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Digestive Diseases and Sciences
Erschienen in: Digestive Diseases and Sciences 11/2020

24.08.2020 | MENTORED REVIEW

A Review of Autoimmune Enteropathy and Its Associated Syndromes

verfasst von: Charles B. Chen, Farah Tahboub, Thomas Plesec, Marsha Kay, Kadakkal Radhakrishnan

Erschienen in: Digestive Diseases and Sciences | Ausgabe 11/2020

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Abstract

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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Metadaten
Titel
A Review of Autoimmune Enteropathy and Its Associated Syndromes
verfasst von
Charles B. Chen
Farah Tahboub
Thomas Plesec
Marsha Kay
Kadakkal Radhakrishnan
Publikationsdatum
24.08.2020
Verlag
Springer US
Erschienen in
Digestive Diseases and Sciences / Ausgabe 11/2020
Print ISSN: 0163-2116
Elektronische ISSN: 1573-2568
DOI
https://doi.org/10.1007/s10620-020-06540-8

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