{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,21]],"date-time":"2026-10-21T23:49:08Z","timestamp":1792626548099,"version":"4.5.0"},"reference-count":18,"publisher":"Wiley","issue":"6","license":[{"start":{"date-parts":[[2010,12,1]],"date-time":"2010-12-01T00:00:00Z","timestamp":1291161600000},"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":[[2010,12]]},"abstract":"<jats:title>ABSTRACT<\/jats:title><jats:sec><jats:title>Objectives:<\/jats:title><jats:p>The aim of the study was to assess health\u2010related quality of life (HRQOL) in children with Alagille syndrome (AGS) in comparison with a normative population and other chronic diseases, and also to examine the effect of AGS\u2010specific morbidities on HRQOL.<\/jats:p><\/jats:sec><jats:sec><jats:title>Patients and Methods:<\/jats:title><jats:p>A cross\u2010sectional study was performed using the Child Health Questionnaire Parent Form 50 (CHQ\u2010PF50) to measure HRQOL in patients with AGS. AGS HRQOL was compared with that of a normative population and those previously studied by the CHQ, including juvenile rheumatoid arthritis, attention\u2010deficit\/hyperactivity disorder, and liver transplantation. AGS\u2010specific questions were used in multiple regression analysis to determine correlation of features and symptoms of AGS with HRQOL.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results:<\/jats:title><jats:p>Seventy\u2010one patients with AGS, ages 5 to 18 years, were studied. Those families completing surveys demonstrated that children with AGS had significantly lower HRQOL (<jats:italic>P<\/jats:italic> &lt; 0.05) compared with the normative sample. In comparison with children with juvenile rheumatoid arthritis, children with AGS had lower psychosocial function scores (<jats:italic>P<\/jats:italic> &lt; 0.0005). In comparison with children with attention\u2010deficit\/hyperactivity disorder, children with AGS had lower physical function scores (<jats:italic>P<\/jats:italic> &lt; 0.0005) but higher psychosocial function scores (<jats:italic>P<\/jats:italic> &lt; 0.0005). Children with AGS had lower physical function scores than a liver transplant population (<jats:italic>P<\/jats:italic> &lt; 0.05). Regression analysis indicated that cardiac catheterization or surgery, mental health diagnoses, and poor sleep were associated with lower CHQ scores in children with AGS.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions:<\/jats:title><jats:p>In the first descriptive report of HRQOL in a large cohort of patients with AGS, HRQOL was impaired, indicating a significant burden of chronic disease in both physical and psychosocial health. Additional prospective evaluation is needed in multicenter collaboration.<\/jats:p><\/jats:sec>","DOI":"10.1097\/mpg.0b013e3181ef3771","type":"journal-article","created":{"date-parts":[[2010,10,13]],"date-time":"2010-10-13T12:17:24Z","timestamp":1286972244000},"page":"759-765","source":"Crossref","is-referenced-by-count":34,"title":["Health Status of Patients With Alagille Syndrome"],"prefix":"10.1002","volume":"51","author":[{"given":"Scott A","family":"Elisofon","sequence":"first","affiliation":[{"name":"Division of Pediatric Gastroenterology and Nutrition Children's Hospital Boston Harvard Medical School Boston MA USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karan M","family":"Emerick","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition Connecticut Children's Medical Center University of Connecticut School of Medicine, Hartford, CT the Department of Preventive Medicine and Epidemiology USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James M","family":"Sinacore","sequence":"additional","affiliation":[{"name":"Loyola University Stritch School of Medicine USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Estella M","family":"Alonso","sequence":"additional","affiliation":[{"name":"Division of Pediatric Gastroenterology, Hepatology, and Nutrition Children's Memorial Hospital Northwestern University Feinberg School of Medicine Chicago IL USA"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2010,12]]},"reference":[{"key":"e_1_2_5_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(75)80706-2"},{"key":"e_1_2_5_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(87)80153-1"},{"key":"e_1_2_5_4_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.510290331"},{"key":"e_1_2_5_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.49.3.431"},{"key":"e_1_2_5_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-8703(94)90684-X"},{"key":"e_1_2_5_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.1536-4801.2001.tb07367.x"},{"key":"e_1_2_5_8_1","volume-title":"The CHQ User's Manual","author":"Landgraf JM","year":"1996"},{"key":"e_1_2_5_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.1536-4801.2003.tb08134.x"},{"key":"e_1_2_5_10_1","doi-asserted-by":"publisher","DOI":"10.1017\/CBO9780511547409.017"},{"key":"e_1_2_5_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/j.1536-4801.1987.tb09426.x"},{"key":"e_1_2_5_12_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0022-3476(95)70298-9"},{"key":"e_1_2_5_13_1","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2002.33332"},{"key":"e_1_2_5_14_1","doi-asserted-by":"publisher","DOI":"10.1016\/0016-5085(88)90301-0"},{"key":"e_1_2_5_15_1","doi-asserted-by":"publisher","DOI":"10.1053\/jhep.2000.16600"},{"key":"e_1_2_5_16_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005176-199910000-00011"},{"key":"e_1_2_5_17_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840020413"},{"key":"e_1_2_5_18_1","doi-asserted-by":"publisher","DOI":"10.1001\/archotol.1990.01870020093025"},{"key":"e_1_2_5_19_1","doi-asserted-by":"publisher","DOI":"10.1086\/341327"}],"container-title":["Journal of Pediatric Gastroenterology and Nutrition"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1097\/MPG.0b013e3181ef3771","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,1,18]],"date-time":"2024-01-18T13:14:55Z","timestamp":1705583695000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1097\/MPG.0b013e3181ef3771"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2010,12]]},"references-count":18,"journal-issue":{"issue":"6","published-print":{"date-parts":[[2010,12]]}},"alternative-id":["10.1097\/MPG.0b013e3181ef3771"],"URL":"https:\/\/doi.org\/10.1097\/mpg.0b013e3181ef3771","archive":["Portico"],"relation":{},"ISSN":["0277-2116","1536-4801"],"issn-type":[{"value":"0277-2116","type":"print"},{"value":"1536-4801","type":"electronic"}],"subject":[],"published":{"date-parts":[[2010,12]]}}}