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- Agnes Trautmann
- Division of Pediatric Nephrology, University Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany;
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- Sven Schnaidt
- Institute of Medical Biometry and Informatics, University of Heidelberg, Germany;
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- Beata S. Lipska-Ziętkiewicz
- Biology and Genetics and
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- Monica Bodria
- Dipartimento di Medicina Clinica e Sperimentale, University of Studies of Parma, Parma, Italy;
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- Fatih Ozaltin
- Department of Pediatric Nephrology, Nephrogenetics Laboratory and Center for Biobanking and Genomics, Hacettepe University, Ankara, Turkey;
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- Francesco Emma
- Nephrology and Dialysis Unit, Children’s Hospital Bambino Gesù, Istitutio di Ricovero e Cura a Carattere Scientificio (IRCCS), Rome, Italy;
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- Ali Anarat
- Pediatric Nephrology Department, Cukurova University Medical Faculty, Adana, Turkey;
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- Anette Melk
- Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany;
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- Marta Azocar
- Pediatric Nephrology, Hospital Luis Calvo Mackenna-Facultad de Chile, Santiago, Chile;
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- Jun Oh
- Department of Pediatric Nephrology, University Children’s Hospital, Hamburg, Germany;
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- Bassam Saeed
- Department of Pediatric Nephrology, Kidney Hospital of Damascus, Damascus, Syria;
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- Alaleh Gheisari
- Pediatric Nephrology Department, Isfahan University of Medical Science, St. Al Zahra Hospital, Isfahan, Iran;
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- Salim Caliskan
- Pediatric Nephrology Department, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey;
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- Jutta Gellermann
- Clinic for Pediatric Nephrology, Charite Hospital, Berlin, Germany;
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- Lina Maria Serna Higuita
- Pediatric Nephrology, Hospital Pablo Tobon Uribe, Medellin Antioquia, Columbia;
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- Augustina Jankauskiene
- Pediatric Center, Vilnius University, Vilnius, Lithuania;
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- Dorota Drozdz
- Department of Pediatric Nephrology, Jagiellonian University Medical College, Krakow, Poland;
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- Sevgi Mir
- Department of Pediatric Nephrology, Ege University Medical Faculty, Izmir, Turkey;
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- Ayse Balat
- Department of Pediatric Nephrology, Gaziantep University Medical Faculty, Gaziantep, Turkey;
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- Maria Szczepanska
- Department of Pediatrics, Division of Dentistry, School of Medicine, Zabrze, Poland;
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- Dusan Paripovic
- Department of Pediatric Nephrology, University Children’s Hospital, Belgrade, Serbia;
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- Alexandra Zurowska
- Pediatric Nephrology, Medical University of Gdansk, Gdansk, Poland;
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- Radovan Bogdanovic
- Department of Pediatric Nephrology, Institute of Mother Child and Healthcare of Serbia, Belgrade, Serbia;
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- Alev Yilmaz
- Department of Pediatric Nephrology, Istanbul Medical Faculty, Istanbul, Turkey;
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- Bruno Ranchin
- Pediatric Nephrology Unit, Hôpital Femme Mere Enfant, Hospices Civils de Lyon, Lyon, France;
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- Esra Baskin
- Department of Pediatric Nephrology, Baskent University Hospital, Ankara, Turkey;
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- Ozlem Erdogan
- Department of Pediatric Nephrology, Sami Ulus Children’s Hospital, Ankara, Turkey;
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- Giuseppe Remuzzi
- Clinical Research Center for Rare Diseases Aldo & Cele Daccò, IRCCS, Istituto di Ricerche Farmacologiche Mario Negri, Bergamo, Italy;
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- Agnieszka Firszt-Adamczyk
- Department of Pediatric Nephrology, Ludwik Rydygier Hospital, Torun, Poland;
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- Elzbieta Kuzma-Mroczkowska
- Department of Pediatrics and Nephrology, Medical University of Warsaw, Warsaw, Poland;
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- Mieczyslaw Litwin
- Department of Pediatric Nephrology, Centrum Zdrowia Dziecka, Warsaw, Poland;
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- Luisa Murer
- Pediatric Nephrology, Dialysis and Transplant Unit, Department of Women’s and Child’s Health, Hospital of Padua, Padua, Italy;
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- Marcin Tkaczyk
- Pediatric Nephrology Division, Polish Mothers Memorial Hospital Research Institute, Lodz, Poland;
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- Helena Jardim
- Department of Pediatric Nephrology, Centre Hospitalar, Porto, Portugal;
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- Anna Wasilewska
- Department of Pediatric Nephrology, University Hospital, Bialystok, Poland;
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- Nikoleta Printza
- First Pediatric Department, Hippokration General Hospital, Aristotle University, Thessaloniki, Greece;
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- Kibriya Fidan
- Pediatric Nephrology Department, Gazi University Hospital, Ankara, Turkey;
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- Eva Simkova
- Department of Pediatric Nephrology, Dubai Hospital, Dubai, United Arab Emirates;
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- Halina Borzecka
- Department of Pediatric Nephrology, Medical University, Lublin, Poland; and
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- Hagen Staude
- Department of Pediatric Nephrology, University Children’s Hospital, Rostock, Germany
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- Katharina Hees
- Institute of Medical Biometry and Informatics, University of Heidelberg, Germany;
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- Franz Schaefer
- Division of Pediatric Nephrology, University Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany;
抄録
<jats:p>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.</jats:p>
収録刊行物
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- Journal of the American Society of Nephrology
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Journal of the American Society of Nephrology 28 (10), 3055-3065, 2017-05-31
Ovid Technologies (Wolters Kluwer Health)