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[蛋白C和S缺乏对手术患者的临床重要性]

[The clinical importance of protein C and S deficiency for surgical patients].

作者信息

Rabl H, Fruhwirth H

机构信息

Universitätsklinik für Chirurgie, Graz, Osterreich.

出版信息

Langenbecks Arch Chir. 1992;377(2):75-80. doi: 10.1007/BF00184339.

Abstract

Protein C and S are important factors in blood coagulation reported in many papers about people who suffered from thromboembolic diseases related to inherited or acquired deficiencies. Homozygous protein C/S deficiency is lethal in most cases without therapy. Heterozygous deficiency is moderate and complications occur between the 20.-50. year of age. Acquired protein C/S deficiency is a strong parameter for liver function. The typical clinical manifestations of protein C/S deficiencies are superficial and deep leg vein thrombosis, thrombosis of the mesenterial, cerebral, renal and axillary veins, portal vein thrombosis and pulmonary embolism. Most of the affected people live disease free over a longer period and develop thromboembolic complications during and after trauma, surgical interventions, pregnancy and puerperium. We report our experience with a 60 years old male who had developed a severe bilateral iliofemoral vein thrombosis with signs of pulmonary embolism after total hip replacement. An extended functional protein C deficiency (type II) was investigated by coagulation tests (Protein C Reagent, coagulometric from Behring Institute). A second female patient developed a descending iliofemoral vein thrombosis during pregnancy. Venous thrombectomy with arteriovenous fistula was performed, but reocclusion occurred after delivery. Redo-surgery was undertaken and a second reocclusion took place 10 days later. Further lysis therapy was not able to reopen the venous system. Whereas immunological and functional protein C levels showed normal ranges, the functional protein S level was markedly reduced (IL-Instrumentation Laboratory Protein S-Test).

摘要

蛋白C和蛋白S是血液凝固中的重要因子,许多关于患有与遗传性或获得性缺陷相关的血栓栓塞性疾病患者的论文都有报道。在大多数未经治疗的情况下,纯合子蛋白C/蛋白S缺乏是致命的。杂合子缺乏程度中等,并发症发生在20至50岁之间。获得性蛋白C/蛋白S缺乏是肝功能的一个重要指标。蛋白C/蛋白S缺乏的典型临床表现为浅表和深部下肢静脉血栓形成、肠系膜静脉、脑静脉、肾静脉和腋静脉血栓形成、门静脉血栓形成和肺栓塞。大多数患者在较长时间内无疾病发生,并在创伤、手术干预、妊娠和产褥期期间及之后发生血栓栓塞并发症。我们报告了一名60岁男性的病例,他在全髋关节置换术后发生了严重的双侧髂股静脉血栓形成,并伴有肺栓塞迹象。通过凝血试验(来自贝林研究所的蛋白C试剂,凝固法)检测到存在一种严重的功能性蛋白C缺乏(II型)。另一名女性患者在怀孕期间发生了下行性髂股静脉血栓形成。进行了动静脉瘘的静脉血栓切除术,但产后发生了再闭塞。进行了再次手术,10天后再次发生再闭塞。进一步的溶栓治疗未能重新开通静脉系统。虽然免疫和功能性蛋白C水平显示在正常范围内,但功能性蛋白S水平明显降低(IL-仪器实验室蛋白S检测)。

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