{"orpha":70578,"name":"Adult acute respiratory distress syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Adult ARDS"],"definition":"A very severe form of acute pulmonary failure secondary to capillary permeability impairment. The symptoms include dyspnea, hypotension and multivisceral failure. The disease is characterized by bilateral pulmonary infiltrates and severe hypoxemia due to increased alveolar-capillary permeability. The severity depends on the degree of alveolar epithelial injury, with a mortality rate of 30-50%.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-70578/","orphanet_url":"https://www.orpha.net/en/disease/detail/70578","names":{"fr":{"name":"Syndrome de détresse respiratoire aiguë de l'adulte","synonyms":["Maladie des membranes hyalines de l'adulte"],"definition":"Le syndrome de détresse respiratoire aiguë de l'adulte est une forme très sévère de défaillance pulmonaire aiguë secondaire à une altération de la perméabilité capillaire. Les symptômes sont une dyspnée, une hypotension et une défaillance multiviscérale. Le syndrome de détresse respiratoire aiguë de l'adulte est caractérisé par des infiltrations pulmonaires bilatérales et une hypoxémie sévère dû une perméabilité alvéolaire-capillaire accrue. La sévérité dépend du degré de la lésion épithéliale alvéolaire avec une mortalité de 30 à 50 %."},"de":{"name":"Akutes Atemnotsyndrom des Erwachsenen","synonyms":["ARDS des Erwachsenen","Atemnotsyndrom, adultes"],"definition":"Das Adulte Atemnotsyndrom ist eine entzündliche Lungenerkrankung mit beidseitigen Lungeninfiltraten und schwerer Hypoxämie durch erhöhte alveolär-kapilläre Permeabilität. Symptome sind schwere, schnelle Atmung, Kurzatmigkeit, niedriger Blutdruck und Organversagen. Der Schweregrad wird durch das Ausmaß der Schädigung des Alveolarepithels bestimmt."},"es":{"name":"Síndrome de dificultad respiratoria agudo del adulto","synonyms":["SDRA del adulto","Síndrome de distrés respiratorio agudo del adulto"],"definition":"Es una lesión pulmonar inflamatoria caracterizada por infiltraciones pulmonares bilaterales e hipoxemia grave causadas por el incremento de la permeabilidad alveolo-capilar. Los síntomas son respiración rápida y trabajosa, falta de aliento, presión sanguínea baja y fallo orgánico. La gravedad depende del grado de la lesión alveolo-epitelial."},"it":{"name":"Sindrome da distress respiratorio acuto dell'adulto","synonyms":["ARDS dell'adulto"],"definition":"La sindrome da distress respiratorio acuto dell'adulto è una lesione infiammatoria dei polmoni, caratterizzata da infiltrati polmonari bilaterali e ipossemia grave da aumento della permeabilità della membrana alveolo-capillare. I sintomi comprendono l'accelerazione del respiro, che appare affaticato e corto, l'ipotensione arteriosa e l'insufficienza d'organo. La gravità del quadro clinico dipende dall'entità delle lesioni epiteliali alveolari."},"nl":{"name":"Acuut ademhalingsnoodsyndroom bij volwassenen","synonyms":["ARDS bij volwassenen","Acute respiratory distress-syndroom bij volwassenen"],"definition":"Een zeer ernstige vorm van acuut longfalen secundair aan verstoorde permeabiliteit van capillairen. De symptomen omvatten dyspneu, hypotensie en multivisceraal falen. De ziekte wordt gekarakteriseerd door bilaterale pulmonale infiltraten en ernstige hypoxemie door verhoogde alveolaire-capillaire permeabiliteit. De ernst hangt af van de mate van beschadiging van alveolair epitheel, met een mortaliteit van 30-50%."},"pt":{"name":"Síndrome de dificuldade respiratória aguda do adulto","synonyms":["SDRA do adulto"]},"pl":{"name":"Zespół ostrej niewydolności oddechowej dorosłych","synonyms":["ARDS dorosłych"]},"cs":{"name":"Syndrom akutní respirační tísně u dospělých","synonyms":["Adultní ARDS"]},"uk":{"name":"Гострий респіраторний дистрес-синдром у дорослих","synonyms":["ARDS у дорослих"],"definition":"A very severe form of acute pulmonary failure secondary to capillary permeability impairment. The symptoms include dyspnea, hypotension and multivisceral failure. The disease is characterized by bilateral pulmonary infiltrates and severe hypoxemia due to increased alveolar-capillary permeability. The severity depends on the degree of alveolar epithelial injury, with a mortality rate of 30-50%."},"zh":{"name":"成人急性呼吸窘迫综合征","synonyms":["成人ARDS"]},"tr":{"name":"Yetişkin akut solunum sıkıntısı sendromu","synonyms":["Erişkin ARDS"]}},"xrefs":[{"source":"ICD-10","code":"J80","qualifier":"E","icd_relation":"specific","validated":true},{"source":"ICD-11","code":"CB00","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1466842111"},{"source":"MONDO","code":"0100130","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C5680115","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":97955,"name":"Rare respiratory disease"},"parents":[{"orpha":264740,"name":"Primary interstitial lung disease specific to adulthood","classifications":[184,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Annual incidence","qualification":"Value and class","class":"6-9 / 10 000","value":64.0,"area":"United States","status":"Validated","source":"16236739[PMID]"},{"type":"Point prevalence","qualification":"Value and class","class":"1-5 / 10 000","value":51.0,"area":"United States","status":"Not yet validated","source":"ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":11.5,"area":"Iceland","status":"Not yet validated","source":"23216361[PMID]_10351930[PMID]_ ORPHANET"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":7.2,"area":"Spain","status":"Validated","source":"21997128[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-9 / 100 000","value":4.9,"area":"Finland","status":"Validated","source":"10579250[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":13.5,"area":"Sweden","status":"Validated","source":"10351930[PMID]"},{"type":"Annual incidence","qualification":"Value and class","class":"1-5 / 10 000","value":13.5,"area":"Denmark","status":"Validated","source":"10351930[PMID]"},{"type":"Point prevalence","qualification":"Class only","class":"1-5 / 10 000","value":0.0,"area":"Europe","status":"Validated","source":"European Medicines Agency 2008[OTHER]"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2019-06-03","source":"30078618[PMID]_29657094[PMID]_28406724[PMID]_30755248[PMID]","terms":[{"hpo":"HP:0012415","label":"Abnormal blood gas level","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011117","label":"Abnormal circulating interleukin concentration","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002094","label":"Dyspnea","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0012418","label":"Hypoxemia","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002113","label":"Pulmonary infiltrates","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0011118","label":"Abnormality of tumor necrosis factor secretion","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0030783","label":"Increased circulating interleukin 6 concentration","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001942","label":"Metabolic acidosis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100598","label":"Pulmonary edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002878","label":"Respiratory failure","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100806","label":"Sepsis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031273","label":"Shock","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002090","label":"Pneumonia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001953","label":"Diabetic ketoacidosis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0001733","label":"Pancreatitis","frequency":"Very rare (<4-1%)","criteria":null},{"hpo":"HP:0002633","label":"Vasculitis","frequency":"Very rare (<4-1%)","criteria":null}]}}