Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease
Clinical manifestations and complications of childbirth and replacement therapy in 385 Iranian patients with type 3 von Willebrand disease
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DOI：
10.1046/j.1365-2141.2000.02507.x
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发表时间：
2000-12-01
影响因子：
6.5
通讯作者：
Mannucci, PM
中科院分区：
文献类型：
--
作者：
Lak, M;Peyvandi, F;Mannucci, PM
3型是最严重的血管性血友病（VWD），以常染色体隐性遗传特征传播。我们通过检查来自300名伊朗患者的385名患者，并与100名年龄匹配的重度血友病A患者进行比较，收集了3型VWD的临床表现数据。关节和肌肉出血的频率低于血友病患者，这可能是因为Vm因子水平普遍较高（中位数为4% vs 1%或更低）。粘液道出血如鼻衄和月经过多是VWD最常见的症状。包皮环切术后，口腔出血经常发生时，预防性替代治疗不进行或不足。妊娠过程通常平安无事，但当受影响的妇女接受替代治疗少于3-4 d时，分娩时出血增加。这些多次输血患者中有10例（2.6%）产生了VWF同种抗体，55%慢性感染丙型肝炎病毒。
Type 3 is the most severe form of von Willebrand disease (VWD) transmitted as an autosomal recessive trait. We collected data on the clinical manifestations of type 3 VWD by examining 385 patients from 300 Iranian kindreds, who were compared with 100 age-matched patients with severe haemophilia A. Joint and muscle bleeding was less frequent than in haemophiliacs, perhaps because factor Vm levels were in general higher (median value 4% vs, 1% or less). Mucosal tract haemorrhages such as epistaxis and menorrhagia were the most prevalent symptoms in VWD. Post-circumcision and oral cavity bleeding occurred frequently when prophylactic replace ment therapy was not carried out or was inadequate. The course of pregnancy was usually uneventful, but increased bleeding occurred at parturition when affected women were treated with replacement therapy for less than 3-4 d. Ten of 385 (2.6%) of these multitransfused patients developed an alloantibody to VWF and 55% are chronically infected with the hepatitis C virus.