Lecerf C, Hutinel B, Combe-Tamzali S, Conard J, Samama M
Phlebologie. 1986 Apr-Jun;39(2):415-29.
The appearance of relapsing venous thrombosis after sclerosis of varices in a woman aged 30 led us to discover a quantitative protein C deficiency, found in her father and 4 of her brothers and sisters. The transmission mode is autosomal and dominant. Within this family, 6 subjects are affected, 4 showing symptoms, clearly showing the heterogeneity of the clinical manifestation of the deficiency. Dealing with the special problem of pregnancy in women with the biological disorder, one ought to consider the literature on the subject, showing the large occurrence of ante- and post-natal accidents. The treatments of thromboembolic complications is based on the use of heparin administered appropriately through antivitamins K for a period which is till to be defined. In asymptomatic subjects, a preventive anticoagulant treatment is recommended in all circumstances likely to encourage the relapse of thrombosis.
一名30岁女性静脉曲张硬化后出现复发性静脉血栓形成,这使我们发现了她父亲以及4名兄弟姐妹存在定量蛋白C缺乏。遗传方式为常染色体显性遗传。在这个家族中,6名成员受影响,4名有症状,清楚显示了该缺乏临床表现的异质性。对于患有这种生物学紊乱的女性在妊娠时的特殊问题,应参考相关文献,这些文献显示产前和产后意外情况发生率很高。血栓栓塞并发症的治疗基于使用肝素,并通过维生素K拮抗剂适当给药,给药时长有待确定。对于无症状个体,在所有可能促使血栓形成复发的情况下,均建议进行预防性抗凝治疗。