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Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome MeSH Supplementary Concept Data 2025


MeSH Supplementary
Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome
Unique ID
C580192
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/C580192
Entry Term(s)
Autoimmunity-Immunodeficiency Syndrome, X-Linked
Diabetes Mellitus, Congenital Insulin-Dependent, with Fatal Secretory Diarrhea
Diarrhea, Polyendocrinopathy, Fatal Infection Syndrome, X-Linked
Enteropathy, Autoimmune, with Hemolytic Anemia and Polyendocrinopathy
IDDM-Secretory Diarrhea Syndrome
Immunodysregulation, Polyendocrinopathy, and Enteropathy, X-Linked
Ipex Syndrome
Polyendocrinopathy, Immune Dysfunction, and Diarrhea, X-Linked
X-Linked Autoimmunity-Allergic Dysregulation Syndrome
Registry Numbers
0
Heading Mapped to
Diabetes Mellitus, Type 1 / congenital
*Diarrhea
Immune System Diseases / congenital
*Genetic Diseases, X-Linked
Frequency
97
Note
An X-linked recessive immunologic disorder characterized by onset in infancy of severe diarrhea due to enteropathy, type 1 diabetes mellitus, and DERMATITIS. Other features may include HYPOTHYROIDISM; AUTOIMMUNE HEMOLYTIC ANEMIA; THROMBOCYTOPENIA; LYMPHADENOPATHY; HEPATITIS, and NEPHRITIS. The disorder may be fatal before age 2 years if not aggressively treated. Mutations in the FOXP3 gene have been identified. OMIM: 304790
Date of Entry
2013/10/24
Revision Date
2016/07/07
Immune Dysregulation, Polyendocrinopathy, Enteropathy, X-Linked Syndrome Preferred
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