{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T03:44:23Z","timestamp":1778643863997,"version":"3.51.4"},"reference-count":42,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2019,11,10]],"date-time":"2019-11-10T00:00:00Z","timestamp":1573344000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Scand J Immunol"],"published-print":{"date-parts":[[2020,1]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Wiskott\u2010Aldrich syndrome (WAS) is an X\u2010linked primary immunodeficiency disease caused by a mutation in the <jats:italic>WAS<\/jats:italic> gene that encodes the WAS protein (WASp); up to 5\u201010% of these patients develop inflammatory bowel disease (IBD). The mechanisms by which WASp deficiency causes IBD are unclear. Intestinal microbial dysbiosis and imbalances in host immune responses play important roles in the pathogenesis of polygenetic IBD; however, few studies have conducted detailed examination of the microbial alterations and their relationship with IBD in WAS. Here, we collected faecal samples from 19 children (all less than 2\u00a0years old) with WAS and samples from WASp\u2010KO mice with IBD and subjected them to 16S ribosomal RNA sequencing. We found that microbial community richness and structure in WAS children were different from those in controls; WAS children revealed reduced microbial community richness and diversity. Relative abundance of <jats:italic>Bacteroidetes<\/jats:italic> and <jats:italic>Verrucomicrobia<\/jats:italic>in in WAS children was significantly lower, while that of <jats:italic>Proteobacteria<\/jats:italic> was markedly higher. WASp\u2010KO mice revealed a significantly decreased abundance of <jats:italic>Firmicutes<\/jats:italic>. Faecal microbial dysbiosis caused by WASp deficiency is similar to that observed for polygenetic IBD, suggesting that WASp may play crucial function in microbial homoeostasis and that microbial dysbiosis may contribute to IBD in WAS. These microbial alterations may be useful targets for monitoring and therapeutically managing intestinal inflammation in WAS.<\/jats:p>","DOI":"10.1111\/sji.12805","type":"journal-article","created":{"date-parts":[[2019,7,3]],"date-time":"2019-07-03T07:29:21Z","timestamp":1562138961000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":11,"title":["Faecal microbial dysbiosis in children with Wiskott\u2010Aldrich syndrome"],"prefix":"10.1111","volume":"91","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-5954-5828","authenticated-orcid":false,"given":"Liang","family":"Zhang","sequence":"first","affiliation":[{"name":"Ministry of Education Key Laboratory of Child Development and Disorders Children's Hospital of Chongqing Medical University Chongqing China"},{"name":"Chongqing Key Laboratory of Child Infection and Immunity Children's Hospital of Chongqing Medical University Chongqing 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