Intractable diarrhea of infancy
Synonyms: IDI
- Type
- Category
- Group
- Group of disorders
- Age of onset
- Childhood
- Status
- active
Intractable diarrhea of infancy (IDI) is a heterogeneous syndrome that includes several diseases with different etiologies. Provisional classification of IDI, according to villous atrophy and based on immunohistological criteria, distinguishes two clearly different groups of IDI: 1) Immune-mediated: characterised by a mononuclear cell infiltration of the lamina propria and considered as being related to T cell activation. 2) The second histological pattern includes early onset severe intractable diarrhea histologically characterised by villous atrophy with low or without mononuclear cell infiltration of the lamina propria but specific histological abnormalities involving the epithelium.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Intractable diarrhea of infancy | IDI |
| fr | Diarrhée intraitable de l'enfant | |
| de | Intraktable Diarrhoe im Kindesalter | = en |
| es | Diarrea intratable infantil | |
| it | Diarrea intrattabile dell'infanzia | = en |
| nl | Refractaire diarree bij zuigeling | IDI · Therapieresistente diarree bij zuigeling |
| pt | Diarreia intratável da infância | DII |
| pl | Trudna do opanowania biegunka dziecięca | = en |
| cs | Persistentní průjem kojenců | = en |
| uk | Невиліковна діарея у немовлят | = en |
| zh | 婴儿顽固性腹泻 | = en |
| tr | Süt çocuğunun inatçı ishali | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 19126 | E | validated | |
| UMLS | C5848079 | 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 (2):
- ORPHA:506216Rare disorder potentially indicated for bowel transplant
rare transplant-related disorders - ORPHA:117569Rare intestinal disease
rare gastroenterological diseases
Children (12):
- ORPHA:1670Chronic diarrhea with villous atrophy
- ORPHA:329242Congenital chronic diarrhea with protein-losing enteropathy
- ORPHA:714487Congenital diarrhea-chronic gastrointestinal inflammation-ocular dysgenesis syndrome
- ORPHA:103908Congenital sodium diarrhea
- ORPHA:92050Congenital tufting enteropathy
- ORPHA:137622Intractable diarrhea-choanal atresia-eye anomalies syndrome
- ORPHA:2290Microvillus inclusion disease
- ORPHA:714490PERCC1-related congenital intractable malabsorptive diarrhea
- ORPHA:522037Primary autoimmune enteropathy
- ORPHA:522043Syndromic autoimmune enteropathy
- ORPHA:563708Syndromic congenital sodium diarrhea
- ORPHA:84064Trichohepatoenteric syndrome
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/73014
- This record as JSON: /data/d/73014.json