Abstract
Very early onset inflammatory bowel disease (VEO-IBD) is a unique disease entity with a complex genetic susceptibility in affected patients. Next-generation gene sequencing techniques have revealed various monogenetic mutations contributing to the pathogenesis of VEO-IBD, including interleukin 10 (IL-10) and IL-10 receptor (IL-10R) mutations. In this article, we reviewed the features of and effective therapeutic options for VEO-IBD with IL-10 and/or IL-10R mutations. The IL-10 signal pathway inhibits the release of several key cytokines and thereby has a significant anti-inflammatory effect in the gastrointestinal tract. Mutations of the genes encoding IL-10 and/or IL-10R have been detected in VEO-IBD patients among myriad populations throughout the world. VEO-IBD patients with IL-10 or IL-10R mutations often present with repeated bouts of bloody diarrhea, marked weight loss, growth retardation, and recurrent perianal problems, including abscesses, fistulas, and significant fissures. Moreover, some patients may have folliculitis and present with pulmonary infections. While the therapeutic efficacy of immunosuppressants is typically poor in these patients, allogeneic hematopoietic stem cell transplantation (HSCT) has been report...Continue Reading
Citations
Jul 13, 2018·BMC Pediatrics·Xin-Qiong WangChun-Di Xu
Aug 14, 2018·Immunology·Cinthia Nóbrega de Sousa DiasTatjana Souza Lima Keesen
Sep 1, 2018·Current Opinion in Gastroenterology·J Magarian Blander
Sep 15, 2018·Inflammatory Bowel Diseases·Margaret DeldayGeorge Grant
Dec 28, 2018·Journal of Crohn's & Colitis·Marianne R SpalingerIsabelle Frey-Wagner
Aug 3, 2019·The Journal of Pathology·Raquel Toribio-FernándezJose M Gonzalez-Granado
May 11, 2020·European Journal of Immunology·Dominic A BoardmanMegan K Levings
Sep 7, 2018·Experimental and Therapeutic Medicine·Jihong ZhuTaidi Zhong
Aug 2, 2020·Inflammatory Bowel Diseases·Yonghong YangGuangxi Zhou
Aug 28, 2020·Pediatric Transplantation·Maged KhederAmir Ali Hamidieh
May 15, 2018·Frontiers in Genetics·Fahad A Al-AbbasiNoor A Shaik
Sep 4, 2019·Journal of Clinical Immunology·Peter GeorgievTalal A Chatila
Apr 4, 2020·Nature Reviews. Disease Primers·Giulia RodaSilvio Danese
Jul 28, 2020·Nature Reviews. Gastroenterology & Hepatology·Matthew R KudelkaAndrew S Neish
May 20, 2020·Frontiers in Immunology·Mengxiao XieJian Xu
Jan 6, 2019·Journal of Clinical Immunology·Jaclyn A BjelacJames Fernandez
Sep 13, 2018·Marine Drugs·Alessandra M M LucenaJaneusa T Souto
Sep 17, 2020·Science Signaling·Claire GorbyIgnacio Moraga
Sep 18, 2020·The Journal of Allergy and Clinical Immunology·Lingli ZhouJun Cui
Feb 18, 2021·Journal of Medicinal Chemistry·Chufeng ZhangLijuan Chen
Feb 20, 2021·Frontiers in Immunology·Abigail R BassonAlex Rodriguez-Palacios
Mar 12, 2021·Journal of Inflammation·Paulina KrawiecElżbieta Pac-Kożuchowska
Mar 30, 2021·Gastroenterology·Alireza LorzadehUnmesh Jadhav
May 4, 2021·Cell and Tissue Research·Shiwa SoukouChristian F Krebs
May 13, 2021·Food & Function·Bing PangJunling Shi
Jul 24, 2021·Molecular Biology Reports·K A DvornikovaA Lerner
Nov 5, 2019·Seminars in Immunology·Huiyuan Zhang, Vijay Kuchroo
Oct 13, 2021·MBio·Qiufen MoFengqin Feng
Nov 20, 2020·Intestinal research·Rupa BanerjeeD Nageshwar Reddy