{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T02:03:37Z","timestamp":1789610617634,"version":"build-2803163510"},"reference-count":39,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2026,4,16]],"date-time":"2026-04-16T00:00:00Z","timestamp":1776297600000},"content-version":"vor","delay-in-days":15,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"},{"start":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T00:00:00Z","timestamp":1775001600000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Background<\/jats:title>\n                    <jats:p>\n                      A20 haploinsufficiency (HA20) is an autoinflammatory disease driven by pathogenic variants in\n                      <jats:italic>TNFAIP3<\/jats:italic>\n                      , which plays a crucial role in regulating immune responses. The clinical manifestations of HA20 resemble those of inflammatory bowel disease (IBD), with prominent gastrointestinal (GI) involvement. Given the well\u2010established association between gut microbiota alterations and IBD, this study aimed to describe the GI involvement of HA20 patients and to investigate their fecal microbiota using shotgun sequencing and metabolomics.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>This study included 16 HA20 patients and 22 healthy age and sex\u2010matched controls. GI clinical phenotype, liver imaging, and liver and GI tissue histology were assessed. Shotgun metagenomic sequencing was performed on fecal DNA. Fecal metabolomic profiling of bile acids, short\u2010chain fatty acids (SCFAs), and tryptophan metabolites was performed.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Liver imaging revealed chronic liver disease in 3\/5 patients, showing as liver dysmorphia and portal hypertension. Histological analysis showed lymphoplasmocytic infiltrate of the GI tract and the liver. The fecal microbiota of HA20 patients was characterized by marked alterations, including a reduction in microbial diversity and an increase in the pro\u2010inflammatory bacterium\n                      <jats:italic>Ruminococcus gnavus<\/jats:italic>\n                      . Microbial bile acid deconjugation and desulfation were impaired. Additionally, tryptophan metabolism was altered, with a shift towards the kynurenine pathway.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Our results show that HA20 is associated with gut microbiota alterations and significant disruptions in metabolic pathways, particularly involving bile acids. These alterations could contribute to the chronic inflammation observed in HA20. These findings highlight the role of the gut\u2010liver axis and of mucosal barrier dysfunction in HA20.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1111\/pai.70343","type":"journal-article","created":{"date-parts":[[2026,4,17]],"date-time":"2026-04-17T02:59:16Z","timestamp":1776394756000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Functional changes in the gut microbiota are associated with the intestinal phenotype in A20 haploinsufficiency"],"prefix":"10.1111","volume":"37","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9767-2262","authenticated-orcid":false,"given":"In\u00e8s","family":"Elhani","sequence":"first","affiliation":[{"name":"Centre de Recherche Saint\u2010Antoine, CRSA, Sorbonne Universit\u00e9, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Gastroenterology Department, Assistance publique\u2010H\u00f4pitaux de Paris, Saint\u2010Antoine Hospital, Paris, France Sorbonne Universit\u00e9  Paris France"},{"name":"Paris Center for Microbiome MedICIsne (PaCeMM) FHU  Paris France"},{"name":"Sorbonne University, Internal medicine department, Tenon hospital, CEREMAIA, AP\u2010HP, DMU3ID, FHU INFLAMME, INSERM UMRS1155, Paris, France &amp; ERN RITA Sorbonne University  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marius","family":"Bredon","sequence":"additional","affiliation":[{"name":"Centre de Recherche Saint\u2010Antoine, CRSA, Sorbonne Universit\u00e9, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Gastroenterology Department, Assistance publique\u2010H\u00f4pitaux de Paris, Saint\u2010Antoine Hospital, Paris, France Sorbonne Universit\u00e9  Paris France"},{"name":"Paris Center for Microbiome MedICIsne (PaCeMM) FHU  Paris France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana","family":"Enea","sequence":"additional","affiliation":[{"name":"Department of Pathology, 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of Tours  Tours France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Achille","family":"Aouba","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Caen University Hospital  Caen France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adrien","family":"Bigot","sequence":"additional","affiliation":[{"name":"Service de M\u00e9decine Interne CHRU de Tours  Tours France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Claire","family":"de Moreuil","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Brest University Hospital  Brest France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Iria","family":"Alonso","sequence":"additional","affiliation":[{"name":"Centre de Recherche Saint\u2010Antoine, CRSA, Sorbonne Universit\u00e9, Institut National de la Sant\u00e9 et de la Recherche M\u00e9dicale, Gastroenterology Department, Assistance publique\u2010H\u00f4pitaux de 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