{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T08:47:26Z","timestamp":1791103646671,"version":"4.1.0"},"reference-count":33,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[1999,8,1]],"date-time":"1999-08-01T00:00:00Z","timestamp":933465600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1999,8]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Liver transplantation may be indicated in patients with GSD type Ia when dietary treatment fails or when hepatic adenomas develop, because they carry a risk of liver cancer or severe intratumoral haemorrhage. Published reports on the results of liver transplantation in patients with GSD Ia include 10 patients and provide little information on long\u2010term outcome. In particular, it is not known whether liver transplantation prevents renal failure due to focal segmental glomerulosclerosis. We report here on 3 patients with GSD Ia in whom liver transplantation was performed at 15, 17 and 23 years of age because of multiple hepatic adenomas in all 3 patients with a fear of malignant transformation, and of poor metabolic balance and severe growth retardationin the youngest one. Renal function was normal in all patients. During the 6\u20138 years following transplantation, the quality of life has initially greatly improved, with none of the previous dietary restraints and a spectacular increase in height. However, long\u2010term complications included chronic hepatitis C in one patient, gouty attacks in another and focal segmental glomerulosclerosis with progressive renal insufficiency in the third. These results: (1) confirm that liver transplantation restores a normal metabolic balance in patients with GSD Ia, allows catch\u2010up growth and improves the quality of life; (2) suggest that liver transplantation may be considered in teenagers with unresectable multiple adenomas because of a lack of clear\u2010cut criteria to detect malignant transformation early; and (3) suggest that liver transplantation does not prevent focal segmental glomerulosclerosis associated with GSD Ia.<\/jats:p>","DOI":"10.1023\/a:1005544117285","type":"journal-article","created":{"date-parts":[[2002,12,21]],"date-time":"2002-12-21T17:31:09Z","timestamp":1040491869000},"page":"723-732","source":"Crossref","is-referenced-by-count":68,"title":["Long\u2010term outcome of liver transplantation in patients with glycogen storage disease type Ia"],"prefix":"10.1002","volume":"22","author":[{"given":"L.","family":"Faivre","sequence":"first","affiliation":[{"name":"H\u00e9patologie et Chirurgie P\u00e9diatriques H\u00f4pital de Bic\u00eatre  Le Kremlin Bic\u00eatre France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"D.","family":"Houssin","sequence":"additional","affiliation":[{"name":"Clinique Chirurgicale H\u00f4pital Cochin  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Valayer","sequence":"additional","affiliation":[{"name":"H\u00e9patologie et Chirurgie P\u00e9diatriques H\u00f4pital de Bic\u00eatre  Le Kremlin Bic\u00eatre France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Brouard","sequence":"additional","affiliation":[{"name":"Service de P\u00e9diatrie A Centre Hospitalier Universitaire  Caen France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"M.","family":"Hadchouel","sequence":"additional","affiliation":[{"name":"H\u00e9patologie et Chirurgie P\u00e9diatriques H\u00f4pital de Bic\u00eatre  Le Kremlin Bic\u00eatre France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"O.","family":"Bernard","sequence":"additional","affiliation":[{"name":"H\u00e9patologie et Chirurgie P\u00e9diatriques H\u00f4pital de Bic\u00eatre  Le Kremlin Bic\u00eatre France"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[1999,8]]},"reference":[{"key":"e_1_2_1_2_1","doi-asserted-by":"publisher","DOI":"10.1038\/ki.1989.133"},{"key":"e_1_2_1_3_1","first-page":"2722","article-title":"Cyclosporine\u2010induced hyperuricemia and gout in renal transplants","volume":"27","author":"Ben Hamida M","year":"1995","journal-title":"Transplant 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