G33(P) Overview of rare renal diseases at a paediatric renal centre through the national registry of rare kidney diseases (radar) in the united kingdom

Abstract

Aims Rare kidney diseases need better understanding and the starting point is collection of clinical data. Therefore our centre is participating in RaDaR which is a UK Renal Association initiative designed to gather information. RaDaR recruitment began in 2010 and now covers more than 40 conditions. There are around 10 000 recruits from 78 renal adult and paediatric units in the UK. Our Paediatric Renal Centre is the leading recruiting hospital in the UK. To describe the range of range of conditions and patient numbers recruited to RADAR at BCH which is a national tertiary renal referral hospital in the UK. Methods The RaDaR dataset is defined by the UK Renal Registry in association with the Rare Disease Groups, made up of experts in each eligible condition. Data fields include demographics, blood and urine results, medications, transplant and dialysis history, genetics and co-morbidities. Data is entered retrospectively from the patient’s medical records following consent. Results 319 patients have been consented at BCH. The age range is from birth to 16 years with mean of 4.9 years with male to female ratio of 55%:45%. The most common condition is Idiopathic Nephrotic Syndrome (n=128; 39%), followed by Alport Syndrome (n=33; 10%), ARPKD (n=25; 8%), Hyperoxaluria (n=24; 7%) and STEC HUS (n=22; 7%). The other conditions with numbers of patients recruited so far include: ADPKD (n=14), aHUS (n=13); Cystinosis (n=9); Cystinuria (n=3); Dent and Lowe (n=7); HNF1b (n=6); Hypokalaemic Alkalosis (n=8); MPGN (n=14) and Vasculitis (n=7). Conclusion RaDaR provides important epidemiology data based on the whole country population which is shared amongst the renal team to develop further research into rare kidney diseases and improve the quality of care for these patients. It also gives an opportunity to define the best treatment practices across the country in the future.