Venkateswaran Lakshmi, Yee Donald L
Department of Pediatrics, Section of Hematology/Oncology, Baylor College of Medicine, Houston, Texas, USA.
J Pediatr Adolesc Gynecol. 2010 Dec;23(6 Suppl):S38-42. doi: 10.1016/j.jpag.2010.08.011.
Rare bleeding disorders can cause significant bleeding in children and adolescents. These encompass rare clotting factor deficiencies, and fibrinolytic pathway defects. Vascular anomalies can cause recurrent and refractory bleeding, and are included in this review of rare causes of abnormal bleeding in children and adolescents. Menorrhagia is often reported as a manifestation of these conditions. Succinct knowledge about these disorders, their clinical presentation, diagnostic work-up, and therapeutic options are crucial to the accurate diagnosis and optimal management of affected patients. This review provides an overview of these infrequently encountered disorders, discusses their recognition based on results of suggested screening tests and offers a general guideline for approach to therapy.
罕见出血性疾病可导致儿童和青少年出现严重出血。这些疾病包括罕见的凝血因子缺乏症和纤维蛋白溶解途径缺陷。血管异常可导致反复难治性出血，本文对儿童和青少年异常出血的罕见原因进行综述时将其纳入其中。月经过多常被报道为这些疾病的一种表现。对这些疾病、其临床表现、诊断检查及治疗选择有简洁的了解，对于准确诊断和优化治疗受影响患者至关重要。本综述概述了这些不常见的疾病，讨论了基于建议筛查试验结果对它们的识别，并提供了治疗方法的一般指南。
