Article
Allogeneic transplantation successfully corrects immune defects, but not susceptibility to colitis, in a patient with nuclear factor-kappaB essential modulator deficiency.
The Journal of allergy and clinical immunology - 1 Dec 2008
Pai Sung-Yun, Levy Ofer, Jabara Haifa H, Glickman Jonathan N, Stoler-Barak Liat, Sachs Jessica, Nurko Samuel, Orange Jordan S, Geha Raif S
Abstract excerpt
BACKGROUND: Boys with X-linked ectodermal dysplasia and immunodeficiency caused by mutations of nuclear factor-kappaB essential modulator have defects in innate and adaptive immunity, and some have colitis. OBJECTIVE: We sought to determine whether curing the immune defect in such patients by means of allogeneic hematopoietic stem cell transplantation abolishes the susceptibility to colitis. METHODS: A boy with...
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