The European Rare Kidney Disease Registry (ERKReg): objectives, design and initial results

  1. Bassanese, Giulia
  2. Wlodkowski, Tanja ORCID logo
  3. Servais, Aude
  4. Heidet, Laurence
  5. Roccatello, Dario
  6. Emma, Francesco
  7. Levtchenko, Elena
  8. Ariceta, Gema
  9. Bacchetta, Justine
  10. Capasso, Giovambattista
  11. Jankauskiene, Augustina
  12. Miglinas, Marius
  13. Ferraro, Pietro Manuel
  14. Montini, Giovanni
  15. Oh, Jun
  16. Decramer, Stephane
  17. Levart, Tanja Kersnik
  18. Wetzels, Jack
  19. Cornelissen, Elisabeth
  20. Devuyst, Olivier
  21. Zurowska, Aleksandra
  22. Pape, Lars
  23. Buescher, Anja
  24. Haffner, Dieter
  25. Marcun Varda, Natasa
  26. Ghiggeri, Gian Marco
  27. Remuzzi, Giuseppe
  28. Konrad, Martin
  29. Longo, Germana
  30. Bockenhauer, Detlef
  31. Awan, Atif
  32. Andersone, Ilze
  33. Groothoff, Jaap W.
  34. Schaefer, Franz

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1000 Titel
  • The European Rare Kidney Disease Registry (ERKReg): objectives, design and initial results
1000 Autor/in
  1. Bassanese, Giulia |
  2. Wlodkowski, Tanja |
  3. Servais, Aude |
  4. Heidet, Laurence |
  5. Roccatello, Dario |
  6. Emma, Francesco |
  7. Levtchenko, Elena |
  8. Ariceta, Gema |
  9. Bacchetta, Justine |
  10. Capasso, Giovambattista |
  11. Jankauskiene, Augustina |
  12. Miglinas, Marius |
  13. Ferraro, Pietro Manuel |
  14. Montini, Giovanni |
  15. Oh, Jun |
  16. Decramer, Stephane |
  17. Levart, Tanja Kersnik |
  18. Wetzels, Jack |
  19. Cornelissen, Elisabeth |
  20. Devuyst, Olivier |
  21. Zurowska, Aleksandra |
  22. Pape, Lars |
  23. Buescher, Anja |
  24. Haffner, Dieter |
  25. Marcun Varda, Natasa |
  26. Ghiggeri, Gian Marco |
  27. Remuzzi, Giuseppe |
  28. Konrad, Martin |
  29. Longo, Germana |
  30. Bockenhauer, Detlef |
  31. Awan, Atif |
  32. Andersone, Ilze |
  33. Groothoff, Jaap W. |
  34. Schaefer, Franz |
1000 Erscheinungsjahr 2021
1000 Publikationstyp
  1. Artikel |
1000 Online veröffentlicht
  • 2021-06-02
1000 Erschienen in
1000 Quellenangabe
  • 16(1):251
1000 Copyrightjahr
  • 2021
1000 Lizenz
1000 Verlagsversion
  • https://doi.org/10.1186/s13023-021-01872-8 |
  • https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8173879/ |
1000 Publikationsstatus
1000 Sprache der Publikation
1000 Abstract/Summary
  • Background!#!The European Rare Kidney Disease Reference Network (ERKNet) recently established ERKReg, a Web-based registry for all patients with rare kidney diseases. The main objectives of this core registry are to generate epidemiological information, identify current patient cohort for clinical research, explore diagnostic and therapeutic management practices, and monitor treatment performance and patient's outcomes. The registry has a modular design that allows to integrate comprehensive disease-specific registries as extensions to the core database. The diagnosis (Orphacode) and diagnostic information (clinical, imaging, histopathological, biochemical, immunological and genetic) are recorded. Anthropometric, kidney function, and disease-specific management and outcome items informing a set of 61 key performance indicators (KPIs) are obtained annually. Data quality is ensured by automated plausibility checks upon data entry and regular offline database checks prompting queries. Centre KPI statistics and benchmarking are calculated automatically.!##!Results!#!Within the first 24 months since its launch, 7607 patients were enrolled to the registry at 45 pediatric and 12 specialized adult nephrology units from 21 countries. A kidney disease diagnosis had been established in 97.1% of these patients at time of enrolment. While 199 individual disease entities were reported by Orphacode, 50% of the cohort could be classified with 11, 80% with 43 and 95% with 92 codes. Two kidney diagnoses were assigned in 6.5% of patients; 5.9% suffered from syndromic disease. Whereas glomerulopathies (54.8%) and ciliopathies including autosomal dominant polycystic kidney disease (ADPKD) (31.5%) were the predominant disease groups among adults, the pediatric disease spectrum encompassed congenital anomalies of the kidney and urinary tract (CAKUT) (33.7%), glomerulopathies (30.7%), ciliopathies (14.0%), tubulopathies (9.2%), thrombotic microangiopathies (5.6%), and metabolic nephropathies (4.1%). Genetically confirmed diagnoses were reported in 24% of all pediatric and 12% adult patients, whereas glomerulopathies had been confirmed by kidney biopsy in 80.4% adult versus 38.5% pediatric glomerulopathy cases.!##!Conclusions!#!ERKReg is a rapidly growing source of epidemiological information and patient cohorts for clinical research, and an innovative tool to monitor management quality and patient outcomes.
1000 Sacherschließung
lokal European Rare Kidney Disease Reference Network (ERKNet)
lokal Rare Diseases/epidemiology [MeSH]
lokal Adult [MeSH]
lokal Humans [MeSH]
lokal Kidney Diseases/epidemiology [MeSH]
lokal Kidney [MeSH]
lokal Kidney Diseases/diagnosis [MeSH]
lokal Cohort Studies [MeSH]
lokal Pediatric nephrology
lokal Epidemiology
lokal Rare kidney diseases
lokal Research
lokal Child [MeSH]
lokal Nephrology
lokal Registry
lokal Registries [MeSH]
1000 Liste der Beteiligten
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