{"orpha":454831,"name":"Acute radiation syndrome","type":"Particular clinical situation in a disease or syndrome","group":"Disorder","status":"active","flags":[],"synonyms":["Acute radiation sickness"],"definition":"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.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-454831/","orphanet_url":"https://www.orpha.net/en/disease/detail/454831","names":{"fr":{"name":"Syndrome d'irradiation aiguë","synonyms":["Maladie des rayons aïgue"],"definition":"Maladie radio-induite rare résultant de l'exposition du corps entier à de fortes doses de rayonnement pénétrant (>0,7 gray) dans un laps de temps très court (généralement de quelques minutes), caractérisée par un syndrome hématopoïétique avec pancytopénie (dont les symptômes légers peuvent apparaître à 0,3 gray), un syndrome gastro-intestinal entraînant une infection, une déshydratation et un déséquilibre électrolytique dont l'issue est le plus souvent fatale (survenant à des doses >10 gray), et un syndrome neurovasculaire avec atteinte du système nerveux central, diarrhée aqueuse, convulsions, coma et décès dans les trois jours suivant l'exposition (survenant à des doses >50 gray). Le syndrome suit quatre phases cliniques de durée variable (phase prodromique/phase de latence/phase d'état/phase de rémission ou décès)."},"de":{"name":"Akutes Strahlensyndrom","synonyms":["Akute Strahlenkrankheit"],"definition":"Eine seltene strahleninduzierte Erkrankung, die durch eine Ganzkörperexposition mit hohen Dosen durchdringender Strahlung (>0,7 Gray) innerhalb eines sehr kurzen Zeitraums (in der Regel Minuten) entsteht und durch ein Knochenmarkssyndrom mit Panzytopenie (leichte Symptome können bereits bei 0. 3 Gray), das gastrointestinale Syndrom mit meist tödlichen Infektionen, Dehydrierung und Elektrolytungleichgewicht (bei Dosen >10 Gray) und das kardiovaskuläre/zentralnervöse Syndrom mit wässrigem Durchfall, Krämpfen, Koma und Tod innerhalb von drei Tagen nach der Exposition (bei Dosen >50 Gray). Das Syndrom entwickelt sich in vier klinischen Stadien (Prodromalstadium/spätes Auftreten/manifeste Erkrankung/Erholung oder Tod) von unterschiedlicher Dauer."},"es":{"name":"Síndrome de irradiación aguda","synonyms":["Enfermedad de irradiación aguda"],"definition":"Es un trastorno poco frecuente inducido por la radiación que resulta de la exposición de todo el cuerpo a dosis elevadas de radiación penetrante (>0,7 Gray) en un período de tiempo muy breve (normalmente minutos) y caracterizado por síndrome de médula ósea con pancitopenia (síntomas leves que pueden estar presentes a partir de 0,3 Gray), síndrome gastrointestinal que resulta en infección, deshidratación y desequilibrio electrolítico en su mayoría fatales (que ocurre a dosis >10 Gray), y síndrome cardiovascular/del sistema nervioso central con diarrea acuosa, crisis epiléptica, coma y fallecimiento dentro de los tres días posteriores a la exposición (que ocurre a dosis >50 Gray). El síndrome se desarrolla en cuatro etapas clínicas (prodrómica/latente/enfermedad manifiesta/recuperación o muerte) de duración variable."},"it":{"name":"Sindrome acuta da irradiazione","synonyms":["Malattia acuta da irradiazione"]},"nl":{"name":"Acuut stralingssyndroom","synonyms":["Acute stralingsziekte"],"definition":"Een zeldzame stralingsgeïnduceerde aandoening als gevolg van blootstelling van geheel lichaam aan grote doses doordringende straling (meer dan 0,7 Gray) op korte tijd (doorgaans minuten), gekenmerkt door beenmerg-syndroom met pancytopenie (milde symptomen die al kunnen optreden bij 0,3 Gray), gastro-intestinaal syndroom dat resulteert in overwegend fatale infectie, dehydratie en verstoorde elektrolytenbalans (bij doses boven 10 Gray), en cardiovasculair/centraal zenuwstelsel-syndroom met waterige diarree, convulsies, coma, en sterfte binnen drie dagen na blootstelling (bij doses boven 50 Gray). Het syndroom ontwikkelt zich in vier klinische stadia (prodromaal/latent/manifeste ziekte/herstel of sterfte) met variabele duur."},"pt":{"name":"Síndrome agudo de radiação","definition":"Doença rara induzida por radiação, resultante da exposição global do corpo a grandes doses de radiação penetrante (>0,7 Gray) num período de tempo muito curto (geralmente minutos) e caracterizado por síndrome medular ósseo com pancitopenia (cujos sintomas leves podem ocorrer com 0,3 Gray), síndrome gastrointestinal resultando essencialmente em infeção fatal, desidratação e desequilíbrio eletrolítico (ocorrendo em doses >10 Gray), e síndrome do sistema cardiovascular/nervoso central com diarreia aquosa, convulsões, coma e morte em três dias após a exposição (ocorrendo em doses >50 Gray). A síndrome desenvolve-se em quatro estágios clínicos (doença prodrómica/latente/manifestada/recuperação ou morte) de duração variável."},"pl":{"name":"Ostry zespół popromienny","synonyms":["Ostra choroba popromienna"],"definition":"Rzadko występujące zaburzenie wywołane promieniowaniem, związane z narażeniem całego ciała na duże dawki promieniowania (sup 0,7 Gy) w bardzo krótkim czasie (zwykle minut) i manifestujące się jako postać hematologiczna z pancytopenią (łagodne objawy mogą wystąpić już przy ekspozycji na dawkę 0,3Gy), postać jelitowa z odwodnieniem i zaburzeniami równowagi elektrolitowej (występująca przy dawkach sup 10 Gy), postać mózgowa/enzymatyczna z drgawkami, śpiączką, biegunką i śmiercią w ciągu 3 dni od narażenia (występująca przy dawkach sup 50Gy). Zespół rozwija się w czterech stadiach klinicznych (okres prodromalny/choroba utajona/jawna/wyzdrowienie lub śmierć) o zmiennym czasie trwania."},"cs":{"name":"Akutní syndrom z ozáření","synonyms":["Akutní radiační syndrom"]},"uk":{"name":"Гострий променевий синдром","synonyms":["Гостра променева хвороба"],"definition":"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."},"zh":{"name":"急性放射病综合征","synonyms":["急性放射病"]},"tr":{"name":"Akut radyasyon sendromu","synonyms":["Akut radyasyon hastalığı"]}},"xrefs":[{"source":"ICD-10","code":"T66","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"NF00","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"MONDO","code":"0033938","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D054508","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0520799","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":108999,"name":"Rare disorder due to toxic effects"},"parents":[{"orpha":521132,"name":"Radiation-induced disorder","classifications":[204]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"1-9 / 1 000 000","value":0.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2018[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2023-02-10","source":"28650707[PMID]_18049222[PMID]_20445398[PMID]","terms":[{"hpo":"HP:0004334","label":"Dermal atrophy","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0001892","label":"Abnormal bleeding","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001945","label":"Fever","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002315","label":"Headache","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000953","label":"Hyperpigmentation of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001010","label":"Hypopigmentation of the skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001888","label":"Lymphopenia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001324","label":"Muscle weakness","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0040189","label":"Scaling skin","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001009","label":"Telangiectasia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001873","label":"Thrombocytopenia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002013","label":"Vomiting","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000518","label":"Cataract","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001913","label":"Granulocytopenia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000962","label":"Hyperkeratosis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011123","label":"Inflammatory abnormality of the skin","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006515","label":"Interstitial pneumonitis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0007185","label":"Loss of consciousness","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001250","label":"Seizure","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0200042","label":"Skin ulcer","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002321","label":"Vertigo","frequency":"Occasional (29-5%)","criteria":null}]}}