伴有中层钙化和内膜增生的尿毒症性小动脉疾病（所谓的钙化防御）：慢性肾衰竭的一种并发症及甲状旁腺切除术后的获益情况。

Uremic small-artery disease with medial calcification and intimal hyperplasia (so-called calciphylaxis): a complication of chronic renal failure and benefit from parathyroidectomy.

作者信息

Hafner J, Keusch G, Wahl C, Sauter B, Hürlimann A, von Weizsäcker F, Krayenbühl M, Biedermann K, Brunner U, Helfenstein U

机构信息

Department of Dermatology, University Hospital of Zurich, Switzerland.

出版信息

J Am Acad Dermatol. 1995 Dec;33(6):954-62. doi: 10.1016/0190-9622(95)90286-4.

DOI:10.1016/0190-9622(95)90286-4

PMID:7490365

Abstract

BACKGROUND

Uremic small-artery disease with medial calcification and intimal hyperplasia can lead to life-threatening skin necrosis or acral gangrene. It is a distinct complication of chronic renal failure that must be differentiated from soft-tissue calcification. An increased calcium-phosphate product and secondary hyperparathyroidism are the main underlying conditions. The benefit of parathyroidectomy is controversial.

OBJECTIVE

This article is based on a literature search to determine prognostic factors and, in particular, the benefit of parathyroidectomy.

METHODS

The literature on uremic small-artery disease (so-called calciphylaxis) was reviewed (full data set: 104 cases, including five of our own). The therapeutic benefit of parathyroidectomy and the relation between prognostic predictors (localization, dialysis, and transplant) and outcome were analyzed. The relation between diabetes and acral gangrene was also examined. Further epidemiologic data on the reviewed group of patients were established.

RESULTS

Thirty-eight of 58 patients who underwent parathyroidectomy survived compared with 13 of 37 patients who did not undergo parathyroidectomy (p = 0.007, n = 95). Forty of 53 patients with distal localization of necrosis survived compared with 11 of 42 patients with proximal pattern (p < 0.00001; n = 95). Dialysis and kidney transplantation followed by immunosuppression showed no relation to disease outcome. No association was found between diabetes and acral gangrene (p = 0.50).

CONCLUSION

Uremic small-artery disease is a distinct complication of chronic renal failure. Its recognition and early diagnosis should allow more effective treatment. In our retrospective study parathyroidectomy was significantly related to survival. Only a randomized, controlled, prospective trial (parathyroidectomy vs conservative treatment of secondary hyperparathyroidism) can establish the value of parathyroidectomy in uremic small-artery disease.

摘要

背景

伴有中层钙化和内膜增生的尿毒症性小动脉疾病可导致危及生命的皮肤坏死或肢端坏疽。它是慢性肾衰竭的一种独特并发症，必须与软组织钙化相鉴别。钙磷乘积升高和继发性甲状旁腺功能亢进是主要的潜在病因。甲状旁腺切除术的益处存在争议。

目的

本文基于文献检索以确定预后因素，尤其是甲状旁腺切除术的益处。

方法

对有关尿毒症性小动脉疾病（所谓的钙化防御）的文献进行了综述（完整数据集：104例，包括我们自己的5例）。分析了甲状旁腺切除术的治疗益处以及预后预测因素（部位、透析和移植）与结局之间的关系。还研究了糖尿病与肢端坏疽之间的关系。建立了所综述患者组的进一步流行病学数据。

结果

接受甲状旁腺切除术的58例患者中有38例存活，而未接受甲状旁腺切除术的37例患者中有13例存活（p = 0.007，n = 95）。坏死位于远端的53例患者中有40例存活，而坏死位于近端的42例患者中有11例存活（p < 0.00001；n = 95）。透析和肾移植后免疫抑制与疾病结局无关。未发现糖尿病与肢端坏疽之间存在关联（p = 0.50）。