{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,7,30]],"date-time":"2025-07-30T12:41:56Z","timestamp":1753879316211,"version":"3.41.2"},"reference-count":9,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,1,17]],"date-time":"2017-01-17T00:00:00Z","timestamp":1484611200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Transplantation"],"published-print":{"date-parts":[[2017,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">AIE<\/jats:styled-content> is a rare disorder in children that presents with severe diarrhea and malabsorption, caused by immune\u2010mediated damage to intestinal mucosa. <jats:styled-content style=\"fixed-case\">AIE<\/jats:styled-content> is often associated with various syndromes of immunodeficiency including <jats:styled-content style=\"fixed-case\">IPEX<\/jats:styled-content> syndrome (immune dysregulation, polyendocrinopathy and enteropathy, X\u2010linked). Dysfunctional T regulatory cells are the source of pathology in both <jats:styled-content style=\"fixed-case\">IPEX<\/jats:styled-content> syndrome and <jats:styled-content style=\"fixed-case\">AIE<\/jats:styled-content> as they are essential in maintaining tolerance to self\u2010antigens and eliminating autoreactive B cells. This case report describes a 10\u2010year\u2010old cardiac transplant and total thymectomy patient on chronic immunosuppression with tacrolimus that presented with <jats:styled-content style=\"fixed-case\">AIE<\/jats:styled-content> and extraintestinal manifestations of cyclical hepatitis. Transition from tacrolimus to sirolimus successfully increased T regulatory cells and resolved enteritis and hepatitis symptoms. Data support that thymectomy at &lt;1\u00a0year of age increases risk of autoimmune disease due to abnormal immune maturation. Studies suggest that the sirolimus promotes the upregulation of the FoxP3 protein that is classically associated with Tregs. In turn, Tregs prevent the maturation of autoreactive B cells that lead to autoimmune reactions.<\/jats:p>","DOI":"10.1111\/petr.12877","type":"journal-article","created":{"date-parts":[[2017,1,18]],"date-time":"2017-01-18T04:16:44Z","timestamp":1484713004000},"source":"Crossref","is-referenced-by-count":4,"title":["Autoimmune enteropathy and hepatitis in pediatric heart transplant recipient"],"prefix":"10.1111","volume":"21","author":[{"given":"Kimberly","family":"Lewis","sequence":"first","affiliation":[{"name":"Medical University of South Carolina \u2013 MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ryan","family":"Butts","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cardiology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Antonio Quiros","sequence":"additional","affiliation":[{"name":"Department of Pediatric Gastroenterology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michelle","family":"Hudspeth","sequence":"additional","affiliation":[{"name":"Department of Hematology\/Oncology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Katherine","family":"Twombley","sequence":"additional","affiliation":[{"name":"Department of Pediatric Nephrology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Andrew","family":"Savage","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cardiology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Sally","family":"Self","sequence":"additional","affiliation":[{"name":"Department of Pathology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ali","family":"Burnette","sequence":"additional","affiliation":[{"name":"Department of Pediatrics Cardiology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Shaoli","family":"Sun","sequence":"additional","affiliation":[{"name":"Department of Pathology MUSC  Charleston SC USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2017,1,17]]},"reference":[{"key":"e_1_2_7_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaci.2005.08.047"},{"key":"e_1_2_7_3_1","doi-asserted-by":"publisher","DOI":"10.1080\/00365520902783691"},{"key":"e_1_2_7_4_1","doi-asserted-by":"publisher","DOI":"10.1007\/s11894-012-0276-2"},{"key":"e_1_2_7_5_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2015.04.006"},{"key":"e_1_2_7_6_1","doi-asserted-by":"publisher","DOI":"10.1111\/petr.12205"},{"key":"e_1_2_7_7_1","first-page":"1105","article-title":"Clinical and molecular profile of a new series of patients with immune dysregulation, polyendocrinopathy, enteropathy, X\u2010linked syndrome: inconsistent correlation between forkhead box protein 3 expression and disease severity","volume":"122","author":"Gambineri E","year":"2008","journal-title":"Am Acad Allergy Asthma Immunol"},{"key":"e_1_2_7_8_1","doi-asserted-by":"publisher","DOI":"10.4049\/jimmunol.1402710"},{"key":"e_1_2_7_9_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.autrev.2014.10.012"},{"key":"e_1_2_7_10_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2005.04.017"}],"container-title":["Pediatric Transplantation"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1111%2Fpetr.12877","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/petr.12877","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,18]],"date-time":"2023-09-18T12:31:36Z","timestamp":1695040296000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/petr.12877"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,1,17]]},"references-count":9,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2017,3]]}},"alternative-id":["10.1111\/petr.12877"],"URL":"https:\/\/doi.org\/10.1111\/petr.12877","archive":["Portico"],"relation":{},"ISSN":["1397-3142","1399-3046"],"issn-type":[{"type":"print","value":"1397-3142"},{"type":"electronic","value":"1399-3046"}],"subject":[],"published":{"date-parts":[[2017,1,17]]},"article-number":"e12877"}}