Evidence map›Paper›PMID 38492578›Full record

ArticleLancet (London, England)2024

Effects of rare kidney diseases on kidney failure: a longitudinal analysis of the UK National Registry of Rare Kidney Diseases (RaDaR) cohort.

Katie Wong, David Pitcher, Fiona Braddon, Lewis Downward, Retha Steenkamp, Nicholas Annear, Jonathan Barratt, Coralie Bingham, Constantina Chrysochou, Richard J Coward and 28 more

Erratum issuedOpen access · hybridAbstract read
PubMed Full text at PMC Publisher
In one paragraph

Article in Lancet (London, England), 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. An erratum has been issued. Cited by 57 papers, 3 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
57citing papers in PubMed, 3 pooled it
21.7field-weighted citation impact, top 1% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

57 citing papers in PubMed, 3 syntheses or guidelines pooled it, 65 citations in OpenAlex.

  1. Pooled it
  2. Diagnosis, management and treatment of the Alport syndrome - 2024 guideline on behalf of ERKNet, ERA and ESPN.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025
    Guideline
  3. Dent disease: clinical practice recommendations.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2025
    Guideline
  4. Article
  5. Review
  6. Review
  7. Article
  8. Association of UACR and UPCR with kidney failure: analysis of observational data in patients with rare kidney diseases.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2026
    Observational
  9. IgA nephropathy management: what does the future hold?Kidney international supplements · 2026
    Review
  10. Review
  11. Review
  12. Observational
  13. Article
  14. Article
  15. Database ofKidney international reports · 2026
    Article
  16. Article
  17. [Primary glomerulonephritides].Innere Medizin (Heidelberg, Germany) · 2026
    Article
  18. From data to drug: the translational impact of RaDaR, the UK national registry of rare kidney diseases.Nephrology, dialysis, transplantation : official publication of the European Dialysis and Transplant Association - European Renal Association · 2026
    Review
  19. Review
  20. Article
4 · The record

Corrections and comments

5 · Who and what money

Authors and funding

38 authors at 20 institutions in 2 countries.

Katie WongNational Registry of Rare Kidney Diseases, Bristol, UK; Department of Renal Medicine, University College London, London, UK.
David PitcherNational Registry of Rare Kidney Diseases, Bristol, UK.
Fiona BraddonNational Registry of Rare Kidney Diseases, Bristol, UK.
Lewis DownwardNational Registry of Rare Kidney Diseases, Bristol, UK.
Retha SteenkampUK Renal Registry, Bristol, UK.
Nicholas AnnearInstitute of Medical and Biomedical Education, St George's University of London, London, UK.
Jonathan BarrattDepartment of Cardiovascular Sciences, University of Leicester, Leicester, UK.
Coralie BinghamUniversity of Exeter Medical School, University of Exeter, Exeter, UK.
Constantina ChrysochouDivision of Cardiovascular Sciences, University of Manchester, Manchester, UK.
Richard J CowardTranslational Health Sciences, University of Bristol, Bristol, UK.
David GameGuy's and St Thomas' NHS Foundation Trust, London, UK.
Sian GriffinDepartment of Nephrology, University Hospital Wales, Cardiff, UK.
Matt HallNottingham Renal and Transplant Unit, Nottingham University Hospitals NHS Foundation Trust, Nottingham, UK.
Sally JohnsonGreat North Children's Hospital, Newcastle upon Tyne, UK.
Durga KanigicherlaDivision of Cardiovascular Sciences, University of Manchester, Manchester, UK.
Fiona Karet FranklCambridge Institute for Medical Research, University of Cambridge, Cambridge, UK.
David KavanaghNational Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK; Complement Therapeutics Research Group, Newcastle University, Newcastle upon Tyne, UK; Translational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
Larissa KerecukBirmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
Eamonn R MaherDepartment of Medical Genetics, University of Cambridge, Cambridge, UK.
Shabbir MoochhalaDepartment of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK.
Jenny PinneyDepartment of Renal Medicine, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK.
John A SayerTranslational and Clinical Research Institute, Newcastle University, Newcastle upon Tyne, UK.
Roslyn SimmsAcademic Unit of Nephrology, Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, UK.
Smeeta SinhaDivision of Cardiovascular Sciences, University of Manchester, Manchester, UK; Department of Renal Medicine, Northern Care Alliance NHS Foundation Trust, Manchester, UK.
Shalabh SrivastavaDepartment of Renal Medicine, South Tyneside and Sunderland NHS Foundation Trust, Sunderland, UK.
Frederick W K TamCentre for Inflammatory Disease, Department of Immunology and Inflammation, Imperial College London, London, UK.
Andrew Neil TurnerMedical Research Council Centre for Inflammation, Edinburgh University, Edinburgh, UK.
Stephen B WalshDepartment of Renal Medicine, University College London, London, UK; Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK.
Aoife WatersDepartment of Paediatrics and Child Health, University College Cork, Cork, Ireland.
Patricia WilsonDepartment of Renal Medicine, University College London, London, UK.
Edwin WongNational Renal Complement Therapeutics Centre, Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK.
Christopher Mark TaylorBirmingham Women's and Children's NHS Foundation Trust, Birmingham, UK.
Dorothea NitschUK Renal Registry, Bristol, UK; London School of Hygiene and Tropical Medicine, London, UK.
Moin SaleemTranslational Health Sciences, University of Bristol, Bristol, UK.
Detlef BockenhauerDepartment of Renal Medicine, University College London, London, UK; Great Ormond Street Hospital for Children NHS Foundation Trust, London, UK.
Kate BramhamNational Registry of Rare Kidney Diseases, Bristol, UK; King's Health Partners, King's College London, London, UK.
Daniel P GaleNational Registry of Rare Kidney Diseases, Bristol, UK; Department of Renal Medicine, University College London, London, UK; Department of Renal Medicine, Royal Free London NHS Foundation Trust, London, UK. Electronic address: d.gale@ucl.ac.uk.
RaDaR consortium
Royal Free London NHS Foundation Trust · GBNewcastle upon Tyne Hospitals NHS Foundation Trust · GBUniversity of Manchester · GBBirmingham Women’s and Children’s NHS Foundation Trust · GBUniversity of Cambridge · GBUniversity of Bristol · GBCentre for Inflammation Research · GBKing's College London · GBUniversity Hospitals Birmingham NHS Foundation Trust · GBGuy's and St Thomas' NHS Foundation Trust · GBRenal Association · GBSt George's, University of London · GBUniversity College Cork · IEUniversity College London · GBUniversity of Leicester · GBGreat North Children's Hospital · GBNewcastle University · GBNottingham University Hospitals NHS Trust · GBUniversity of Exeter · GBKidney Care UK · GB

Funding

Medical Research Council
6 · The paper itself

Abstract

backgroundIndividuals with rare kidney diseases account for 5-10% of people with chronic kidney disease, but constitute more than 25% of patients receiving kidney replacement therapy. The National Registry of Rare Kidney Diseases (RaDaR) gathers longitudinal data from patients with these conditions, which we used to study disease progression and outcomes of death and kidney failure.

methodsPeople aged 0-96 years living with 28 types of rare kidney diseases were recruited from 108 UK renal care facilities. The primary outcomes were cumulative incidence of mortality and kidney failure in individuals with rare kidney diseases, which were calculated and compared with that of unselected patients with chronic kidney disease. Cumulative incidence and Kaplan-Meier survival estimates were calculated for the following outcomes: median age at kidney failure; median age at death; time from start of dialysis to death; and time from diagnosis to estimated glomerular filtration rate (eGFR) thresholds, allowing calculation of time from last eGFR of 75 mL/min per 1·73 m

findingsBetween Jan 18, 2010, and July 25, 2022, 27 285 participants were recruited to RaDaR. Median follow-up time from diagnosis was 9·6 years (IQR 5·9-16·7). RaDaR participants had significantly higher 5-year cumulative incidence of kidney failure than 2·81 million UK patients with all-cause chronic kidney disease (28% vs 1%; p<0·0001), but better survival rates (standardised mortality ratio 0·42 [95% CI 0·32-0·52]; p<0·0001). Median age at kidney failure, median age at death, time from start of dialysis to death, time from diagnosis to eGFR thresholds, and therapeutic trial window all varied substantially between rare diseases.

interpretationPatients with rare kidney diseases differ from the general population of individuals with chronic kidney disease: they have higher 5-year rates of kidney failure but higher survival than other patients with chronic kidney disease stages 3-5, and so are over-represented in the cohort of patients requiring kidney replacement therapy. Addressing unmet therapeutic need for patients with rare kidney diseases could have a large beneficial effect on long-term kidney replacement therapy demand.

fundingRaDaR is funded by the Medical Research Council, Kidney Research UK, Kidney Care UK, and the Polycystic Kidney Disease Charity.

Indexed as

Kidney Failure, ChronicRenal InsufficiencyRenal Insufficiency, ChronicAdolescentAdultAgedAged, 80 and overChildChild, PreschoolGlomerular Filtration RateHumansInfantInfant, NewbornKidneyMiddle AgedRadar

Identifiers

PMID38492578
PMCPMC11750427
OpenAlexW4392743128

What OpenQuestion holds

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LicenceCC BY
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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.