{"orpha":357502,"name":"Idiopathic nephrotic syndrome","type":"Clinical group","group":"Group of disorders","status":"active","flags":[],"synonyms":[],"definition":"A rare primary glomerular group of diseases characterized by the triad of edema, massive, or nephrotic-range, proteinuria and hypoalbuminemia, for which there is no known cause. Depending on response to treatment, disease is distinguished into steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS), with the latter being further divided, depending on occurrence, into familial or sporadic forms.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-357502/","orphanet_url":"https://www.orpha.net/en/disease/detail/357502","names":{"fr":{"name":"Syndrome néphrotique idiopathique","synonyms":["Néphrose idiopathique"],"definition":"Ensemble de néphropathies glomérulaires primitives rares, caractérisé par la triade oedèmes, protéinurie importante ou franchement néphrotique et hypoalbuminémie, et sans cause connue. Selon la réponse au traitement, on distingue les syndromes néphrotiques cortico-sensibles et les syndromes néphrotiques cortico-résistants, et, parmi ces derniers, les formes familiales et les formes sporadiques selon leur contexte de survenue."},"de":{"name":"Nephrotisches Syndrom, idiopathisches","definition":"Eine seltene Gruppe primärer glomerulärer Erkrankungen, die durch die Trias von Ödemen, massiver oder nephrotischer Proteinurie und Hypoalbuminämie gekennzeichnet sind und für die es keine bekannte Ursache gibt. Je nach Ansprechen auf die Behandlung wird die Krankheit in ein steroidempfindliches nephrotisches Syndrom (SSNS) und ein steroidresistentes nephrotisches Syndrom (SRNS) unterschieden, wobei letzteres je nach Auftreten weiter in familiäre oder sporadische Formen unterteilt wird."},"es":{"name":"Síndrome nefrótico idiopático","definition":"Es un grupo de enfermedades glomerulares primarias poco frecuentes caracterizado por la tríada de edema, proteinuria masiva o en rango nefrótico e hipoalbuminemia, de etiología desconocida. En función de la respuesta al tratamiento, la enfermedad se diferencia en síndrome nefrótico corticosensible (SNCS) y síndrome nefrótico corticorresistente (SNCR). A su vez, este último se divide según su ocurrencia en formas familiares o esporádicas."},"it":{"name":"Sindrome nefrosica idiopatica","definition":"Comprende un gruppo di malattie primitive rare dei glomeruli, caratterizzate dalla triade edema, proteinuria marcata o di tipo nefrosico ed ipoalbuminemia. L'eziologia non è nota. A seconda della risposta al trattamento, la malattia viene distinta nella sindrome nefrosica sensibile agli steroidi (SSNS) o nella sindrome nefrosica resistente agli steroidi (SRNS). In base all'occorrenza, la seconda forma viene suddivisa ulteriormente in una forma familiare e in una forma sporadica."},"nl":{"name":"Idiopathisch nefrotisch syndroom","definition":"Een groep zeldzame primaire glomerulaire ziekten die gekarakteriseerd worden door de triade van oedeem, massale proteïnurie of proteïnurie in het nefrotische bereik, en hypoalbuminemie, en waarvan de oorzaak niet gekend is. Afhankelijk van de reactie op behandeling, worden steroïdsensitief nefrotisch syndroom (SSNS) en steroïdresistent nefrotisch syndroom (SRNS) onderscheiden, en deze laatste wordt verder onderverdeeld in familiale en sporadische vormen, afhankelijk van het voorkomen."},"pt":{"name":"Síndrome nefrótica idiopática"},"pl":{"name":"Idiopatyczny zespół nefrotyczny","synonyms":["Idiopatyczny zespół nerczycowy"]},"cs":{"name":"Idiopatický nefrotický syndrom"},"uk":{"name":"Ідіопатичний нефротичний синдром","definition":"A rare primary glomerular group of diseases characterized by the triad of edema, massive, or nephrotic-range, proteinuria and hypoalbuminemia, for which there is no known cause. Depending on response to treatment, disease is distinguished into steroid-sensitive nephrotic syndrome (SSNS) and steroid-resistant nephrotic syndrome (SRNS), with the latter being further divided, depending on occurrence, into familial or sporadic forms."},"zh":{"name":"特发性肾病综合征","definition":"本病是一种罕见的原发性肾小球疾病，其特征为水肿、大量或肾病范围的蛋白尿和低白蛋白血症的三联征，其原因不明。根据对治疗的反应，疾病可分为类固醇敏感性肾病综合征(SSNS)和类固醇抗性肾病综合征(SRNS)，后者根据发生情况可进一步分为家族性或散发性。"},"tr":{"name":"İdiyopatik nefrotik sendrom"}},"xrefs":[{"source":"MONDO","code":"0018170","qualifier":"E","icd_relation":null,"validated":true},{"source":"MONDO","code":"18170","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C3496337","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":567564,"name":"Nephrotic syndrome without extrarenal manifestations","classifications":[188,233]}],"children":[{"orpha":567548,"name":"Idiopathic steroid-resistant nephrotic syndrome","type":"Clinical syndrome"},{"orpha":69061,"name":"Idiopathic steroid-sensitive nephrotic syndrome","type":"Clinical syndrome"},{"orpha":567546,"name":"Idiopathic steroid-sensitive nephrotic syndrome with secondary steroid resistance","type":"Clinical syndrome"}],"genes":[],"genes_via_subtypes":[],"inheritance":[],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":null}