Acute radiation syndrome
Synonyms: Acute radiation sickness
- Type
- Particular clinical situation in a disease or syndrome
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare radiation-induced disorder resulting from whole body exposure to large doses of penetrating radiation (>0.7 Gray) within a very short period of time (usually minutes) and characterized by bone marrow syndrome with pancytopenia (mild symptoms of which may occur already at 0.3 Gray), gastrointestinal syndrome resulting in mostly fatal infection, dehydration, and electrolyte imbalance (occurring at doses >10 Gray), and cardiovascular/central nervous system syndrome with watery diarrhea, convulsions, coma, and death within three days of exposure (occurring at doses >50 Gray). The syndrome develops in four clinical stages (prodromal/latent/manifest illness/recovery or death) of variable duration.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Acute radiation syndrome | Acute radiation sickness |
| fr | Syndrome d'irradiation aiguë | Maladie des rayons aïgue |
| de | Akutes Strahlensyndrom | Akute Strahlenkrankheit |
| es | Síndrome de irradiación aguda | Enfermedad de irradiación aguda |
| it | Sindrome acuta da irradiazione | Malattia acuta da irradiazione |
| nl | Acuut stralingssyndroom | Acute stralingsziekte |
| pt | Síndrome agudo de radiação | |
| pl | Ostry zespół popromienny | Ostra choroba popromienna |
| cs | Akutní syndrom z ozáření | Akutní radiační syndrom |
| uk | Гострий променевий синдром | Гостра променева хвороба |
| zh | 急性放射病综合征 | 急性放射病 |
| tr | Akut radyasyon sendromu | Akut radyasyon hastalığı |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | T66 | E | specific | validated |
| ICD-11 | NF00 | NTBT | attributed | validated |
| MONDO | 0033938 | E | validated | |
| MeSH | D054508 | E | validated | |
| UMLS | C0520799 | 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:521132Radiation-induced disorder
rare disorders due to toxic effects
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 | 1-9 / 1 000 000 | — | Europe | Class only | Validated | European Medicines Agency 2018[OTHER] |
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
24 annotations · validated 2023-02-10| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0004334 | Dermal atrophy | |
| Frequent (79-30%) | ||
| HP:0001892 | Abnormal bleeding | |
| HP:0002014 | Diarrhea | |
| HP:0001945 | Fever | |
| HP:0002315 | Headache | |
| HP:0000953 | Hyperpigmentation of the skin | |
| HP:0001010 | Hypopigmentation of the skin | |
| HP:0001888 | Lymphopenia | |
| HP:0001324 | Muscle weakness | |
| HP:0040189 | Scaling skin | |
| HP:0001009 | Telangiectasia | |
| HP:0001873 | Thrombocytopenia | |
| HP:0002013 | Vomiting | |
| Occasional (29-5%) | ||
| HP:0000518 | Cataract | |
| HP:0012378 | Fatigue | |
| HP:0001913 | Granulocytopenia | |
| HP:0000962 | Hyperkeratosis | |
| HP:0002615 | Hypotension | |
| HP:0011123 | Inflammatory abnormality of the skin | |
| HP:0006515 | Interstitial pneumonitis | |
| HP:0007185 | Loss of consciousness | |
| HP:0001250 | Seizure | |
| HP:0200042 | Skin ulcer | |
| HP:0002321 | Vertigo | |
Run the differential on the 1 obligate / very frequent terms
Annotation source: 28650707[PMID]_18049222[PMID]_20445398[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/454831
- This record as JSON: /data/d/454831.json