Erschienen in:
12.11.2019 | Brief Report
Infliximab therapy for inflammatory colitis in an infant with NEMO deficiency
verfasst von:
Hasibe Artac, Ayca Emsen, Hulya Ucaryilmaz, Halil Haldun Emiroglu, Vedat Uygun, Asbjørg Stray-Pedersen
Erschienen in:
Immunologic Research
|
Ausgabe 4-5/2019
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Excerpt
X-linked ectodermal dysplasia with immunodeficiency (X-EDA-ID) is a rare disease caused by hypomorphic mutation in the gene encoding nuclear factor-κB (NF-κB) essential modulator (NEMO) [
1‐
3]. Boys affected with X-EDA-ID have severely impaired host defense, are prone to life-threatening infections, and have susceptibility to atypical mycobacteria. They have also increase risk of inflammatory diseases, in particular inflammatory colitis. NEMO colitis usually occurs early in childhood, causes intractable diarrhea that is difficult to control, and is life-threatening. …