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钙过敏症:两名慢性肾病罕见并发症患者的成功治疗

Calciphylaxis: Successful Management of a Rare Complication of Chronic Kidney Disease in Two Patients.

作者信息

Fares Ibtissam, Bouattar Tarik, Kone Hadja Moussokoro, Benzouina Hajar, Benbella Meryem, Ouzeddoun Naima, Bayahia Rabia, Benamar Loubna

机构信息

Nephrology Dialysis and Transplantation Department, Ibn Sina Teaching Hospital, Rabat, Morocco.

Faculty of Medicine and Pharmacy of Rabat, Morocco.

出版信息

Case Rep Nephrol. 2019 Jun 17;2019:1630613. doi: 10.1155/2019/1630613. eCollection 2019.

Abstract

Calciphylaxis, or calcification uremic arteriolopathy, is a rare disease thought to occur due to arteriolar calcifications of the dermis and is responsible for ischemia with cutaneous necrosis and painful panniculitis. Its mechanism remains poorly understood which makes its management challenging and difficult to standardize. We report our management of two patients diagnosed with calciphylaxis. In one patient, calciphylaxis was mentioned upon admission given the context of preexisting secondary hyperparathyroidism and the existence of multiple risk factors. In both patients, the diagnosis was confirmed histologically. Our two observations highlight the difficulty of the diagnosis and the complexity of the therapeutic management that has been personalized according to patient characteristics and clinical evolution. Several therapeutic means can be implemented once the diagnosis is made; nevertheless, its prognosis remains pejorative despite the therapeutic advances. Broad debridement, good phosphocalcic balance control, and the correction of the risk factors top the list of any therapeutic strategy. One of the major challenges of the therapy is normalizing the calcium-phosphate balance. Thus, Cinacalcet and sodium thiosulfate appear to be promising treatments.

摘要

钙过敏症,即钙化性尿毒症性小动脉病,是一种罕见疾病,被认为是由于真皮小动脉钙化所致,可导致缺血伴皮肤坏死和疼痛性脂膜炎。其发病机制仍知之甚少,这使得其治疗具有挑战性且难以标准化。我们报告了我们对两名被诊断为钙过敏症患者的治疗情况。在一名患者中,鉴于既往存在继发性甲状旁腺功能亢进以及多种危险因素,入院时提及了钙过敏症。在两名患者中,均通过组织学确诊。我们的两例观察结果凸显了诊断的困难以及根据患者特征和临床进展进行个性化治疗管理的复杂性。一旦确诊,可采用多种治疗手段;然而,尽管治疗取得了进展,其预后仍然不佳。广泛清创、良好控制磷钙平衡以及纠正危险因素是任何治疗策略的首要任务。治疗的主要挑战之一是使钙磷平衡正常化。因此,西那卡塞和硫代硫酸钠似乎是有前景的治疗方法。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/7f17/6601500/1e89f110de9d/CRIN2019-1630613.001.jpg

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