1 Ocak 2017 Dergi makalesi Açık Erişim

Trautmann, Agnes; Schnaidt, Sven; Lipska-Zietkiewicz, Beata S.; Bodria, Monica; Ozaltin, Fatih; Emma, Francesco; Anarat, Ali; Melk, Anette; Azocar, Marta; Oh, Jun; Saeed, Bassam; Gheisari, Alaleh; Caliskan, Salim; Gellermann, Jutta; Higuita, Lina Maria Serna; Jankauskiene, Augustina; Drozdz, Dorota; Mir, Sevgi; Balat, Ayse; Szczepanska, Maria; Szczepanska, Maria

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    "creators": [
      {
        "affiliation": "Univ Ctr Pediat & Adolescent Med, Div Pediat Nephrol, Heidelberg, Germany", 
        "name": "Trautmann, Agnes"
      }, 
      {
        "affiliation": "Heidelberg Univ, Inst Med Biometry & Informat, Heidelberg, Germany", 
        "name": "Schnaidt, Sven"
      }, 
      {
        "affiliation": "Med Univ Gdansk, Dept Biol & Genet, Gdansk, Poland", 
        "name": "Lipska-Zietkiewicz, Beata S."
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      {
        "name": "Bodria, Monica"
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      {
        "name": "Ozaltin, Fatih"
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      {
        "affiliation": "IRCCS, Nephrol & Dialysis Unit, Childrens Hosp Bambino Gesu, Rome, Italy", 
        "name": "Emma, Francesco"
      }, 
      {
        "affiliation": "Cukurova Univ, Pediat Nephrol Dept, Med Fac, Adana, Turkey", 
        "name": "Anarat, Ali"
      }, 
      {
        "affiliation": "Hannover Med Sch, Dept Pediat Kidney Liver & Metab Dis, Hannover, Germany", 
        "name": "Melk, Anette"
      }, 
      {
        "affiliation": "Hosp Ninos Luis Calvo Mackenna, Pediat Nephrol, Fac Chile, Santiago, Chile", 
        "name": "Azocar, Marta"
      }, 
      {
        "affiliation": "Univ Childrens Hosp, Dept Pediat Nephrol, Hamburg, Germany", 
        "name": "Oh, Jun"
      }, 
      {
        "affiliation": "Kidney Hosp Damascus, Dept Pediat Nephrol, Damascus, Syria", 
        "name": "Saeed, Bassam"
      }, 
      {
        "affiliation": "Isfahan Univ Med Sci, St Al Zahra Hosp, Pediat Nephrol Dept, Esfahan, Iran", 
        "name": "Gheisari, Alaleh"
      }, 
      {
        "affiliation": "Istanbul Univ, Cerrahpasa Med Fac, Pediat Nephrol Dept, Istanbul, Turkey", 
        "name": "Caliskan, Salim"
      }, 
      {
        "affiliation": "Charite Hosp, Clin Pediat Nephrol, Berlin, Germany", 
        "name": "Gellermann, Jutta"
      }, 
      {
        "affiliation": "Hosp Pablo Tobon Uribe, Pediat Nephrol, Medellin Antioquia, Colombia", 
        "name": "Higuita, Lina Maria Serna"
      }, 
      {
        "affiliation": "Vilnius Univ, Pediat Ctr, Vilnius, Lithuania", 
        "name": "Jankauskiene, Augustina"
      }, 
      {
        "affiliation": "Jagiellonian Univ, Dept Pediat Nephrol, Med Coll, Krakow, Poland", 
        "name": "Drozdz, Dorota"
      }, 
      {
        "affiliation": "Ege Univ, Dept Pediat Nephrol, Med Fac, Izmir, Turkey", 
        "name": "Mir, Sevgi"
      }, 
      {
        "affiliation": "Gaziantep Univ, Dept Pediat Nephrol, Med Fac, Gaziantep, Turkey", 
        "name": "Balat, Ayse"
      }, 
      {
        "affiliation": "Sch Med, Div Dent, Dept Pediat, Zabrze, Poland", 
        "name": "Szczepanska, Maria"
      }, 
      {
        "affiliation": "Sch Med, Div Dent, Dept Pediat, Zabrze, Poland", 
        "name": "Szczepanska, Maria"
      }
    ], 
    "description": "We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52% in children with histopathologic findings of minimal change glomerulopathy and FSGS, respectively. Five-year ESRD-free survival rate was 21% for diffuse mesangial sclerosis. IIS responsiveness, presence of a genetic diagnosis, and FSGS or diffuse mesangial sclerosis on initial biopsy as well as age, serum albumin concentration, and CKD stage at onset affected ESRD risk. Our findings suggest that responsiveness to initial IIS and detection of a hereditary podocytopathy are prognostic indicators of favorable and poor long-term outcome, respectively, in children with steroid-resistant nephrotic syndrome. Children with multidrug-resistant sporadic disease show better renal survival than those with genetic disease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established.", 
    "doi": "10.1681/ASN.2016101121", 
    "has_grant": false, 
    "journal": {
      "issue": "10", 
      "pages": "3055-3065", 
      "title": "JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY", 
      "volume": "28"
    }, 
    "license": {
      "id": "cc-by"
    }, 
    "publication_date": "2017-01-01", 
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      "title": "Dergi makalesi", 
      "type": "publication"
    }, 
    "title": "Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children"
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