ORPHA:101959
Chronic primary adrenal insufficiency
Synonyms: CPAI · Chronic adrenocorticoid insufficiency
- Type
- Category
- Group
- Group of disorders
- Inheritance
- Multigenic/multifactorial
- Age of onset
- All ages
- Status
- active
Chronic primary adrenal insufficiency (CPAI) is a chronic disorder of the adrenal cortex resulting in the inadequate production of glucocorticoid and mineralocorticoid hormones.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Chronic primary adrenal insufficiency | CPAI · Chronic adrenocorticoid insufficiency |
| fr | Insuffisance surrénale primaire chronique | CPAI |
| de | Nebenniereninsuffizienz, chronische primäre | Adrenokortikale Insuffizienz, chronische · CPAI |
| es | Insuficiencia suprarrenal crónica primaria | |
| it | Insufficienza surrenalica primitiva cronica | CPAI · Insufficienza adrenocorticale cronica |
| nl | Chronische primaire adrenale insufficiëntie | CPAI · Chronische bijnierschorsinsufficiëntie · Chronische primaire bijnierinsufficiëntie |
| pt | Insuficiência supra-renal primária crónica | CPAI · Insuficiência adrenocortical crónica |
| pl | Przewlekła pierwotna niedoczynność nadnerczy | CPAI · Przewlekła niedoczynność kory nadnerczy |
| cs | Chronická primární adrenální insuficience | CPAI · Chronická adrenokortikoidní insuficience |
| uk | Хронічна первинна надниркова недостатність | Хронічна адренокортикоїдна недостатність · CPAI |
| zh | 慢性原发性肾上腺功能不全 | 慢性肾上腺功能不全 |
| tr | Kronik primer adrenal yetmezlik | CPAI · Kronik adrenokortikoid yetmezliği |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| MONDO | 0015129 | E | validated | |
| MONDO | 15129 | E | validated | |
| UMLS | C5848257 | E | validated | |
| GARD | 19803 | 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 (1):
- ORPHA:101958Primary adrenal insufficiency
rare endocrine diseases
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Genes
0 directOrphanet records no gene directly on this code.
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Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.4 / 100 000 | Europe | Value and class | Validated | [EXPERT] |
| Point prevalence | 1-5 / 10 000 | 14 / 100 000 | Europe | Value and class | Validated | 12788587[PMID]_[EXPERT] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
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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/101959
- This record as JSON: /data/d/101959.json