Primary unilateral adrenal hyperplasia
Synonyms: PUAH
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
Primary unilateral adrenal hyperplasia (PUAH) is a surgically-correctable form of primary (hyper) aldosteronism (PA) characterized by renin suppression, unilateral aldosterone hypersecretion, and moderate to severe hypertension secondary to hyperplasia of the adrenal gland.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Primary unilateral adrenal hyperplasia | PUAH |
| fr | Hyperplasie surrénalienne unilatérale primitive | = en |
| de | Nebennierenhyperplasie, primäre unilaterale | |
| es | Hiperplasia suprarrenal primaria unilateral | = en |
| it | Iperplasia surrenalica unilaterale primitiva | = en |
| nl | Primaire unilaterale bijnierhyperplasie | PUAH · Primaire unilaterale adrenale hyperplasie |
| pt | Hiperplasia supra-renal unilateral primária | = en |
| pl | Pierwotny jednostronny przerost nadnerczy | = en |
| cs | Primární unilaterální adrenální hyperplázie | = en |
| uk | Первинна одностороння гіперплазія надниркових залоз | = en |
| zh | 原发性单侧肾上腺过度增生 | = en |
| tr | Primer tek taraflı adrenal hiperplazi | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E26.0 | NTBT | attributed | validated |
| ICD-11 | 5A72.0 | NTBT | index | validated |
| MONDO | 0016504 | E | validated | |
| UMLS | C4274967 | 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 (1):
- ORPHA:231637Rare surgically correctable form of primary aldosteronism
rare endocrine diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| 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
15 annotations · validated 2016-08-31| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0003351 | Decreased circulating renin concentration | |
| HP:0011740 | Glucocortocoid-insensitive primary hyperaldosteronism | |
| HP:0000822 | Hypertension | |
| Frequent (79-30%) | ||
| HP:0008221 | Adrenal hyperplasia | |
| HP:0002900 | Hypokalemia | |
| HP:0003081 | Increased urinary potassium | |
| HP:0200114 | Metabolic alkalosis | |
| Occasional (29-5%) | ||
| HP:0000421 | Epistaxis | |
| HP:0002315 | Headache | |
| HP:0003394 | Muscle spasm | |
| HP:0001324 | Muscle weakness | |
| HP:0002018 | Nausea | |
| HP:0001962 | Palpitations | |
| HP:0001959 | Polydipsia | |
| HP:0000360 | Tinnitus | |
Run the differential on the 3 obligate / very frequent terms
Annotation source: 17180480[PMID]_25695882[PMID]_22958674[PMID]_23158191[PMID]_26934393[PMID]_27036860[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/231580
- This record as JSON: /data/d/231580.json