{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,5]],"date-time":"2026-02-05T08:38:16Z","timestamp":1770280696965,"version":"3.49.0"},"reference-count":8,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2016,4,28]],"date-time":"2016-04-28T00:00:00Z","timestamp":1461801600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["RO1 DK 80670"],"award-info":[{"award-number":["RO1 DK 80670"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2016,9]]},"abstract":"Abstract<\/jats:title>Background & Aims<\/jats:title>Primary biliary cirrhosis (PBC<\/jats:styled-content>) is characterized by chronic cholestasis and disease\u2010specific antimitochondrial antibodies (AMA<\/jats:styled-content>). A high prevalence of AMA<\/jats:styled-content>s in first\u2010degree relatives (FDR<\/jats:styled-content>s) of PBC<\/jats:styled-content> probands has been reported, although the natural history of such patients has not been described. We aimed to assess the risk of developing PBC<\/jats:styled-content> in AMA<\/jats:styled-content>+ FDR<\/jats:styled-content>s of patients with PBC<\/jats:styled-content>.<\/jats:p><\/jats:sec>Methods<\/jats:title>First\u2010degree relatives recruited to the Mayo Clinic PBC<\/jats:styled-content> Genetic Epidemiology Registry and Biorepository were followed for disease onset after recruitment. Development of PBC<\/jats:styled-content> was ascertained via self\u2010report during a telephone interview and\/or via proband report on a questionnaire. Chi\u2010squared test and t<\/jats:italic>\u2010test were used to assess the differences between categorical and continuous variables respectively. A mixed\u2010effects model was used to assess the change in biochemical profiles over time.<\/jats:p><\/jats:sec>Results<\/jats:title>Forty AMA<\/jats:styled-content>+ and 423 AMA<\/jats:styled-content>\u2212 subjects were included and followed for a median of 8.9 and 8.4 years respectively. Overall, 3% (n<\/jats:italic> = 15) of FDR<\/jats:styled-content>s were diagnosed with PBC<\/jats:styled-content>, and AMA<\/jats:styled-content>+ FDR<\/jats:styled-content>s had a higher risk than AMA<\/jats:styled-content>\u2212 FDR<\/jats:styled-content>s (24% vs. 0.7%, P<\/jats:italic> < 0.01). However, among undiagnosed FDR<\/jats:styled-content>s, only 4% of AMA<\/jats:styled-content>+ (n<\/jats:italic> = 1) and 0.4% of AMA<\/jats:styled-content>\u2212 (n<\/jats:italic> = 1) FDR<\/jats:styled-content>s were diagnosed with PBC<\/jats:styled-content> (P<\/jats:italic> = 0.17) during the follow\u2010up period. None of the AMA<\/jats:styled-content>+ FDR<\/jats:styled-content>s with normal alkaline phosphatase at baseline developed PBC<\/jats:styled-content> in follow\u2010up.<\/jats:p><\/jats:sec>Conclusions<\/jats:title>Our results suggest a low risk of developing PBC<\/jats:styled-content> over time in FDR<\/jats:styled-content>s of patients with PBC<\/jats:styled-content>, particularly those without biochemical evidence of cholestasis at baseline. These data are useful in counselling and reassuring relatives of their overall favourable prognosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.13143","type":"journal-article","created":{"date-parts":[[2016,4,10]],"date-time":"2016-04-10T18:24:30Z","timestamp":1460312670000},"page":"1378-1382","source":"Crossref","is-referenced-by-count":23,"title":["Low incidence of primary biliary cirrhosis (PBC<\/scp>) in the first\u2010degree relatives of PBC<\/scp> probands after 8 years of follow\u2010up"],"prefix":"10.1111","volume":"36","author":[{"given":"Aliya F.","family":"Gulamhusein","sequence":"first","affiliation":[{"name":"Division of Gastroenterology and Hepatology Mayo Clinic Rochester MN USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Brian D.","family":"Juran","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology Center for Basic Research in Digestive Diseases Mayo Clinic Rochester MN USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Elizabeth J.","family":"Atkinson","sequence":"additional","affiliation":[{"name":"Department of Health Sciences Research Mayo Clinic College of Medicine Rochester MN USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bryan","family":"McCauley","sequence":"additional","affiliation":[{"name":"Department of Health Sciences Research Mayo Clinic College of Medicine Rochester MN USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Erik","family":"Schlicht","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology Center for Basic Research in Digestive Diseases Mayo Clinic Rochester MN USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Konstantinos N.","family":"Lazaridis","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology Center for Basic Research in Digestive Diseases Mayo Clinic Rochester MN USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2016,4,28]]},"reference":[{"key":"e_1_2_8_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22906"},{"key":"e_1_2_8_3_1","doi-asserted-by":"publisher","DOI":"10.1007\/BF02919751"},{"key":"e_1_2_8_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2013.06.015"},{"key":"e_1_2_8_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.21749"},{"key":"e_1_2_8_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(96)04410-8"},{"key":"e_1_2_8_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.1840060609"},{"key":"e_1_2_8_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0168-8278(97)80444-8"},{"key":"e_1_2_8_9_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.20907"}],"container-title":["Liver International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fliv.13143","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fliv.13143","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.13143","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,23]],"date-time":"2023-09-23T21:33:19Z","timestamp":1695504799000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/liv.13143"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2016,4,28]]},"references-count":8,"journal-issue":{"issue":"9","published-print":{"date-parts":[[2016,9]]}},"alternative-id":["10.1111\/liv.13143"],"URL":"https:\/\/doi.org\/10.1111\/liv.13143","archive":["Portico"],"relation":{},"ISSN":["1478-3223","1478-3231"],"issn-type":[{"value":"1478-3223","type":"print"},{"value":"1478-3231","type":"electronic"}],"subject":[],"published":{"date-parts":[[2016,4,28]]}}}