Budisavljevic M N, Cheek D, Ploth D W
Department of Medicine, Medical University of South Carolina, Charleston 29425-2220, USA.
J Am Soc Nephrol. 1996 Jul;7(7):978-82. doi: 10.1681/ASN.V77978.
Calciphylaxis is a rare and life-threatening complication that is estimated to occur in 1% of patients with ESRD each year. Typically, extensive microvascular calcification and occlusion/thrombosis leads to violaceous skin lesions, which progress to nonhealing ulcers and sepsis. Secondary infection of skin lesions is common, often leading to sepsis and death. The lower extremities are predominantly involved (roughly 90% of patients). Patients with skin involvement over the trunk or proximal extremities have a poorer prognosis. Although most calciphylaxis patients have abnormalities of the calcium:phosphate axis or elevated levels of parathyroid hormone, these abnormalities do not appear to be fundamental to the pathophysiology of the disorder, and the etiology of calciphylaxis remains unclear. Recently, functional protein C deficiency has been hypothesized to cause a hypercoagulable state that could induce thrombosis in small vessels, with resulting skin ischemia, necrosis, and gangrene. The lack of understanding of the pathophysiology of the disease results in treatments that are equally unsatisfactory. Patients who undergo parathyroidectomy have a tendency to improve, but the prognosis for the disease is poor and mortality remains high.
钙化防御是一种罕见且危及生命的并发症，据估计每年在1%的终末期肾病患者中发生。通常，广泛的微血管钙化和闭塞/血栓形成会导致皮肤出现紫罗兰色病变，进而发展为不愈合的溃疡和脓毒症。皮肤病变的继发感染很常见，常导致脓毒症和死亡。下肢受累最为常见（约90%的患者）。躯干或近端肢体有皮肤受累的患者预后较差。虽然大多数钙化防御患者存在钙磷轴异常或甲状旁腺激素水平升高，但这些异常似乎并非该疾病病理生理学的根本原因，钙化防御的病因仍不清楚。最近，有人提出功能性蛋白C缺乏会导致高凝状态，进而引发小血管血栓形成，导致皮肤缺血、坏死和坏疽。对该疾病病理生理学的认识不足导致治疗效果同样不尽人意。接受甲状旁腺切除术的患者有改善的趋势，但该病预后较差，死亡率仍然很高。
