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一名患有蛋白S缺乏症的慢性血液透析患者发生了钙化防御。

Calciphylaxis in a chronic hemodialysis patient with protein S deficiency.

作者信息

Rostaing L, el Feki S, Delisle M B, Durand-Malgouyres C, Ton-That H, Bonafe J L, Bories P, Durand D, Suc J M

机构信息

Service de Néphrologie, CHU Rangueil, Toulouse, France.

出版信息

Am J Nephrol. 1995;15(6):524-7. doi: 10.1159/000168900.

DOI:10.1159/000168900
PMID:8546177
Abstract

Vascular calcifications are common in uremic patients whereas calciphylaxis is rare. We report the case of a 45-year-old woman on chronic hemodialysis since 1977. She had a subtotal parathyroidectomy in 1985, aortic and mitral valve replacement in 1986, and has been treated since then with nicoumalone. In June 1991, she presented with repeated, painful cutaneous necrosis suggesting panniculitis. A skin biopsy showed lobular panniculitis and evidence of calciphylaxis. There was an obvious biological hyperparathyroidism. Protein C functional level was in the normal range whereas protein S functional level was low, i.e. 42%. The patient underwent cervical surgery to remove two parathyroid glands, and daily hemodialysis sessions. Despite this treatment, cutaneous necrosis progressed with superinfection. A few weeks later, the patient died from a septic shock after a myocardic infarction. Necropsy was not performed.

摘要

血管钙化在尿毒症患者中很常见,而钙化防御却很罕见。我们报告一例自1977年起接受慢性血液透析的45岁女性病例。她于1985年接受了甲状旁腺次全切除术,1986年进行了主动脉和二尖瓣置换术,此后一直服用苯丙香豆素进行治疗。1991年6月,她出现反复的、疼痛性皮肤坏死,提示脂膜炎。皮肤活检显示小叶性脂膜炎及钙化防御的证据。存在明显的生物性甲状旁腺功能亢进。蛋白C功能水平在正常范围内,而蛋白S功能水平较低,即42%。该患者接受了颈部手术以切除两个甲状旁腺,并进行每日血液透析治疗。尽管进行了这种治疗,皮肤坏死仍进展并出现了感染。几周后,该患者在心肌梗死后死于感染性休克。未进行尸检。

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