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Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children Publisher Pubmed



Trautmann A1 ; Schnaidt S2 ; Lipskazietkiewicz BS3 ; Bodria M4, 5 ; Ozaltin F6 ; Emma F7 ; Anarat A8 ; Melk A9 ; Azocar M10 ; Oh J11 ; Saeed B12 ; Gheisari A13 ; Caliskan S14 ; Gellermann J15 Show All Authors
Authors
  1. Trautmann A1
  2. Schnaidt S2
  3. Lipskazietkiewicz BS3
  4. Bodria M4, 5
  5. Ozaltin F6
  6. Emma F7
  7. Anarat A8
  8. Melk A9
  9. Azocar M10
  10. Oh J11
  11. Saeed B12
  12. Gheisari A13
  13. Caliskan S14
  14. Gellermann J15
  15. Higuita LMS16
  16. Jankauskiene A17
  17. Drozdz D18
  18. Mir S19
  19. Balat A20
  20. Szczepanska M21
  21. Paripovic D22
  22. Zurowska A23
  23. Bogdanovic R24
  24. Yilmaz A25
  25. Ranchin B26
  26. Baskin E27
  27. Erdogan O28
  28. Remuzzi G29, 30, 31
  29. Firsztadamczyk A32
  30. Kuzmamroczkowska E33
  31. Litwin M34
  32. Murer L35
  33. Tkaczyk M36
  34. Jardim H37
  35. Wasilewska A38
  36. Printza N39
  37. Fidan K40
  38. Simkova E41
  39. Borzecka H42
  40. Staude H43
  41. Hees K2
  42. Schaefer F1
Show Affiliations
Authors Affiliations
  1. 1. Division of Pediatric Nephrology, Center for Pediatrics and Adolescent Medicine, Im Neuenheimer Feld 430, Heidelberg, 69120, Germany
  2. 2. Institute of Medical Biometry and Informatics, University of Heidelberg, Heidelberg, Germany
  3. 3. Departments of Biology and Genetics, Medical University of Gdansk, Gdansk, Poland
  4. 4. Dipartimento di Medicina Clinica e Sperimentale, University of Studies of Parma, Parma, Italy
  5. 5. Division of Nephrology, Dialysis and Transplantation, IRCCS Giannina Gaslini, Genoa, Italy
  6. 6. Department of Pediatric Nephrology, Center for Biobanking and Genomics, Hacettepe University, Ankara, Turkey
  7. 7. Nephrology and Dialysis Unit, Children'Shospital Bambino Gesu, Istitutio di Ricovero e Cura A Carattere Scientificio (IRCCS), Rome, Italy
  8. 8. Pediatric Nephrology Department, Cukurova University Medical Faculty, Adana, Turkey
  9. 9. Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School, Hannover, Germany
  10. 10. Pediatric Nephrology, Hospital Luis Calvo Mackenna, Facultad de Chile, Santiago, Chile
  11. 11. Department of Pediatric Nephrology, University Children's Hospital, Hamburg, Germany
  12. 12. Department of Pediatric Nephrology, Kidney Hospital of Damascus, Damascus, Syrian Arab Republic
  13. 13. Pediatric Nephrology Department, Isfahan University of Medical Science, St. Al Zahra Hospital, Isfahan, Iran
  14. 14. Pediatric Nephrology Department, Cerrahpasa Medical Faculty, Istanbul University, Istanbul, Turkey
  15. 15. Clinic for Pediatric Nephrology, Charite Hospital, Berlin, Germany
  16. 16. Pediatric Nephrology, Hospital Pablo Tobon Uribe, Medellin Antioquia, Colombia
  17. 17. Pediatric Center, Vilnius University, Vilnius, Lithuania
  18. 18. Department of Pediatric Nephrology, Jagiellonian University Medical College, Krakow, Poland
  19. 19. Department of Pediatric Nephrology, Ege University Medical Faculty, Izmir, Turkey
  20. 20. Department of Pediatric Nephrology, Gaziantep University Medical Faculty, Gaziantep, Turkey
  21. 21. Department of Pediatrics, Division of Dentistry, School of Medicine, Zabrze, Poland
  22. 22. Department of Pediatric Nephrology, University Children's Hospital, Belgrade, Serbia
  23. 23. Departments of Pediatric Nephrology, Medical University of Gdansk, Gdansk, Poland
  24. 24. Department of Pediatric Nephrology, Institute of Mother Child and Healthcare of Serbia, Belgrade, Serbia
  25. 25. Department of Pediatric Nephrology, Istanbul Medical Faculty, Istanbul, Turkey
  26. 26. Pediatric Nephrology Unit, Hopital Femme Mere Enfant, Hospices Civils de Lyon, Lyon, France
  27. 27. Department of Pediatric Nephrology, Baskent University Hospital, Ankara, Turkey
  28. 28. Department of Pediatric Nephrology, Sami Ulus Children's Hospital, Ankara, Turkey
  29. 29. Clinical Research Center for Rare Diseases Aldo and Cele Dacco, IRCCS, Istituto di Ricerche Farmacologiche Mario Negri, Bergamo, Italy
  30. 30. Unit of Nephrology and Dialysis, Azienda Socio Sanitaria Territoriale Papa Giovanni XXIII, Bergamo, Italy
  31. 31. Department of Biomedical and Clinical Science L. Sacco, University of Milan, Milan, Italy
  32. 32. Department of Pediatric Nephrology, Ludwik Rydygier Hospital, Torun, Poland
  33. 33. Department of Pediatrics and Nephrology, Medical University of Warsaw, Warsaw, Poland
  34. 34. Department of Pediatric Nephrology, Centrum Zdrowia Dziecka, Warsaw, Poland
  35. 35. Pediatric Nephrology, Dialysis and Transplant Unit, Department of Women's and Child's Health, Hospital of Padua, Padua, Italy
  36. 36. Pediatric Nephrology Division, Polish Mothers Memorial Hospital Research Institute, Lodz, Poland
  37. 37. Department of Pediatric Nephrology, Centre Hospitalar, Porto, Portugal
  38. 38. Department of Pediatric Nephrology, University Hospital, Bialystok, Poland
  39. 39. First Pediatric Department, Hippokration General Hospital, Aristotle University, Thessaloniki, Greece
  40. 40. Pediatric Nephrology Department, Gazi University Hospital, Ankara, Turkey
  41. 41. Department of Pediatric Nephrology, Dubai Hospital, Dubai, United Arab Emirates
  42. 42. Department of Pediatric Nephrology, Medical University, Lublin, Poland
  43. 43. Department of Pediatric Nephrology, University Children's Hospital, Rostock, Germany

Source: Journal of the American Society of Nephrology Published:2017


Abstract

Weinvestigated the value of genetic, histopathologic, and early treatment response information inprognosing longterm 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>3months and<20 years of age): 612 haddocumentedresponsiveness tointensifiedimmunosuppression (IIS),1155hadkidneybiopsy results, and212had 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 achievedwith 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. Childrenwithmultidrug-resistant sporadic disease showbetter renal survival than thosewith geneticdisease. Furthermore, histopathologic findings may retain prognostic relevance when a genetic diagnosis is established. © 2017 by the American Society of Nephrology.
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