To describe kidney and patient outcomes for individuals with rare diseases in the UK, Gale and colleagues analysed data from 27,285 patients with 28 different types of rare kidney disease in RaDaR. Over a median follow-up of 9.6 years, RaDaR participants had a significantly higher 5-year cumulative incidence of kidney failure than that of 2.81 million patients with all-cause CKD (28% versus 1%; P < 0.0001), but better survival (standardized mortality ratio 0.42; 95% CI 0.32–0.52; P < 0.0001). Despite substantial variation in measures such as age at kidney failure and death, the researchers found that the trajectory for patients with rare kidney diseases was faster than that of other patients with CKD. “This information might reframe perceptions of CKD, rare diseases and kidney failure among regulators, health-care providers and researchers,” concludes Gale.

为了描述英国罕见疾病患者的肾脏和患者结果，Gale 及其同事分析了 RaDaR 中 27,285 名患有 28 种不同类型罕见肾脏疾病的患者的数据。在中位随访 9.6 年中，RaDaR 参与者的 5 年累积肾衰竭发生率显着高于 281 万全因 CKD 患者（28% 比 1%；P <  0.0001），但生存率更高（标准化死亡率 0.42；95% CI 0.32–0.52；P  < 0.0001）。尽管肾衰竭年龄和死亡年龄等指标存在很大差异，但研究人员发现，罕见肾病患者的发病轨迹比其他 CKD 患者更快。 Gale 总结道：“这些信息可能会重塑监管机构、医疗保健提供者和研究人员对 CKD、罕见疾病和肾衰竭的看法。”