{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,2]],"date-time":"2026-10-02T21:20:55Z","timestamp":1790976055973,"version":"4.1.0"},"reference-count":17,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2009,8,1]],"date-time":"2009-08-01T00:00:00Z","timestamp":1249084800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J. pediatr. gastroenterol. nutr."],"published-print":{"date-parts":[[2009,8]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Background:<\/jats:title><jats:p>Partial external biliary diversion (PEBD) is a promising treatment for children with progressive familial intrahepatic cholestasis (PFIC) and Alagille disease. Little is known about long\u2010term outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods:<\/jats:title><jats:p>A retrospective chart review of all patients undergoing PEBD in the University Medical Centre of Groningen (UMCG).<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>Between 2000 and 2005, PEBD was performed on 14 children with severe pruritus (PFIC 11, mean age 5.3 \u00b1 4.4 years; Alagille 3, mean age 7.4 \u00b1 4.2 years). Stature was &lt;\u22122 standard deviation score (SDS) in 50%. Median preoperative serum bile salt concentration was 318 \u03bcmol\/L (range 23\u2013527 \u03bcmol\/L). Twenty\u2010nine percent had severe liver fibrosis and 71% had mild or moderate fibrosis. Median follow\u2010up was 3.1 years (range 2.0\u20135.7 years). One patient (7%) underwent a liver transplantation at 3.2 years post\u2010PEBD. Two years postoperatively, 50% were without pruritus and 21% had mild pruritus. In 29%, pruritus had not diminished; 3 of them had severe fibrosis preoperatively. In patients with mild or moderate fibrosis, PEBD decreased serum bile salts (105 \u03bcmol\/L [range 8\u2013269 \u03bcmol\/L] 2 years postoperatively). Bile salts did not decrease in the patients with severe fibrosis. Two years after PEBD, 27% had a stature below \u22122 SDS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>At median follow\u2010up of 3.1 years after PEBD, pruritus has been relieved in 75%. Bile salts level and growth are improved in most patients. Longer follow\u2010up is needed to determine whether PEBD can postpone or avoid the demand for liver transplantation.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e31819a4e3d","type":"journal-article","created":{"date-parts":[[2009,7,16]],"date-time":"2009-07-16T07:21:05Z","timestamp":1247728865000},"page":"216-221","source":"Crossref","is-referenced-by-count":58,"title":["Partial External Biliary Diversion in Children With Progressive Familial Intrahepatic Cholestasis and Alagille Disease"],"prefix":"10.1002","volume":"49","author":[{"given":"Huiqi","family":"Yang","sequence":"first","affiliation":[{"name":"Section of Paediatric Surgery The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J","family":"Porte","sequence":"additional","affiliation":[{"name":"Section of Hepatobiliary Surgery Department of Surgery The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Henkjan J","family":"Verkade","sequence":"additional","affiliation":[{"name":"Section of Paediatric Gastroenterology Department of Paediatrics University Medical Centre Groningen and Beatrix Children's Hospital University of Groningen Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zacharias J","family":"De Langen","sequence":"additional","affiliation":[{"name":"Section of Paediatric Surgery The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jan BF","family":"Hulscher","sequence":"additional","affiliation":[{"name":"Section of Paediatric Surgery The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,8]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(88)90301-0"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3468(98)90435-3"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2002.33332"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2005.10.014"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-200002000-00011"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00383-006-1683-y"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1007\/s00595-007-3736-9"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3468(95)90440-9"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1055\/s-2003-43570"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpedsurg.2007.03.029"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1572-0241.2000.03370.x"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0016-5085(03)01199-5"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/0022-3468(95)90440-9"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1097\/01.tp.0000282869.94152.4f"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1093\/ajcp\/76.2.172"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1399-3046.2005.00380.x"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.2174\/1568008054064878"}],"container-title":["Journal of Pediatric Gastroenterology and Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1097\/MPG.0b013e31819a4e3d","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T12:08:27Z","timestamp":1705579707000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1097\/MPG.0b013e31819a4e3d"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,8]]},"references-count":17,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2009,8]]}},"alternative-id":["10.1097\/MPG.0b013e31819a4e3d"],"URL":"https:\/\/doi.org\/10.1097\/mpg.0b013e31819a4e3d","archive":["Portico"],"relation":{},"ISSN":["0277-2116","1536-4801"],"issn-type":[{"value":"0277-2116","type":"print"},{"value":"1536-4801","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,8]]}}}