Short bowel syndrome
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
Short bowel syndrome is an intestinal failure due to either a congenital defect, intestinal infarction or extensive surgical resection of the intestinal tract that results in a functional small intestine of less than 200cm in length and is characterized by diarrhea, nutrient malabsoption, bowel dilation and dysmobility.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Short bowel syndrome | |
| fr | Syndrome du grêle court | Syndrome de l'intestin court |
| de | Kurzdarm-Syndrom | |
| es | Síndrome del intestino corto | |
| it | Sindrome dell'intestino corto | |
| nl | Kortedarmsyndroom | |
| pt | Síndrome do intestino curto | |
| pl | Zespół krótkiego jelita | |
| cs | Syndrom krátkého střeva | |
| uk | Синдром короткої кишки | |
| zh | 短肠综合征 | |
| tr | Kısa bağırsak sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | DA96.04 | E | specific | validated |
| MONDO | 0015183 | E | validated | |
| MONDO | 15183 | E | validated | |
| MeSH | D012778 | E | validated | |
| UMLS | C0036992 | E | validated | |
| GARD | 1502 | E | validated | |
| MedDRA | 10049416 | 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
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 2 / 100 000 | Europe | Value and class | Validated | European Medicines Agency 2019[OTHER] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/104008
- This record as JSON: /data/d/104008.json