Severe immune-mediated enteropathy

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Severe immune-mediated enteropathy

ORPHA:94075Category

Also called Autoimmune enteropathy · Immune-mediated protracted diarrhea of infancy

What it is

Severe-immune mediated enteropathy describes a variety of intestinal disorders that can range from a serious, early-onset systemic disease (IPEX) to a mild isolated gastrointestinal disease. In children it manifests with severe diarrhea and dehydration in the presence of characteristic antibodies (anti-enterocyte and anti-goblet cell) and in adults with chronic diarrhea, malabsorption and weight loss.

Key facts

Classified as
Category

Cross-references

GARD 8689MEDDRA 10081456UMLS C0341305

Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.

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