Immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome
Immune dysregulation-polyendocrinopathy-enteropathy-X-linked syndrome
Definition
Immunodysregulation - polyendocrinopathy - enteropathy - X-linked (IPEX) syndrome is a severe congenital systemic autoimmune disease characterized by refractory diarrhea, endocrinopathies, cutaneous involvement, and infections.
Also known as autoimmune enteropathy type 1, autoimmunity-immunodeficiency syndrome, X-linked, diabetes mellitus, congenital insulin-dependent, with fatal secretory diarrhea, diabetes mellitus, congenital insulin-dependent, with fatal secretory diarrhoea, diarrhea, polyendocrinopathy, fatal infection syndrome, X-linked, DMSD, IDDM-secretory diarrhea syndrome, IDDM-secretory diarrhoea syndrome, immune dysfunction and diarrhea syndrome, immune dysfunction and diarrhoea syndrome, immune dysregulation, polyendocrinopathy, and enteropathy X-linked syndrome, immunodysregulation, polyendocrinopathy, and enteropathy, X-linked, immunodysregulation, polyendocrinopathy, and enteropathy, X-linked, X-linked recessive, IPEX, X linked polyendocrinopathy, X-linked autoimmunity-allergic dysregulation syndrome, XLAAD, XPID — per MONDO