A Review of Autoimmune Enteropathy and Its Associated Syndromes
DIGESTIVE DISEASES AND SCIENCES(2020)
摘要
Autoimmune enteropathy is an extremely rare condition characterized by an abnormal intestinal immune response which typically manifests within the first 6 months of life as severe, intractable diarrhea that does not respond to dietary modification. Affected individuals frequently present with other signs of autoimmunity. The diagnosis is made based on a characteristic combination of clinical symptoms, laboratory studies, and histological features on small bowel biopsy. Autoimmune enteropathy is associated with a number of other conditions and syndromes, most notably immunodysregulation polyendocrinopathy enteropathy X-linked (IPEX) syndrome and autoimmune polyglandular syndrome type 1 (APS-1). Diagnosis and treatment is challenging, and further research is needed to better understand the pathogenesis, disease progression, and long-term outcomes of these conditions.
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关键词
Autoimmune enteropathy,Genetic syndromes,Immunodeficiency,Immunodysregulation polyendocrinopathy enteropathy X-linked syndrome,Autoimmune polyglandular syndrome type 1
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