Wong Katie, Pitcher David, Braddon Fiona, Downward Lewis, Steenkamp Retha, Masoud Sherry, Annear Nicholas, Barratt Jonathan, Bingham Coralie, Coward Richard J, Chrysochou Tina, Game David, Griffin Sian, Hall Matt, Johnson Sally, Kanigicherla Durga, Karet Frankl Fiona, Kavanagh David, Kerecuk Larissa, Maher Eamonn R, Moochhala Shabbir, Pinney Jenny, Sayer John A, Simms Roslyn, Sinha Smeeta, Srivastava Shalabh, Tam Frederick W K, Thomas Kay, Turner A Neil, Walsh Stephen B, Waters Aoife, Wilson Patricia, Wong Edwin, Sy Karla Therese L, Huang Kui, Ye Jamie, Nitsch Dorothea, Saleem Moin, Bockenhauer Detlef, Bramham Kate, Gale Daniel P
National Registry of Rare Kidney Diseases, Bristol, UK.
Department of Renal Medicine, University College London, UK.
Kidney Int Rep. 2024 May 9;9(7):2067-2083. doi: 10.1016/j.ekir.2024.04.062. eCollection 2024 Jul.
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.
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 the following: (i) prevalent RaDaR patients receiving KRT compared with patients with eligible rare disease diagnoses receiving KRT in the UKRR, (ii) patients recruited to RaDaR compared with all eligible unrecruited patients at 2 renal centers, and (iii) the age-stratified ethnicity distribution of RaDaR patients with autosomal dominant polycystic kidney disease (ADPKD) was compared to that of the English census.
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%, -value < 0.0001) and live in more socially deprived areas (30.3% vs. 17.3% in the most deprived Index of Multiple Deprivation (IMD) quintile, -value < 0.0001).
We observed no evidence of systematic biases in recruitment of patients into RaDaR; however, the data provide empirical evidence of negative economic and social consequences (across all ethnicities) experienced by families with children affected by rare kidney diseases.
国家罕见肾病登记处（RaDaR）收集了全英国罕见肾病患者的数据，是世界上最大的罕见肾病登记处。我们展示了25880名现患患者的临床人口统计学数据和肾功能，并寻找了招募进入RaDaR登记处存在偏差的证据。
RaDaR与英国肾脏登记处（UKRR，所有接受肾脏替代治疗[KRT]的英国患者均在该登记处注册）相关联。我们在以下方面评估了种族和社会经济地位：（i）将接受KRT的现患RaDaR患者与UKRR中接受KRT且患有符合条件的罕见病诊断的患者进行比较；（ii）将招募进入RaDaR的患者与两个肾脏中心所有符合条件但未被招募的患者进行比较；（iii）将常染色体显性多囊肾病（ADPKD）的RaDaR患者按年龄分层的种族分布与英国人口普查数据进行比较。
我们发现招募进入RaDaR登记处时在种族和社会剥夺方面存在差异的证据；然而，这些差异在不同比较中并不一致。与招募进入RaDaR的成年人或英国人群相比，招募进入RaDaR的儿童更有可能是亚洲种族（17.3%对7.5%，P值<0.0001），且生活在社会剥夺程度更高的地区（在多重剥夺指数[IMD]最贫困五分位数中为30.3%对17.3%，P值<0.0001）。
我们未观察到招募患者进入RaDaR登记处存在系统性偏差的证据；然而，这些数据提供了经验证据，证明受罕见肾病影响的儿童家庭（所有种族）经历了负面的经济和社会后果。
