Kudumija Boris, Knotek Mladen, Pavlović Drasko, Dits Sonja
Lijec Vjesn. 2015 Mar-Apr;137(3-4):91-5.
Calcific uremic arteriolopathy or alciphylaxis is a malignant form of calcification of small arteries and arterioles, usually present in patients with chronic kidney disease and dialysis therapy. It causes high mortality. Histological distinctive feature are calcium deposits lining vascular intima. Calcification of medial muscle layer, inflammation and necrosis of subcutaneous adipose tissue are frequent. The disease begins with painful violaceous mottling, resembling livedo reticularis. Ths skin lesion progresses to ulcers and eschars, sometimes it becomes very vulnerable to secondary infection which can often develop into fatal sepsis. Our first patient with proximal form of calciphylaxis dies in repeated sepsis. The second patient with the distal form of calciphylaxis was treated successfully. The decisive moment was the use of calcimimetic. A multiinterventional strategy is likely to be more effective than any single therapy. It is necessary to regulate metabolism of calcium phosphate and secondary hyperparathyroidism. Effectiveness has been demonstrated using calcimimetics, sodium thiosulfate, oxygen therapy, careful application of biphosphonates and surgical procedures. Warfarin withdrawal is urgently recommended and subsequent vitamin K supplementation is appropriate. The control of infection is critically important and the use of carbonylated hemoglobin in the stage without infections could accelerate the wound healing.
钙化性尿毒症小动脉病或钙化防御是一种小动脉和微动脉钙化的恶性形式，通常见于慢性肾脏病及接受透析治疗的患者。它导致高死亡率。组织学上的显著特征是血管内膜有钙沉积。中层肌肉层钙化、皮下脂肪组织炎症和坏死很常见。该病始于疼痛性紫蓝色斑纹，类似网状青斑。皮肤病变进展为溃疡和焦痂，有时极易继发感染，常发展为致命性脓毒症。我们的首例近端型钙化防御患者死于反复发生的脓毒症。第二例远端型钙化防御患者治疗成功。关键在于使用拟钙剂。多介入策略可能比任何单一疗法更有效。有必要调节磷酸钙代谢及继发性甲状旁腺功能亢进。使用拟钙剂、硫代硫酸钠、氧疗、谨慎应用双膦酸盐及外科手术已证明有效。强烈建议停用华法林，随后补充维生素K是合适的。控制感染至关重要，在无感染阶段使用碳氧血红蛋白可加速伤口愈合。
