Mikhail Sameh, Kouides Peter
Lombardi Comprehensive Cancer Center, Georgetown University Hospital, Washington, DC, USA.
J Pediatr Adolesc Gynecol. 2010 Dec;23(6 Suppl):S3-10. doi: 10.1016/j.jpag.2010.08.005.
Recent studies indicate that bleeding disorders, particularly von Willebrand disease (VWD) is more prevalent than previously thought in adolescents with menorrhagia. Menorrhagia management in undiagnosed disorders of hemostasis may be associated with unwanted risks and complications. The prevalence of symptomatic VWD in the pediatric primary care setting appears to be 0.11% (95% CI, 0.04-0.25%). Studies evaluating the prevalence of VWD in adolescents with menorrhagia have included over 500 patients with a prevalence range from 3 to 36% depending on the clinical setting studied, with the highest prevalence seen in adolescents referred to an outpatient Hemophilia Center, while the lowest prevalence is seen in the acute hospital setting. Recently, the diagnosis of VWD has been facilitated by the use of pediatric bleeding questionnaires that have proved useful in quantifying the severity of bleeding symptoms. Treatment of VWD is often complex because a combination of therapies is often required. Potential treatment options include estrogen-progesterone preparations, desmopressin, antifibrinolytic agents and von Willebrand factor concentrates. More research is needed to evaluate the effectiveness of the various treatment modalities in the adolescent population.
近期研究表明，出血性疾病，尤其是血管性血友病（VWD）在月经过多的青少年中比之前认为的更为普遍。在未确诊的止血障碍中进行月经过多的管理可能会带来不必要的风险和并发症。在儿科初级保健环境中，有症状的VWD患病率似乎为0.11%（95%置信区间，0.04 - 0.25%）。评估月经过多青少年中VWD患病率的研究纳入了500多名患者，患病率范围为3%至36%，具体取决于所研究的临床环境，在转诊至门诊血友病中心的青少年中患病率最高，而在急性医院环境中患病率最低。最近，儿科出血问卷的使用有助于VWD的诊断，该问卷已被证明有助于量化出血症状的严重程度。VWD的治疗通常很复杂，因为往往需要多种治疗方法联合使用。潜在的治疗选择包括雌激素 - 孕激素制剂、去氨加压素、抗纤维蛋白溶解剂和血管性血友病因子浓缩物。需要更多研究来评估各种治疗方式在青少年人群中的有效性。
