【摘要】 钙性尿毒症性小动脉病(calcific uremic arteriolopathy,CUA)又称钙化防御,是一种少见但具有高病死率的临床综合征。CUA目前缺乏明确的实验室诊断标准,皮肤活检有中小动脉中膜钙化及微血栓形成,放射性检查、骨扫描及骨形态蛋白信号通路激活的证据均能早期提示该病。CUA常通过静脉注射硫代硫酸钠、双磷酸盐,高压氧疗及其他对症支持治疗来进行伤口、疼痛、营养、透析等方面的综合管理。CUA的早期防控主要通过合理控制慢性肾病-矿物质与骨异常,减少透析并发症/合并症,慎用某些药物(如华法林、活性维生素D等)来实现。CUA患者预后差,病死率高,部分患者病情进展迅速,早期诊断并制订有效的多学科协作治疗方案极为重要。更多还原

【Abstract】 Calcific uremic arteriopathy(CUA), termed calciphylaxis, is a rare but highly fatal clinical syndrome. There is no clearly laboratory diagnostic criteria for CUA. The medium and small arterial calcification and microthrombosis discovered by skin biopsy, radiologic imaging,bone scan and the evidence of activation of the bone morphogenetic protein signal(BMPs) transduction pathway are useful for early diagnosis of this disease. Th e common therapies(including intravenous sodium thiosulfate(STS) and bisphosphonates, hyperbaric oxygen therapy and other symptomatic supports) are used for the management of wounds, pain, nutrition, dialysis and so on. Controlling the chronic kidney disease-mineral and bone disorder(CKD-MBD) and some complications of dialysis and drugs(such as warfarin, active vitamin D) can prevent CUA. However, CUA patients still have poor prognosis and high mortality. Since some patients progress rapidly, it is of great importance to make early diagnosis and provide effective treatments with multidisciplinary management.更多还原