关键词: calciphylaxis dialysis kidney failure kidney replacement therapy mineral metabolism registry

来  源:   DOI:10.1016/j.ekir.2024.01.026   PDF(Pubmed)

Abstract:
UNASSIGNED: Calciphylaxis is a rare disorder associated with significant morbidity and mortality. Data registries are an invaluable source of information for rare diseases. We reviewed cases of calciphylaxis recorded in the Australia and New Zealand Dialysis and Transplant Registry (ANZDATA) and evaluated associations and outcomes of this condition.
UNASSIGNED: Data was obtained on all cases of calciphylaxis reported between 2019 and 2022 in Australian and New Zealand patients on kidney replacement therapy (KRT). This cohort was compared to all patients in the registry who received KRT from 2019 to 2022 without an episode of calciphylaxis. Cox proportional hazards regression including a time-varying covariate for calciphylaxis episode was conducted for mortality with models restricted to patients on dialysis only.
UNASSIGNED: From 2019 to 2022, 333 patients had calciphylaxis episodes reported. Overall incidence rate for patients on dialysis was 4.5 (4.1-5.1) episodes per 1000 patient-years on dialysis. Median age was 63 (interquartile range [IQR]: 55-73) years, 54% were female, 66% had diabetes, 59% were obese (body mass index [BMI] ≥ 30 kg/m2) and 77% were receiving hemodialysis (HD) treatment. Compared to patients without calciphylaxis (n = 46,526), patients with calciphylaxis were more likely to be older, female, and have diabetes, greater BMI, coronary artery, and peripheral vascular disease. The median time to calciphylaxis was 3.2 (IQR: 0.9-6.7) years after KRT commencement. Half of the patients with calciphylaxis died by 12 months from diagnosis. Adjusted hazard ratio (HR) of mortality for patients on dialysis with calciphylaxis <1 year and 1 to 4 years after an episode was 5.8 (4.9-6.9) and 1.5 (1.0-2.1), respectively compared to patients on dialysis without calciphylaxis.
UNASSIGNED: Calciphylaxis is a rare but life-threatening condition in people on KRT with the greatest mortality burden within 12 months of diagnosis.
摘要:
钙敏感症是一种罕见的与显著的发病率和死亡率相关的疾病。数据登记处是罕见疾病的宝贵信息来源。我们回顾了澳大利亚和新西兰透析和移植登记处(ANZDATA)中记录的钙化预防病例,并评估了这种情况的关联和结果。
获得了2019年至2022年期间澳大利亚和新西兰接受肾脏替代疗法(KRT)的所有钙化病例的数据。将该队列与2019年至2022年接受KRT但无钙化发作的所有患者进行比较。Cox比例风险回归包括钙化发作的时变协变量,用于死亡率,模型仅限于透析患者。
从2019年到2022年,有333例患者出现钙化事件。透析患者的总发生率为每1000例透析患者年4.5(4.1-5.1)次。中位年龄为63岁(四分位数范围[IQR]:55-73岁),54%是女性,66%有糖尿病,59%的人肥胖(体重指数[BMI]≥30kg/m2),77%的人正在接受血液透析(HD)治疗。与没有钙化的患者相比(n=46,526),有钙化倾向的患者更有可能年龄较大,女性,患有糖尿病,更大的BMI,冠状动脉,和外周血管疾病。KRT开始后,钙化的中位时间为3.2年(IQR:0.9-6.7)。一半的钙化患者在诊断后12个月死亡。钙离子透析<1年和发作后1至4年的患者死亡率的调整后风险比(HR)为5.8(4.9-6.9)和1.5(1.0-2.1),分别与没有钙化的透析患者进行比较。
钙化激素病是一种罕见但危及生命的疾病,在KRT患者诊断后12个月内死亡负担最大。
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