Severe immune-mediated enteropathy
Synonyms: Autoimmune enteropathy · Immune-mediated protracted diarrhea of infancy
- Type
- Category
- Group
- Group of disorders
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Severe immune-mediated enteropathy | Autoimmune enteropathy · Immune-mediated protracted diarrhea of infancy |
| fr | Entéropathie sévère d'origine immunitaire | Diarrhée sévère auto-immune de l'enfant · Entéropathie auto-immune |
| de | Enteropathie, immunvermittelte schwere | Autoimmun-Enteropathie · Diarrhoe, autoimmune schwere, des Kindesalters |
| es | Enteropatía grave mediada por el sistema inmunitario | Diarrea inmunomediada prolongada de la infancia · Enteropatía autoinmune |
| it | Diarrea dell'infanzia intrattabile immuno-mediata | Enteropatia autoimmune |
| nl | Ernstige immuun-gemedieerde enteropathie | Auto-immune enteropathie · Immuun-gemedieerde aanhoudende diarree in de kinderjaren |
| pt | Enteropatia grave imunomediada | |
| pl | Ciężka enteropatia immunologiczna | Enteropatia autoimmunologiczna · Przewlekła biegunka dziecięca o podłożu immunologicznym |
| cs | Autoimunitní enteropatie | Těžká enteropatie na podkladě imunitní reakce · Protrahovaný průjem kojenců na podkladě imunitní reakce |
| uk | Тяжка імуноопосередкована ентеропатія | Аутоімунна ентеропатія · Імуноопосередкована затяжна діарея немовлят |
| zh | 重型免疫相关肠病 | 免疫介导的婴儿拖延性腹泻 |
| tr | Ağır immün aracılı enteropati | Otoimmün enteropati · Bağışıklık aracılı uzun süreli bebeklik diyare |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| UMLS | C0341305 | E | validated | |
| GARD | 8689 | E | validated | |
| MedDRA | 10081456 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:117569Rare intestinal disease
rare gastroenterological diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/94075
- This record as JSON: /data/d/94075.json