Importance Recent studies have documented increased bleeding symptoms and related complications in patients with low von Willebrand factor (VWF), highlighting the clinical significance of this entity. Because children and adolescents with VWF deficiencies often present to primary care physicians with bleeding symptoms, physicians need to be aware of this condition for early detection. Observations Studies have found that children and adolescents with low VWF (VWF levels of 30-50 IU/dL) can present with clinically significant bleeding, including mucosal, menstrual, postsurgical, and posttraumatic bleeding, leading to complications such as anemia, iron deficiency, transfusion, hospitalization, and poor quality of life. Detecting and promptly managing low VWF in children and adolescents with bleeding are essential because failure to do so can lead to significant morbidity in adulthood, especially among female patients, including continued heavy menstrual bleeding; postpartum hemorrhage; related gynecologic complications, such as hemorrhagic ovarian cysts; and surgical interventions for heavy menstrual bleeding, including hysterectomy. This narrative review summarizes the observations of several studies that have shed light on the pathophysiologic mechanisms of low VWF and bleeding in these patients and the available diagnostic modalities and treatment options. Conclusions and Relevance Studies in children and adolescents have provided important insights into the clinical phenotype, complications, pathophysiologic mechanisms, evaluation, and management of low VWF, now recognized as an important clinicopathologic entity, as presented in this review. As gatekeepers, primary care physicians play an important role in guiding patients with this recently recognized clinicopathologic entity toward appropriate specialty care and providing continued comanagement to prevent future complications as the patients enter adulthood.

中文翻译：

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儿童和青少年的低 von Willebrand 因子：综述。

重要性 最近的研究记录了低血管性血友病因子 (VWF) 患者的出血症状和相关并发症增加，突出了该实体的临床意义。由于患有 VWF 缺乏症的儿童和青少年经常出现出血症状的初级保健医生，因此医生需要了解这种情况以便及早发现。观察 研究发现，低 VWF（VWF 水平为 30-50 IU/dL）的儿童和青少年会出现临床上显着的出血，包括黏膜出血、月经出血、手术后出血和创伤后出血，导致贫血、缺铁、缺铁等并发症。输血、住院和生活质量差。发现并及时处理儿童和青少年出血的低 VWF 至关重要，因为不这样做会导致成年期的严重发病，尤其是女性患者，包括持续的大量月经出血；产后出血; 相关的妇科并发症，如出血性卵巢囊肿；月经过多的手术干预，包括子宫切除术。这篇叙述性综述总结了几项研究的观察结果，这些研究揭示了这些患者低 VWF 和出血的病理生理机制以及可用的诊断方式和治疗方案。结论和相关性研究对儿童和青少年的临床表型、并发症、病理生理机制、评估、和管理低 VWF，现在被认为是一个重要的临床病理学实体，如本综述所述。作为看门人，初级保健医生在指导患有这种最近被认可的临床病理学实体的患者进行适当的专科护理和提供持续的共同管理以防止患者进入成年期后的未来并发症方面发挥着重要作用。