{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,13]],"date-time":"2026-10-13T11:58:56Z","timestamp":1791892736410,"version":"4.3.4"},"reference-count":32,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","license":[{"start":{"date-parts":[[2002,6,1]],"date-time":"2002-06-01T00:00:00Z","timestamp":1022889600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"},{"start":{"date-parts":[[2003,12,30]],"date-time":"2003-12-30T00:00:00Z","timestamp":1072742400000},"content-version":"vor","delay-in-days":577,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2002,6]]},"abstract":"<jats:sec>\n            <jats:title\/>\n            <jats:p>Alagille syndrome (AGS) causes intractable pruritus and disfiguring xanthomas because of retained bile acids and cholesterol. This study was performed to determine whether partial external biliary diversion (PEBD) is effective for relief of pruritus and xanthomas in AGS patients who fail conventional medical therapy. Between the years 1985 and 2001, 9 AGS patients underwent PEBD. Complete follow-up data were available for all patients. The average age at PEBD was 4.8 (range 1.4-10) years. The average duration of follow-up was 7.5 (range 0.5-16.0) years. All 9 patients had severe, mutilating pruritus (grade 4) prior to diversion. At 1 year post-PEBD, the average pruritus score was 1.1; 8 patients had only mild scratching when undistracted. Three patients with extensive xanthomas prior to PEBD had complete resolution within 1 year. Mean serum bile salt levels (n = 5) decreased from 136.5 to 37.1 \u03bcmol\/L and mean cholesterol (n = 7) from 724 to 367 mg\/dL 1 year after PEBD. A single 21-year-old patient with PEBD for 14 years experienced an increase in pruritus from grade 1 to grade 4 within 2 months of elective reversal of PEBD. In conclusion, PEBD is effective for treating severe pruritus and hypercholesterolemia in AGS patients without cirrhosis who did not respond to medical therapy. PEBD should be considered as a therapeutic option for these patients before referral for liver transplantation because of morbid complications<\/jats:p>\n          <\/jats:sec>","DOI":"10.1053\/jhep.2002.33332","type":"journal-article","created":{"date-parts":[[2004,3,19]],"date-time":"2004-03-19T18:41:36Z","timestamp":1079721696000},"page":"1501-1506","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":109,"title":["Partial external biliary diversion for intractable pruritus and xanthomas in Alagille syndrome"],"prefix":"10.1097","volume":"35","author":[{"given":"Karan M.","family":"Emerick","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter F.","family":"Whitington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"10.1053\/jhep.2002.33332-BIB1|cit1","doi-asserted-by":"crossref","first-page":"431","DOI":"10.1136\/gut.49.3.431","article-title":"Outcome of liver disease in children with Alagille syndrome: a study of 163 patients","volume":"49","author":"Lykavieris","year":"2001","journal-title":"Gut"},{"key":"10.1053\/jhep.2002.33332-BIB2|cit2","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1016\/0016-5085(90)90027-X","article-title":"Rifampin relieves pruritus in children with cholestatic liver disease","volume":"98","author":"Cynamon","year":"1990","journal-title":"Gastroenterology"},{"key":"10.1053\/jhep.2002.33332-BIB3|cit3","first-page":"1404","article-title":"Management of the pruritus of cholestasis: potential role of opiate antagonists","volume":"86","author":"Bergasa","year":"1991","journal-title":"Am J Gastroenterol"},{"key":"10.1053\/jhep.2002.33332-BIB4|cit4","doi-asserted-by":"crossref","first-page":"114","DOI":"10.1001\/jama.1961.03040180034008","article-title":"Relief of the pruritus of jaundice with a bile-acid sequestering resin","volume":"176","author":"Carey","year":"1961","journal-title":"J Am Med Assoc"},{"key":"10.1053\/jhep.2002.33332-BIB5|cit5","doi-asserted-by":"crossref","first-page":"1582","DOI":"10.1016\/0016-5085(95)90709-2","article-title":"The pruritus of cholestasis: potential pathogenic and therapeutic implications of opioids","volume":"108","author":"Bergasa","year":"1995","journal-title":"Gastroenterology"},{"key":"10.1053\/jhep.2002.33332-BIB6|cit6","doi-asserted-by":"crossref","first-page":"573","DOI":"10.1097\/00005176-199705000-00016","article-title":"Bile acid therapy in pediatric hepatobiliary disease: the role of ursodeoxycholic acid","volume":"24","author":"Balistreri","year":"1997","journal-title":"J Pediatr Gastroenterol Nutr"},{"key":"10.1053\/jhep.2002.33332-BIB7|cit7","first-page":"323","volume-title":"Pediatric Cholestasis. 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