Monfort-Gouraud M, Leroy B, Favier R, Bensman A
Service de néphrologie pédiatrique, hôpital Trousseau, Paris, France.
Arch Pediatr. 1995 Feb;2(2):156-9. doi: 10.1016/0929-693x(96)89877-0.
Plasma concentration of several hemostatic proteins may be modified during the acute phase of nephrotic syndrome. The case of such a syndrome in a patient with congenital factor V deficiency is presented. CASE REPORT--A 5 year-old girl with partial congenital factor V deficiency (level: 30%), was admitted for nephrotic syndrome complicating Henoch-Schönlein purpura. Urine protein excretion was 4 g/24 h. Initial plasma concentrations were: protein: 5.5 g/dl; albumin: 3.3 g/dl; factor II: 85%; factor V: 56%; factors VII + X: 80%. The patient was given methylprednisolone (1 g/1.73 m2) followed by prednisone (2 mg/kg/day). Under this treatment, the plasma concentrations were: protein: 4.5 g/dl; albumin: 2.0 g/dl; factor II: 180%; factor V: 84%; factors VII + X: 120%. Values at the onset of remission were: albumin: 3.4 g/dl; factor V: 49%. CONCLUSION--This observation suggests that hypoalbuminemia may enhance liver synthesis of factor V as known for some other coagulation factors, and transiently correct the hereditary deficiency.
在肾病综合征急性期，几种止血蛋白的血浆浓度可能会发生改变。本文报告了一名先天性因子V缺乏患者出现这种综合征的病例。病例报告——一名5岁女童，患有部分先天性因子V缺乏（水平：30%），因肾病综合征并发过敏性紫癜入院。尿蛋白排泄量为4g/24小时。初始血浆浓度为：蛋白质：5.5g/dl；白蛋白：3.3g/dl；因子II：85%；因子V：56%；因子VII + X：80%。给予患者甲泼尼龙（1g/1.73m²），随后给予泼尼松（2mg/kg/天）。在这种治疗下，血浆浓度为：蛋白质：4.5g/dl；白蛋白：2.0g/dl；因子II：180%；因子V：84%；因子VII + X：120%。缓解开始时的值为：白蛋白：3.4g/dl；因子V：49%。结论——该观察结果表明，低白蛋白血症可能会增强肝脏对因子V的合成，就像其他一些凝血因子一样，并暂时纠正遗传性缺乏。
