ACTH-dependent Cushing syndrome
Synonyms: ACTH-dependent CS · Adrenocorticotropic hormone-dependent Cushing syndrome · Corticotropin-dependent Cushing syndrome
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
A group of endogenous Cushing syndrome (CS) caused by abnormal production of ACTH due, in 80% of cases, to adrenocorticotropic hormone (ACTH) oversecretion by a pituitary adenoma (Cushing disease, CD) and in 20% of cases to ectopic ACTH secretion (CS due to EAS) by an extrapituitary tumor or very rarely due to a tumor secreting both ACTH and corticotrophin-releasing hormone (CRH).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | ACTH-dependent Cushing syndrome | ACTH-dependent CS · Adrenocorticotropic hormone-dependent Cushing syndrome · Corticotropin-dependent Cushing syndrome |
| fr | Syndrome de Cushing dépendant de l'ACTH | CS dépendant de l'ACTH · Syndrome de Cushing dépendant de l'adrénocorticotropine |
| de | Cushing-Syndrom, ACTH-abhängiges | ACTH-abhängiges CS · Adrenocorticotropes Hormon-bedingtes Cushing-Syndrom · Corticotropin-abhängiges Cushing-Syndrom |
| es | Síndrome de Cushing dependiente de ACTH | CS ACTH-dependiente · Síndrome de Cushing dependiente de corticotropina · Síndrome de Cushing dependiente de la hormona adrenocorticotropa |
| it | Sindrome di Cushing ACTH-dipendente | CS ACTH-dipendente · Sindrome di Cushing corticotropina-dipendente · Sindrome di Cushing dipendente dall'ormone adrenocorticotropo |
| nl | ACTH-afhankelijk syndroom van Cushing | ACTH-afhankelijk CS · Adrenocorticotroop hormoon-afhankelijke syndroom van Cushing · Corticotropineafhankelijk syndroom van Cushing |
| pt | Síndrome Cushing dependente de ACTH | |
| pl | Zespół Cushinga zależny od ACTH | CS zależny od ACTH · Zespół Cushinga zależny od hormonu adrenokortykotropowego · Zespół Cushinga zależny od kortykotropiny |
| cs | ACTH dependentní Cushingův syndrom | ACTH-dependentní CS · Adrenokortikotropní hormon-dependentní Cushingův syndrom · Kortikotropin-dependentní Cushingův syndrom |
| uk | АКТГ-залежний синдром Кушинга | АКТГ-залежний КС · Синдром Кушинга, залежний від адренокортикотропного гормону · Кортикотропін-залежний синдром Кушинга |
| zh | 促肾上腺皮质激素依赖性库欣综合征 | ACTH依赖性CS |
| tr | ACTH-bağımlı Cushing sendromu | ACTH-bağımlı CS · Corticotropin-bağımlı Cushing sendromu · Adrenokortikotropik hormon-bağımlı Cushing sendromu |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E24.0 | BTNT | specific | validated |
| MONDO | 0020528 | E | validated | |
| UMLS | C0342442 | 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 (3):
- ORPHA:641613Endogenous Cushing syndrome
rare endocrine diseases - ORPHA:399849Rare female infertility due to an adrenal disorder
rare infertility disorders - ORPHA:399584Rare male infertility due to adrenal disorder
rare infertility disorders
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.55 / 100 000 | Worldwide | Value and class | Not yet validated | 20829610[PMID]_18226430[PMID]_15903005[PMID] |
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet validated | ORPHANET |
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/99892
- This record as JSON: /data/d/99892.json