Katie Wong is a clinical research fellow at the University College London Department of Renal Medicine (London, UK). She details some of the research on rare kidney diseases that will be outlined at UK Kidney Week (UKKW; 5–7 June, Newport, UK).
Our team behind the UK National Registry of Rare Kidney Disease (RaDaR) are presenting the results of several research projects at UKKW. I will set out some of what they will discuss, what is possible with RaDaR data, and what RaDaR might be capable of in the future.
A rare disease is defined in Europe as a condition affecting less than one in 2,000 people. As rare kidney diseases only impact a small number of people, it can be difficult to find reliable information on them. RaDaR was formed to help provide detailed information on rare kidney conditions for patients and clinicians. It collects information about people living with 30 rare kidney diseases and is now in its thirteenth year. RaDaR’s oldest recruiting site is Bristol Royal Hospital for Children (Bristol, UK), which started recruiting in 2010, and it now enrols patients from 108 renal units across all four nations of the UK. To date, RaDaR has recruited over 30,000 patients. It is the largest rare kidney disease registry in the world.
Improving our data
We are constantly working to make sure the data we collect is as in-depth and as up-to-date as possible. We have added 362,503 kidney function results to the RaDaR database in the last six months using older data supplied by renal units. We now have a total of over 2.1 million kidney function readings stretching back over 40 years to the 1980’s for some participants.
We have also received nearly 350 genetic reports for RaDaR patients with Alport Syndrome from Guy’s and St Thomas’ Genetic Laboratory (London, UK). The analysis team at RaDaR have been able to use these to identify the exact genetic change for each of these patients. These genetic results will help us understand how different types of genetic changes might affect how severe each person’s kidney disease may become. We are in the process of obtaining more reports from genetics laboratories at Great Ormond Street Hospital (London, UK) and Bristol.
How we can use RaDaR
We will be presenting the results from several research projects using enriched RaDaR data at UKKW:
- Centre-level variation in tolvaptan prescribing for autosomal dominant polycystic kidney disease (ADPKD) in the UK: This study looked at whether there were any differences in how tolvaptan, a medication for ADPKD, was prescribed in different renal units across the UK.
- The RaDaR: cross-sectional analyses of 25,817 adults and children with rare kidney diseases in the UK: Here we describe important clinical and social characteristics of more than 25,000 patients living with rare kidney diseases in the UK. We identify differences in ethnicity and social deprivation between rare diseases, and between adults and children recruited to RaDaR.
- The clinical demographics and natural history of rare kidney diseases in the UK: A longitudinal descriptive analysis using data from the RaDaR: In this study we used large-scale patient data from RaDaR to describe patterns and risks of kidney function decline, end-stage kidney disease (ESKD) and death for 20 rare kidney diseases.
- Proteinuria and its association with disease progression in IgA nephropathy: analysis of the UK national RaDaR IgA nephropathy cohort: This study, recently published in the Clinical Journal of the American Society of Nephrology (CJASN), used RaDaR data to describe relationships between proteinuria, rates of kidney function decline and lifetime risks of kidney failure for individuals with IgA nephropathy.