{"orpha":69061,"name":"Idiopathic steroid-sensitive nephrotic syndrome","type":"Clinical syndrome","group":"Disorder","status":"active","flags":[],"synonyms":[],"definition":"A rare primary glomerulopathy of unknown cause characterized by edema, nephrotic-range proteinuria and hypoalbuminemia that responds to standard prednisone treatment within 4-6 weeks.","note":null,"url":"https://raremap.alftech.space/d/ORPHA-69061/","orphanet_url":"https://www.orpha.net/en/disease/detail/69061","names":{"fr":{"name":"Syndrome néphrotique idiopathique corticosensible","definition":"Glomérulopathie primaire rare de cause inconnue caractérisée par un oedème, une protéinurie de type néphrotique et une hypoalbuminémie qui répond au traitement standard à la prednisone en quatre à six semaines."},"de":{"name":"Nephrotisches Syndrom, steroid-sensitives","definition":"Eine seltene primäre Glomerulopathie unbekannter Ursache mit Ödemen, nephrotischer Proteinurie und Hypoalbuminämie, die innerhalb von 4-6 Wochen auf eine Standardtherapie mit Prednison anspricht."},"es":{"name":"Síndrome nefrótico idiopático sensible a esteroides","definition":"Es una glomerulopatía primaria poco frecuente de causa desconocida caracterizada por edema, proteinuria en rango nefrótico e hipoalbuminemia que responde al tratamiento estándar con prednisona en 4-6 semanas."},"it":{"name":"Sindrome nefrosica idiopatica sensibile agli steroidi","definition":"Si tratta di una glomerulopatia primitiva rara ad eziologia non nota, caratterizzata da edema, proteinuria del tipo nefrosico e ipoalbuminemia, che risponde al trattamento standard con prednisone nell'arco di 4-6 settimane."},"nl":{"name":"Idiopathisch steroïdgevoelig nefrotisch syndroom","definition":"Een zeldzame primaire glomerulopathie met ongekende oorzaak, gekarakteriseerd door oedeem, proteïnurie in het nefrotische bereik en hypoalbuminemie die binnen 4-6 weken reageert op standaard behandeling met prednison."},"pt":{"name":"Síndrome nefrótico sensível aos esteróides"},"pl":{"name":"Idiopatyczny zespół nerczycowy wrażliwy na steroidy"},"cs":{"name":"Idiopatický steroid-sensitivní nefrotický syndrom"},"uk":{"name":"Ідіопатичний стероїд-чутливий нефротичний синдром","definition":"A rare primary glomerulopathy of unknown cause characterized by edema, nephrotic-range proteinuria and hypoalbuminemia that responds to standard prednisone treatment within 4-6 weeks."},"zh":{"name":"特发性类固醇敏感性肾病综合征"},"tr":{"name":"İdiyopatik steroide duyarlı nefrotik sendrom"}},"xrefs":[{"source":"OMIM","code":"615861","qualifier":"NTBT","icd_relation":null,"validated":true},{"source":"ICD-10","code":"N04.0","qualifier":"NTBT","icd_relation":"attributed","validated":true},{"source":"ICD-11","code":"GB41","qualifier":"NTBT","icd_relation":"index","validated":true,"icd11_uri":"1426920554"},{"source":"MONDO","code":"0019067","qualifier":"E","icd_relation":null,"validated":true},{"source":"UMLS","code":"C0403396","qualifier":"E","icd_relation":null,"validated":true}],"associations":[],"incoming_associations":[{"type":"Referred to","orpha":93206,"name":"OBSOLETE: Idiopathic steroid-sensitive nephrotic syndrome with focal segmental hyalinosis","status":"inactive"},{"type":"Referred to","orpha":93207,"name":"OBSOLETE: Idiopathic steroid-sensitive nephrotic syndrome with minimal change","status":"inactive"},{"type":"Referred to","orpha":93209,"name":"OBSOLETE: Idiopathic steroid-sensitive nephrotic syndrome with diffuse mesangial proliferation","status":"inactive"},{"type":"Moved to","orpha":97552,"name":"Steroid-sensitive nephrotic syndrome without renal biopsy","status":"inactive"}],"preferential_parent":{"orpha":93626,"name":"Rare renal disease"},"parents":[{"orpha":357502,"name":"Idiopathic nephrotic syndrome","classifications":[188,233]}],"children":[],"genes":[],"genes_via_subtypes":[],"inheritance":["Unknown"],"onset":["All ages"],"prevalence":[{"type":"Point prevalence","qualification":"Class only","class":"Unknown","value":0.0,"area":"Europe","status":"Not yet validated","source":"ORPHANET"}],"phenotypes":{"validation_status":"y","online":"y","validation_date":"2016-06-01","source":null,"terms":[{"hpo":"HP:0001004","label":"Lymphedema","frequency":"Very frequent (99-80%)","criteria":null},{"hpo":"HP:0000100","label":"Nephrotic syndrome","frequency":"Very frequent (99-80%)","criteria":null}]}}