{"orpha":188,"name":"Systemic capillary leak syndrome","type":"Disease","group":"Disorder","status":"active","flags":[],"synonyms":["Capillary hyperpermeability syndrome","Capillary leak syndrome","Clarkson disease","Idiopathic capillary leak syndrome","SCLS"],"definition":"Systemic capillary leak syndrome (SCLS) is a severe systemic disease due to increased capillary permeability, characterized by episodes of hypotension, edema and hypovolemia.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-188/","orphanet_url":"https://www.orpha.net/en/disease/detail/188","names":{"fr":{"name":"Syndrome de fuite capillaire systémique","synonyms":["Syndrome d'hyperperméabilité capillaire","Syndrome de fuite capillaire","Syndrome de fuite capillaire idiopathique","Maladie de Clarkson","SCLS","SFC","SFCI"],"definition":"Le syndrome de fuite capillaire systémique (SFC) est une maladie systémique sévère causée par une augmentation de la perméabilité capillaire, et caractérisée par des épisodes d'hypotension, d'oedème et d'hypovolémie."},"de":{"name":"Capillary-Leak-Syndrom, systemisches","synonyms":["Clarkson-Krankheit","Kapillarleck-Syndrom"],"definition":"Das Systemische Capillary-Leak-Syndrom (SCLS) ist eine schwere systemische Erkrankung, verursacht durch erhöhte Kapillarpermeabilität. Charakteristische Merkmale des Syndroms sind Hypotensions-Episoden, Ödeme und Hypovolämie."},"es":{"name":"Síndrome de extravasación capilar sistémica","synonyms":["SCLS","Síndrome de Clarkson","Síndrome de fuga capilar","Síndrome de fuga capilar idiopática","Síndrome de hiperpermeabilidad capilar"],"definition":"El síndrome de extravasación capilar (SCLS) es una grave enfermedad sistémica debida a un incremento de la permeabilidad capilar, caracterizada por episodios de hipotensión, edema e hipovolemia."},"it":{"name":"Sindrome sistemica da aumentata permeabilità capillare","synonyms":["Malattia di Clarkson","SCLS","Sindrome da aumentata permeabilità capillare","Sindrome da iperpermeabilità capillare","Sindrome idiopatica da aumentata permeabilità capillare"],"definition":"La sindrome sistemica da aumentata permeabilità capillare (SCLS) è una grave malattia sistemica da aumento della permeabilità capillare, con episodi di ipotensione, edema e ipovolemia."},"nl":{"name":"Systemisch capillair leksyndroom","synonyms":["Capillair leksyndroom","Capillaire hyperpermeabiliteitsyndroom","Idiopathisch capillair leksyndroom","SCLS","Ziekte van Clarkson"],"definition":"Systemisch capillair leksyndroom (SCLS) is een ernstige systemische aandoening ten gevolge van een verhoogde capillaire permeabiliteit en wordt gekarakteriseerd door episodes met hypotensie, oedeem en hypovolemie."},"pt":{"name":"Síndrome de derrame capilar sistémico","synonyms":["Doença de Clarkson","SCLS","Síndrome de derrame capilar","Síndrome de derrame capilar idiopático","Síndrome de hiperpermeabilidade capilar"]},"pl":{"name":"Uogólniony zespół przesiąkania włośniczek","synonyms":["Choroba Clarksona","Idiopatyczny zespół przesiąkania włośniczek","SCLS","Zespół przesiąkania włośniczek","Zespół zwiększonej przepuszczalności naczyń włosowatych"]},"cs":{"name":"Systémový syndrom děravých kapilár","synonyms":["Syndrom hyperpermeability kapilár","Clarksonova choroba","SCLS","Syndrom děravých kapilár","Idiopatický syndrom děravých kapilár"]},"uk":{"name":"Синдром системного капілярного просочування","synonyms":["Синдром гіперпроникності капілярів","Синдром капілярного витоку","Хвороба Кларксона","Синдром ідіопатичного капілярного просочування","SCLS"],"definition":"Systemic capillary leak syndrome (SCLS) is a severe systemic disease due to increased capillary permeability, characterized by episodes of hypotension, edema and hypovolemia."},"zh":{"name":"系统性毛细血管渗漏综合征","synonyms":["毛细血管高渗透性综合征"]},"tr":{"name":"Sistemik kılcal sızıntı sendromu","synonyms":["SCLS","Clarkson hastalığı","Kılcal sızıntı sendromu","İdiyopatik kılcal sızıntı sendromu","Kılcal aşırı geçirgenlik sendromu"]}},"xrefs":[{"source":"ICD-10","code":"I78.8","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"BD5Y","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1008326816"},{"source":"MONDO","code":"1956","qualifier":"E","icd_relation":null,"validated":true},{"source":"MeSH","code":"D019559","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0343084","qualifier":"E","icd_relation":null,"validated":true},{"source":"GARD","code":"1084","qualifier":"E","icd_relation":null,"validated":true},{"source":"MedDRA","code":"10007196","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":98023,"name":"Rare systemic or rheumatologic disease"},"parents":[{"orpha":182222,"name":"Rare systemic disease","classifications":[196]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Not applicable"],"onset":["Adult"],"prevalence":[{"type":"Cases/families","qualification":"Case(s)","class":null,"value":150.0,"area":"Worldwide","status":"Validated","source":"[EXPERT]"},{"type":"Point prevalence","qualification":"Class only","class":"<1 / 1 000 000","value":0.0,"area":"Worldwide","status":"Validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2018-01-22","source":null,"terms":[{"hpo":"HP:0001974","label":"Increased total leukocyte count","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0010741","label":"Pedal edema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0002027","label":"Abdominal pain","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0025142","label":"Constitutional symptom","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002014","label":"Diarrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0012378","label":"Fatigue","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0002615","label":"Hypotension","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0003326","label":"Myalgia","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001733","label":"Pancreatitis","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0100598","label":"Pulmonary edema","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0031417","label":"Rhinorrhea","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0001824","label":"Weight loss","frequency":"Frequent (79-30%)","criteria":null},{"hpo":"HP:0000091","label":"Abnormal renal tubule morphology","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0011675","label":"Arrhythmia","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006543","label":"Cardiorespiratory arrest","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012735","label":"Cough","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0006775","label":"Multiple myeloma","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0012819","label":"Myocarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0100520","label":"Oliguria","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0001701","label":"Pericarditis","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0002202","label":"Pleural effusion","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0000083","label":"Renal insufficiency","frequency":"Occasional (29-5%)","criteria":null},{"hpo":"HP:0004936","label":"Venous thrombosis","frequency":"Occasional (29-5%)","criteria":null}]}}