Description and cross-sectional analyses of 25,880 adults and children in the UK National Registry of Rare Kidney Diseases cohort

Introduction The National Registry of Rare Kidney Diseases (RaDaR) collects data from people living with rare kidney diseases across the UK, and is the world’s largest rare kidney disease registry. We present the clinical demographics and renal function of 25,880 prevalent patients and sought evidence of bias in recruitment to RaDaR. Methods RaDaR is linked with the UK Renal Registry (UKRR, with which all UK patients receiving Kidney Replacement Therapy (KRT) are registered). We assessed ethnicity and socioeconomic status in: 1) prevalent RaDaR patients receiving KRT compared with patients with eligible rare disease diagnoses receiving KRT in the UKRR; 2) patients recruited to RaDaR compared with all eligible unrecruited patients at two renal centres; 3) the age-stratified ethnicity distribution of RaDaR patients with Autosomal Dominant Polycystic Kidney Disease was compared to that of the English Census. Results We found evidence of disparities in ethnicity and social deprivation in recruitment to RaDaR, however these were not consistent across comparisons. Compared with either adults recruited to RaDaR or the English population, children recruited to RaDaR were more likely to be of Asian ethnicity (17.3% vs 7.5% , p-value < 0.0001) and live in more socially deprived areas (30.3% vs 17.3% in the most deprived IMD quintile, p-value < 0.0001). Conclusion We observed no evidence of systematic biases in recruitment of patients into RaDaR but the data provide empirical evidence of negative economic and social consequences (across all ethnicities) experienced by families with children affected by rare kidney diseases.