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钙敏感受体病:超越慢性肾脏病-矿物质和骨异常

Calciphylaxis: Beyond CKD-MBD.

作者信息

Fernández María, Morales Enrique, Gutierrez Eduardo, Polanco Natalia, Hernández Eduardo, Mérida Eva, Praga Manuel

机构信息

Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.

Servicio de Nefrología, Hospital Universitario 12 de Octubre, Madrid, España.

出版信息

Nefrologia. 2017 Sep-Oct;37(5):501-507. doi: 10.1016/j.nefro.2017.02.006. Epub 2017 Apr 5.

DOI:10.1016/j.nefro.2017.02.006
PMID:28390776
Abstract

INTRODUCTION

Calcific uraemic arteriolopathy (CUA), also called calciphylaxis, is a rare but potentially fatal vascular disorder that almost exclusively affects patients with chronic renal failure. The objective of this study was to analyse various risk factors for developing CUA and its subsequent clinical course according to the treatment received.

MATERIALS AND METHODS

A retrospective study that included patients diagnosed with CUA from December 1999 to December 2015. Various risk factors, clinical course and treatment options were analysed.

RESULTS

A total of 28 patients (53.6% females) with a mean age of 67.2±11.8 (38-88) years were included. At the time of diagnosis, 53.6% were on haemodialysis, 25% were kidney transplant patients and 21.4% had normal renal function. The use of steroids (100%, P=.001) was the main risk factor in renal transplant patients. Skin lesions resolved in 60.7% (especially in those receiving multitargeted therapy). Patient survival at 12 months was 29% in transplant patients, 57% in haemodialysis patients and 100% in normal renal function patients (log-rank 6.88, P=.032). Chronic renal failure (P=.03) and hypoalbuminaemia (P=.02) were the main risk factor for CUA mortality.

CONCLUSIONS

Although the incidence of CUA remains low, CUA mortality is very high, Special attention to its occurrence in kidney transplant patients and «non-renal» CUA forms is required. Oral anticoagulants and steroids appear to be the main risk factors, CUA is a challenge; a registry of patients and determining standard therapy are required.

摘要

引言

钙化性尿毒症小动脉病(CUA),也称为钙化防御,是一种罕见但可能致命的血管疾病,几乎只影响慢性肾衰竭患者。本研究的目的是根据所接受的治疗分析发生CUA的各种危险因素及其后续临床病程。

材料与方法

一项回顾性研究,纳入了1999年12月至2015年12月期间诊断为CUA的患者。分析了各种危险因素、临床病程和治疗选择。

结果

共纳入28例患者(女性占53.6%),平均年龄67.2±11.8(38 - 88)岁。诊断时,53.6%的患者接受血液透析,25%为肾移植患者,21.4%肾功能正常。使用类固醇(100%,P = .001)是肾移植患者的主要危险因素。60.7%的皮肤病变得到缓解(尤其是接受多靶点治疗的患者)。肾移植患者12个月时的生存率为29%,血液透析患者为57%,肾功能正常患者为100%(对数秩检验6.88,P = .032)。慢性肾衰竭(P = .03)和低白蛋白血症(P = .02)是CUA死亡的主要危险因素。

结论

尽管CUA的发病率仍然较低,但其死亡率非常高,需要特别关注其在肾移植患者和“非肾性”CUA形式中的发生情况。口服抗凝剂和类固醇似乎是主要危险因素,CUA是一项挑战;需要建立患者登记系统并确定标准治疗方法。

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