{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,29]],"date-time":"2026-07-29T11:16:39Z","timestamp":1785323799662,"version":"3.55.0"},"reference-count":31,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2017,12,21]],"date-time":"2017-12-21T00:00:00Z","timestamp":1513814400000},"content-version":"vor","delay-in-days":20,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/501100002426","name":"Fondazione Telethon","doi-asserted-by":"publisher","award":["201545X003661"],"award-info":[{"award-number":["201545X003661"]}],"id":[{"id":"10.13039\/501100002426","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Allergy Immunology"],"published-print":{"date-parts":[[2017,12]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Chronic granulomatous disease (<jats:styled-content style=\"fixed-case\">CGD<\/jats:styled-content>) is a primary immunodeficiency of phagocytes, characterized by life\u2010threatening infections and hyperinflammation. Due to survival improvement, inflammatory bowel disease (<jats:styled-content style=\"fixed-case\">IBD<\/jats:styled-content>) is becoming increasingly relevant. Here, we report our 20\u00a0year experience.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We retrospectively analyzed clinic, endoscopic, and histologic features, as well as the management of <jats:styled-content style=\"fixed-case\">CGD<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">IBD<\/jats:styled-content> patients referred to the Bambino Ges\u00f9 Children's Hospital in Rome, Italy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Of 20 patients with <jats:styled-content style=\"fixed-case\">CGD<\/jats:styled-content>, 9 presented with <jats:styled-content style=\"fixed-case\">CGD<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">IBD<\/jats:styled-content> at diagnosis and\/or during follow\u2010up. Symptoms occurred at a median age of 16\u00a0years (range 3.2\u201042), with a median delay of 6\u00a0months for endoscopic confirmation. Patients mainly complained of nonspecific diarrhea (55%), with discrepancy between symptom paucity and severe endoscopic appearance, mainly represented by extensive colonic involvement (44%). Histology revealed at least 2 characteristic features (epithelioid granulomas, pigmented macrophages, and increased eosinophils) in 78% of patients. Eight of 9 patients received oral mesalamine, and 5 required systemic steroids. One patient received azathioprine due to steroid dependence. No patient required biological therapy or surgery. Clinical remission was obtained in all patients, but the majority complained of mild relapses. Two episodes of severe infection occurred early after steroid therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Penetrance of <jats:styled-content style=\"fixed-case\">CGD<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">IBD<\/jats:styled-content> increases with age. Clinical manifestations may be subtle, and clinicians should have a low threshold to recommend endoscopy. Treatment with <jats:styled-content style=\"fixed-case\">NSAID<\/jats:styled-content>s and\/or steroids achieves a good response, but relapses usually occur. Infection surveillance is mandatory during treatment, to prevent opportunistic infections. A close collaboration between pediatric immunologists and gastroenterologists is pivotal, including combined follow\u2010up.<\/jats:p><\/jats:sec>","DOI":"10.1111\/pai.12814","type":"journal-article","created":{"date-parts":[[2017,10,5]],"date-time":"2017-10-05T19:45:21Z","timestamp":1507232721000},"page":"801-809","source":"Crossref","is-referenced-by-count":36,"title":["Inflammatory bowel disease in chronic granulomatous disease: An emerging problem over a twenty years' experience"],"prefix":"10.1111","volume":"28","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8719-9670","authenticated-orcid":false,"given":"Giulia","family":"Angelino","sequence":"first","affiliation":[{"name":"Digestive Endoscopy and Surgery Unit Bambino Ges\u00f9 Children's Hospital IRCCS  Rome Italy"},{"name":"University Department of Pediatrics Unit of Immune and Infectious Diseases Bambino Ges\u00f9 Children's Hospital IRCCS University of Rome Tor Vergata  Rome 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