/doi.org\/10.1111\/petr.13872","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"value":"1397-3142","type":"print"},{"value":"1399-3046","type":"electronic"}],"subject":[],"published":{"date-parts":[[2020,10,12]]},"assertion":[{"value":"2020-05-26","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-09-07","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-10-12","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}],"article-number":"e13872"}}