Immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome is due to a FOXP3 gene mutation leading to regulatory T cell dysfunction and multi-organ autoimmunity. Patients present in infancy with protracted diarrhea, dermatitis, insulin-dependent diabetes mellitus, and thyroiditis. Some patients may have severe food allergies. We present a patient with IPEX syndrome whose milk protein allergy resolved after receiving a stem cell transplant for his primary immunodeficiency.
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