{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,4]],"date-time":"2026-10-04T20:59:33Z","timestamp":1791147573997,"version":"4.1.0"},"reference-count":26,"publisher":"SAGE Publications","issue":"6","license":[{"start":{"date-parts":[[2007,7,1]],"date-time":"2007-07-01T00:00:00Z","timestamp":1183248000000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cell Transplant"],"published-print":{"date-parts":[[2007,7]]},"abstract":"<jats:p>Glycogen storage disease type I (GSD-I) is a group of autosomal recessive disorders with an incidence of 1 in 100,000. The two major subtypes are GSD-Ia, caused by a deficiency of glucose-6-phosphatase (G6Pase), and GSD-Ib, caused by a deficiency of glucose-6-phosphate transporter (G6PT). We report that a substantial improvement was achieved following several infusions of hepatocytes in a patient with GSD-Ib. Hepatocytes were isolated from the unused cadaveric whole livers of two donors. At the first transplantation, approximately 2 \u00d7 109 cells (2% of the estimated recipient's total hepatocytes) were infused. Seven days later 1 \u00d7 109 (1% of liver mass) cryopreserved hepatocytes from the same donor were infused, and an additional 3 \u00d7 109 (3% of liver mass) cells from the second donor were infused 1 month after the second transplantation. After the hepatocyte transplantation, the patient showed no hypoglycemic symptoms despite the discontinuation of cornstarch meals. Liver biopsies on posttransplantation days 20 and 250 showed a normal level of glucose-6-phosphatase activity in presolubilization assay that was very low before transplantation. This was the first and successful clinical hepatocyte transplantation in Korea. In this study, hepatocyte transplantation allowed a normal diet in a patient with GSD-Ib, with substantial improvement in their quality of life. Hepatocyte transplantation might be an alternative to liver transplantation and dietary therapy in GSD-Ib.<\/jats:p>","DOI":"10.3727\/000000007783465019","type":"journal-article","created":{"date-parts":[[2014,9,17]],"date-time":"2014-09-17T21:52:25Z","timestamp":1410990745000},"page":"629-637","source":"Crossref","is-referenced-by-count":89,"title":["Hepatocyte Transplantation for Glycogen Storage Disease Type Ib"],"prefix":"10.1177","volume":"16","author":[{"given":"Kwang-Woong","family":"Lee","sequence":"first","affiliation":[{"name":"Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ji-Hyun","family":"Lee","sequence":"additional","affiliation":[{"name":"Samsung Biomedical Research Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung Wook","family":"Shin","sequence":"additional","affiliation":[{"name":"Department of Radiology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sung Joo","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jae Won","family":"Joh","sequence":"additional","affiliation":[{"name":"Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doo-Hoon","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Chemical and Biochemical Engineering, Dongguk University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jong-Won","family":"Kim","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hwa-Young","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Soo-Youn","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hwan Hyo","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jin Wan","family":"Park","sequence":"additional","affiliation":[{"name":"Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shi-Yeon","family":"Kim","sequence":"additional","affiliation":[{"name":"Samsung Biomedical Research Institute, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hee-Hoon","family":"Yoon","sequence":"additional","affiliation":[{"name":"Department of Chemical and Biochemical Engineering, Dongguk University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Doo-Hee","family":"Jung","sequence":"additional","affiliation":[{"name":"Department of Chemical and Biochemical Engineering, Dongguk University, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yon Ho","family":"Choe","sequence":"additional","affiliation":[{"name":"Department of Pediatrics, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suk-Koo","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Surgery, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"179","published-online":{"date-parts":[[2017,6,22]]},"reference":[{"key":"bibr1-000000007783465019","doi-asserted-by":"publisher","DOI":"10.1007\/s00431-004-1405-1"},{"key":"bibr2-000000007783465019","first-page":"10396","volume":"255","author":"Arion W. 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