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透析患者的钙化防御,一种对治疗反应不佳的严重疾病:4 例报告。

Calciphylaxis in dialysis patients, a severe disease poorly responding to therapies: report of 4 cases.

机构信息

Unit of Dermatology, AUSL Ravenna, Ravenna, Italy -

出版信息

G Ital Dermatol Venereol. 2013 Oct;148(5):531-6.

Abstract

Calciphylaxis or calciphic uremic arteriolopathy (CUA) is a rare syndrome characterized by the deposition of calcium within the walls of small and medium size vessels in the dermis and in the subcutaneous tissue. The disease mainly affects patients with end-stage renal disease. We report here our experience with 4 cases of calciphylaxis in dialysis patients. The main predisposing factor observed in our 4 patients was warfarin use (2 patients, 50%), while local traumas and diabetes were respectively present in only one patient. None of our patients was obese. Lower legs were the most frequently involved site of CUA (3/4 patients, 75%). In our experience biopsy was crucial to achieve a correct diagnosis and did not cause aggravation of the ulcers. Therapeutic approach was multimodal: mainly hyperbaric oxygen therapy, cinacalcet and sodium thiosulphate. Although many recent case reports have shown exceptional results and healing with the use of sodium thiosulphate, we did not experience any change in the poor prognosis of our patients with the use of this drug, at a dosage of 5 g thrice weekly endovenously.

摘要

钙化防御或钙化尿毒症性小动脉病(CUA)是一种罕见的综合征,其特征是在真皮和皮下组织中小血管和中等大小血管的壁内沉积钙。该疾病主要影响终末期肾病患者。我们在此报告 4 例透析患者的钙化防御经验。在我们的 4 例患者中观察到的主要诱发因素是华法林使用(2 例,50%),而局部创伤和糖尿病分别仅见于 1 例患者。我们的患者均无肥胖。小腿是 CUA 最常受累的部位(3/4 例患者,75%)。根据我们的经验,活检对于明确诊断至关重要,且不会加重溃疡。治疗方法是多模式的:主要是高压氧治疗、西那卡塞和硫代硫酸钠。尽管最近的许多病例报告显示,使用硫代硫酸钠可获得极好的效果并使溃疡愈合,但我们在使用该药物时,并未发现患者预后的任何改善,其剂量为每周 3 次,5 g 静脉内给药。

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