Multidisciplinary management of calciphylaxis: a series of 5 patients at a single facility
Multidisciplinary management of calciphylaxis: a series of 5 patients at a single facility
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DOI：
10.1007/s13730-019-00439-8
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发表时间：
2020-05-01
期刊：
影响因子：
1
通讯作者：
Honda, Hirokazu
中科院分区：
文献类型：
--
作者：
Saito, Tomohiro;Mima, Yuuki;Honda, Hirokazu
钙过敏症是一种罕见而严重的疾病，表现为疼痛的皮肤溃疡和坏死。在此，我们报告了五例血液透析患者皮肤活检证实钙化耐受在一个单一的设施。1例患者因重度继发性甲状旁腺功能亢进接受甲状旁腺切除术（PTx），4例患者接受维生素D受体激活剂治疗，2例患者接受华法林治疗。所有患者均有高磷血症，1例有高钙血症。PTx后患者诊断时的全段甲状旁腺激素水平为2 pg/ml，而3例患者在目标范围内。诊断为钙过敏症后的平均时间为2个月。两名患者的大腿和小腿出现皮肤病变，一名患者的足背出现皮肤病变。在皮肤活检中，钙化被发现在小动脉介质中的四名患者，和钙（Ca）沉积在皮肤病变的一名患者。所有患者均接受局部治疗、手术清创、抗生素控制感染性疾病，并严格控制血清钙和磷。除1例1个月前接受PTx的患者外，所有患者均使用了模拟钙剂，4例患者使用了硫代硫酸钠，3例患者使用了低钙透析液。平均随访时间为7.4个月。4名患者治愈，1名患者因感染死亡。我们认为，对感染性疾病的多学科管理，手术清创，从早期开始严格控制矿物质和骨标志物，消除危险因素可能会改善钙化耐受症的病程，这是一种危及生命的疾病。
Calciphylaxis is a rare and severe disease that manifests with painful skin ulceration and necrosis. Herein, we report five patients of hemodialysis patients with skin biopsy-proven calciphylaxis at a single facility. One patient had undergone parathyroidectomy (PTx) due to severe secondary hyperparathyroidism, four had been treated with vitamin D receptor activators, and two were on warfarin therapy. All patients had hyperphosphatemia, and one had hypercalcemia. The intact parathyroid hormone level at diagnosis was 2 pg/ml in the patient after PTx, while three patients were within the target range. The average period after diagnosis of calciphylaxis was 2 months. Skin lesions were present on the thighs and lower legs in two patients, and on the dorsum of the foot in one patient. In skin biopsy, calcification was found in the arteriolar media in four patients, and calcium (Ca) was deposited in the dermal lesion in one patient. All patients received local cures, surgical debridement, antibiotics to control infectious diseases, and strict control of serum Ca and phosphate. Calcimimetics were used in all patients except one who had undergone PTx one month before, sodium thiosulfate was used in 4 patients, and low Ca dialysate was used in three patients. The average follow-up period was 7.4 months. Four patients were cured, and one died due to infection. We suggest that multidisciplinary management for infectious diseases, surgical debridement, strict control of mineral and bone markers from the early stage, and elimination of risk factors may improve the course of calciphylaxis, which is a life-threatening disease.