Clinical Characteristics of Enterocolitis Associated with Inborn Errors of Immunitiy Ichiro Takeuchi 1 , Katsuhiro Arai 1 1Division of Gastroenterology, National Center for Child Health and Development, Tokyo Keyword: 原発性免疫不全症 , inborn errors of immunity , monogenic IBD , 超早期発症型炎症性腸疾患 , 造血幹細胞移植 pp.1692-1701
Published Date 2019/12/25
DOI https://doi.org/10.11477/mf.1403201900
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 Enterocolitis associated with inborn errors of immunity is a known cause of pediatric-onset IBD(inflammatory bowel disease). Recent advancements in genetics have revealed numerous hereditary diseases that may result in IBD-like phenotypes ; they are known as “monogenic IBD” or “Mendelian disease-associated IBD.” Immunosuppressive therapy for IBD may result in serious infection. However hematopoietic stem cell transplantation may be a treatment of choice for some monogenic IBDs. Therefore, prompt diagnosis of monogenic IBDs is crucial for appropriate treatment and management. In this chapter, we discuss enterocolitis associated with inborn errors of immunity and present some cases experienced at our hospital.

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