ORPHA:84065
Idiopathic malabsorption due to bile acid synthesis defects
Synonyms: Idiopathic bile acid malabsorption
- Type
- Disease
- Group
- Disorder
- Status
- active
A disorder that is due to increased acid bile synthesis is an intestinal disease of unknown etiology characterized by an overproduction of bile acids which leads to chronic watery diarrhea.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic malabsorption due to bile acid synthesis defects | Idiopathic bile acid malabsorption |
| fr | Malabsorption idiopathique par défaut de synthèse des acides biliaires | |
| de | Malabsorption durch Gallensäuresynthesedefekt, idiopathische Form | |
| es | Malabsoción idiopática por defectos de la síntesis de ácidos biliares | |
| it | Malassorbimento idiopatico da difetto della sintesi degli acidi biliari | Malassorbimento idiopatico degli acidi biliari |
| nl | Idiopathische malabsorptie door defecten van galzuursynthese | Idiopathische galzuurmalabsorptie |
| pt | Má absorção idiopática por defeitos da síntese de ácidos biliares | Má absorção idiopática de ácidos biliares |
| pl | Idiopatyczny zespół złego wchłaniania z powodu defektu syntezy kwasów żółciowych | Idiopatyczne złe wchłanianie kwasów żółciowych |
| cs | Idiopatická malabsorpce způsobená poruchou syntézy žlučových kyselin | Idiopatická malabsorpce žlučových kyselin |
| uk | Ідіопатична мальабсорбція через дефекти синтезу жовчних кислот | Ідіопатична мальабсорбція жовчних кислот |
| zh | 胆酸合成缺陷所致特发性吸收不良 | 特发性胆汁酸吸收障碍 |
| tr | Safra asidi sentez defektlerine bağlı idiyopatik malabsorpsiyon | İdiyopatik safra asidi malabsorpsiyonu |
Definition in:
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Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K90.8 | NTBT | attributed | validated |
| ICD-11 | DA96.02 | NTBT | index | validated |
| MONDO | 0019393 | E | validated | |
| UMLS | C4274509 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:79168Disorder of bile acid synthesis
rare inborn errors of metabolism - ORPHA:104005Intestinal disease due to fat malabsorption
rare gastroenterological diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
0 recordsNo prevalence record in this release.
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HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/84065
- This record as JSON: /data/d/84065.json