Abstract:
Glanzmann\'s Thrombasthenia (GT) is an inherited genetic disorder caused by defects in the platelet membrane glycoproteins IIb/IIIA, and is associated with heavy menstrual bleeding (HMB). HMB is a common complication in female patients, and many adolescent girls with this disease have issues with HMB beginning at menarche. The available treatment modalities including anti-fibrinolytics, nonsteroidal anti-inflammatory drugs (NSAIDs) and hormonal therapies though are effective, their associated side effects, limited efficacy and the poor compliance is a challenge in management of HMB. Levonorgestrel-releasing intrauterine system (LNG-IUS) has been a potential alternative to overcome this challenge. The use of the LNG-IUS for the management of HMB in adolescents with GT is explored in this case series.
Two adolescents diagnosed with GT and received the LNG-IUS as treatment modality for management of HMB is discussed in this case series.
For patients with poor compliance to oral hormonal therapies, the use of LNG-IUS is associated with a significant reduction of menstrual blood loss along with improved quality of life. These findings support the use of LNG-IUS to control adolescent GT-related HMB.
摘要:
Glanzmann的血栓形成(GT)是一种遗传性遗传疾病,由血小板膜糖蛋白IIb/IIIA的缺陷引起,并与大量月经出血(HMB)有关。HMB是女性患者常见的并发症,许多患有这种疾病的青春期女孩在初潮时就有HMB的问题。可用的治疗方式,包括抗纤维蛋白溶解,非甾体抗炎药(NSAIDs)和激素治疗虽然是有效的,它们相关的副作用,疗效有限和依从性差是HMB管理中的一个挑战。左炔诺孕酮宫内释放系统(LNG-IUS)已成为克服这一挑战的潜在替代品。在本案例系列中,探讨了使用LNG-IUS管理GT青少年中的HMB。
本病例系列讨论了两名诊断为GT并接受LNG-IUS作为HMB管理的治疗方式的青少年。
对于口服激素疗法依从性差的患者,LNG-IUS的使用与月经失血量的显著减少以及生活质量的改善相关.这些发现支持使用LNG-IUS控制青少年GT相关的HMB。