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钙化性尿毒症小动脉病导致的皮肤坏死。

Cutaneous necrosis from calcific uremic arteriolopathy.

作者信息

Coates T, Kirkland G S, Dymock R B, Murphy B F, Brealey J K, Mathew T H, Disney A P

机构信息

Department of Medicine, University of Adelaide, The Queen Elizabeth Hospital Campus, Woodville, South Australia, Australia.

出版信息

Am J Kidney Dis. 1998 Sep;32(3):384-91. doi: 10.1053/ajkd.1998.v32.pm9740153.

Abstract

Calcific uremic arteriolopathy (calciphylaxis) is an uncommon complication of chronic renal failure that is associated with high morbidity and mortality. We report 16 patients (13 female) who presented between 1985 and 1996. All patients developed painful livido reticularis that progressed to cutaneous necrosis and ulceration (11 cases on the proximal extremities and five cases on the distal extremities). Two patients with predominately distal leg disease survived; the cause of death in the other 14 patients was sepsis (six patients), withdrawal from dialysis (three), cardiac arrest (three), and gastrointestinal hemorrhage (two). Mesenteric ischemia from intestinal vascular calcification occurred in two cases. Clinical factors identified included the use of warfarin therapy in seven cases and significant weight loss (>10% body weight) in seven cases in the 6 months preceding the development of calcific uremic arteriolopathy. Skin pathology was studied in 12 cases, with all showing calcific panniculitis and small vessel calcification. Electron microscopic spectral analysis of the mineral content of the calcific lesions in the subcutaneous tissue showed only calcium and phosphorous. In two cases, substitution of low molecular weight heparin for warfarin therapy resulted in clinical improvement. Current theories of pathogenesis and treatment are reviewed. This study confirms the high morbidity and mortality of calcific uremic arteriolopathy producing ischemic tissue necrosis while drawing attention to significant weight loss and warfarin therapy as risk factors for the development of ischemic tissue necrosis. Hyperbaric oxygen therapy warrants further study.

摘要

钙化性尿毒症小动脉病(calciphylaxis)是慢性肾衰竭的一种罕见并发症,与高发病率和死亡率相关。我们报告了1985年至1996年间就诊的16例患者(13例女性)。所有患者均出现疼痛性网状青斑,并进展为皮肤坏死和溃疡(11例发生在近端肢体,5例发生在远端肢体)。2例以远端腿部病变为主的患者存活;其他14例患者的死亡原因分别为败血症(6例)、停止透析(3例)、心脏骤停(3例)和胃肠道出血(2例)。2例发生肠道血管钙化导致的肠系膜缺血。确定的临床因素包括7例使用华法林治疗,以及在钙化性尿毒症小动脉病发生前6个月内7例出现显著体重减轻(体重减轻>10%)。对12例患者进行了皮肤病理研究,均显示钙化性脂膜炎和小血管钙化。对皮下组织钙化病变矿物质含量的电子显微镜光谱分析显示仅含钙和磷。2例患者将华法林治疗换为低分子量肝素治疗后临床症状改善。本文对当前的发病机制和治疗理论进行了综述。本研究证实了钙化性尿毒症小动脉病导致缺血性组织坏死的高发病率和死亡率,同时提请注意显著体重减轻和华法林治疗是缺血性组织坏死发生的危险因素。高压氧治疗值得进一步研究。

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