Riegert-Johnson D L, Kaur J S, Pfeifer E A
Department of Internal Medicine, Mayo Clinic, Rochester, Minn 55905, USA.
Mayo Clin Proc. 2001 Jul;76(7):749-52. doi: 10.4065/76.7.749.
Calciphylaxis is a rare disorder of small-vessel calcification and cutaneous infarction associated with chronic renal failure. Rare cases of calciphylaxis not associated with chronic renal failure have been reported with breast cancer, hyperparathyroidism, and alcoholic cirrhosis. To our knowledge, we report the first case of calciphylaxis without chronic renal failure associated with cholangiocarcinoma and the first attempt to treat calciphylaxis with vitamin K. A 56-year-old woman presented with necrotic leg ulceration. She was treated initially with low-molecular-weight heparin, with no effect. A coagulation work-up showed vitamin K deficiency. During vitamin K therapy, the patient had fulminant progression of the calciphylaxis. She died, and an autopsy showed metastatic cholangiocarcinoma. Thrombosis and protein C deficiency have been implicated in the pathophysiology of calciphylaxis. Functional protein C deficiency may be one of several factors contributing to the development of calciphylaxis. Vitamin K therapy was ineffective in our patient and may have been detrimental.
钙过敏症是一种与慢性肾衰竭相关的小血管钙化和皮肤梗死的罕见病症。有报道称，在乳腺癌、甲状旁腺功能亢进和酒精性肝硬化患者中出现了罕见的与慢性肾衰竭无关的钙过敏症病例。据我们所知，我们报告了首例与胆管癌相关的无慢性肾衰竭的钙过敏症病例，以及首次尝试用维生素K治疗钙过敏症的情况。一名56岁女性出现腿部坏死性溃疡。她最初接受低分子量肝素治疗，但无效。凝血检查显示维生素K缺乏。在维生素K治疗期间，患者的钙过敏症迅速进展。她死亡，尸检显示为转移性胆管癌。血栓形成和蛋白C缺乏与钙过敏症的病理生理学有关。功能性蛋白C缺乏可能是导致钙过敏症发生的几个因素之一。维生素K治疗对我们的患者无效，甚至可能有害。
