Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM), Hospital do Rim, São Paulo, SP, Brasil.
Universidade Federal de São Paulo (UNIFESP), Escola Paulista de Medicina (EPM), Departamento de Medicina, São Paulo, SP, Brasil.
J Bras Nefrol. 2021 Apr-Jun;43(2):274-278. doi: 10.1590/2175-8239-JBN-2020-0025.
The clinical impact of vascular calcification is well established in the context of cardiovascular morbidity and mortality, but other clinical syndromes, such as calciphylaxis, although less frequent, have a significant impact on chronic kidney disease.
Case report of a 27-year-old woman, who had complained of bilateral pain in her toes for 3 days, with the presence of small necrotic areas in the referred sites. She had a history of type 1 diabetes (25 years ago), with chronic kidney disease, on peritoneal dialysis, in addition to rheumatoid arthritis. She was admitted to the hospital, which preceded the current condition, due to exacerbation of rheumatoid arthritis, evolving with intracardiac thrombus due to venous catheter complications, when she started using warfarin. Ischemia progressed to her feet, causing the need for bilateral amputations. Her chirodactyls were also affected. Thrombophilia, vasculitis, endocarditis or other embolic sources were investigated and discarded. Her pathology report evidenced skin necrosis and superficial soft parts with recent arterial thrombosis, and Monckeberg's medial calcification. We started treatment with bisphosphonate and sodium thiosulfate, conversion to hemodialysis and replacement of warfarin with unfractionated heparin. Despite all the therapy, the patient died after four months of evolution.
Calciphylaxis is a rare microvasculature calcification syndrome that results in severe ischemic injuries. It has pathogenesis related to the mineral and bone disorder of chronic kidney disease combined with the imbalance between promoters and inhibitors of vascular calcification, with particular importance to vitamin K antagonism.
The preventive strategy is fundamental, since the therapy is complex with poorly validated effectiveness.
血管钙化在心血管发病率和死亡率方面的临床影响已得到充分证实，但其他临床综合征，如钙化防御，尽管不太常见，但对慢性肾脏病有重大影响。
报告 1 例 27 岁女性病例，她因双侧脚趾疼痛 3 天就诊，疼痛部位有小的坏死区。她有 25 年前的 1 型糖尿病病史，患有慢性肾脏病，正在接受腹膜透析，此外还患有类风湿关节炎。她因类风湿关节炎恶化而入院，由于静脉导管并发症导致的心脏内血栓形成，开始使用华法林，病情恶化到脚部，导致需要双侧截肢。她的手指也受到影响。曾对血栓形成倾向、血管炎、心内膜炎或其他栓塞源进行了调查，但都被排除了。她的病理报告显示皮肤坏死和浅层软组织有近期动脉血栓形成，以及 Monckeberg 中层钙化。我们开始用双膦酸盐和硫代硫酸钠治疗，转为血液透析，并将华法林换为未分馏肝素。尽管进行了所有治疗，但患者在 4 个月的病程后死亡。
钙化防御是一种罕见的微血管钙化综合征，可导致严重的缺血性损伤。其发病机制与慢性肾脏病的矿物质和骨代谢紊乱有关，同时伴有血管钙化促进剂和抑制剂之间的失衡，维生素 K 拮抗剂尤为重要。
预防策略是基础，因为治疗复杂，疗效也未经充分验证。
