Acute adrenal insufficiency
Synonyms: Acute adrenal failure · Acute adrenocortical insufficiency · Addisonian crisis · Adrenal crisis · Adrenocortical crisis
- Type
- Clinical syndrome
- Group
- Disorder
- Inheritance
- Autosomal recessive, Not applicable, X-linked dominant
- Age of onset
- All ages
- Status
- active
A primary adrenal insufficiency caused by a sudden defective production of adrenal steroids (cortisol and aldosterone). It represents an emergency, thus the rapid recognition and prompt therapy are critical for survival even before the diagnosis is made.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute adrenal insufficiency | Acute adrenal failure · Acute adrenocortical insufficiency · Addisonian crisis · Adrenal crisis · Adrenocortical crisis |
| fr | Insuffisance surrénalienne aiguë | Crise addisonienne · Crise cortico-surrénalienne · Crise corticosurrénalienne · Crise surrénalienne · Insuffisance cortico-surrénale aiguë · Insuffisance cortico-surrénalienne aiguë · Insuffisance corticosurrénale aiguë · Insuffisance corticosurrénalienne aiguë · Insuffisance surrénale aiguë |
| de | Nebenniereninsuffizienz, akute | Addison-Krise · Akute Nebennierenrindeninsuffizienz · Nebennierenkrise · Nebennierenrindenkrise · Nebennierenversagen, akutes |
| es | Insuficiencia suprarrenal aguda | Fallo suprarrenal agudo · Insuficiencia adrenocortical aguda · Crisis corticosuprarrenal · Crisis de Addison · Crisis suprarrenal |
| it | Insufficienza surrenalica acuta | Crisi addisoniana · Crisi adrenocorticale · Crisi surrenalica · Insufficienza acuta dei surreni · Insufficienza adrenocorticale acuta |
| nl | Acute adrenale insufficiëntie | Acute adrenocorticale insufficiëntie · Acute bijnierinsufficiëntie · Acute bijnierschorsinsufficiëntie · Acuut bijnierfalen · Addisoniaanse crisis · Adrenocorticale crisis · Bijniercrisis |
| pt | Insuficiência supra-renal aguda | Crise addisoniana · Crise supra-renal |
| pl | Ostra niewydolność nadnerczy | Ostra niewydolność kory nadnerczy · Ostra niewydolność nadnerczy · Przełom addisonoidalny · Przełom korowo-nadnerczowy · Przełom nadnerczowy |
| cs | Akutní adrenální insuficience | Akutní selhání nadledvin · Akutní adrenokortikální insuficience · Addisonská krize · Adrenální krize · Adrenokortikální krize |
| uk | Гостра надниркова недостатність | Гостра надниркова недостатність · Гостра адренокортикальна недостатність · Аддісонова криза · Наднирниковий криз · Адренокортикальний криз |
| zh | 急性肾上腺皮质功能不全 | 急性肾上腺皮质功能减退 |
| tr | Akut adrenal yetmezlik | Adrenal kriz · Addison krizi · Akut adrenal yetmezlik · Adrenokortikal kriz · Akut adrenokortikal yetmezlik |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E27.2 | NTBT | attributed | validated |
| ICD-11 | 5A74.1 | E | specific | validated |
| MONDO | 0019801 | E | validated | |
| UMLS | C0151467 | E | validated | |
| MedDRA | 10001389 | 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:101958Primary adrenal insufficiency
rare endocrine 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 | 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
44 annotations · validated 2016-09-02| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Obligate (100%) | ||
| HP:0008163 | Decreased circulating cortisol level | |
| HP:0008207 | Primary adrenal insufficiency | |
| Very frequent (99-80%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0002039 | Anorexia | |
| HP:0002960 | Autoimmunity | |
| HP:0002019 | Constipation | |
| HP:0002014 | Diarrhea | |
| HP:0001508 | Failure to thrive | |
| HP:0012378 | Fatigue | |
| HP:0000953 | Hyperpigmentation of the skin | |
| HP:0002615 | Hypotension | |
| HP:0011106 | Hypovolemia | |
| HP:0003154 | Increased circulating ACTH level | |
| HP:0001324 | Muscle weakness | |
| HP:0002017 | Nausea and vomiting | |
| HP:0001824 | Weight loss | |
| Frequent (79-30%) | ||
| HP:0011948 | Acute respiratory tract infection | |
| HP:0008226 | Androgen insufficiency | |
| HP:0004319 | Decreased circulating aldosterone level | |
| HP:0012364 | Decreased urinary potassium | |
| HP:0002153 | Hyperkalemia | |
| HP:0005976 | Hyperkalemic metabolic acidosis | |
| HP:0002149 | Hyperuricemia | |
| HP:0001943 | Hypoglycemia | |
| HP:0002902 | Hyponatremia | |
| HP:0001252 | Hypotonia | |
| HP:0000848 | Increased circulating renin level | |
| HP:0001897 | Normocytic anemia | |
| HP:0001278 | Orthostatic hypotension | |
| HP:0000083 | Renal insufficiency | |
| HP:0000127 | Renal salt wasting | |
| HP:0001250 | Seizure | |
| Occasional (29-5%) | ||
| HP:0000835 | Adrenal hypoplasia | |
| HP:0002829 | Arthralgia | |
| HP:0030018 | Decreased female libido | |
| HP:0000823 | Delayed puberty | |
| HP:0000958 | Dry skin | |
| HP:0003072 | Hypercalcemia | |
| HP:0001658 | Myocardial infarction | |
| HP:0030083 | Salt craving | |
| HP:0002215 | Sparse axillary hair | |
| HP:0001297 | Stroke | |
| HP:0002321 | Vertigo | |
| HP:0001045 | Vitiligo | |
Run the differential on the 12 obligate / very frequent terms
Annotation source: 20400889[PMID]_24695602[PMID]_25295623[PMID]_20399314[PMID]_15654180[PMID]_24613067[PMID]_23697517[PMID]_18000094[PMID]_16968806[PMID]_19574315[PMID]_15950720[PMID]_8614434[PMID]_11443143[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/95409
- This record as JSON: /data/d/95409.json