Study Objective

To assess the treatment patterns and efficacy of hormonal (HM) and non-HM (NHM) management of heavy menstrual bleeding (HMB) in young women with inherited platelet function disorders (IPFDs).

Design, Setting, and Participants

A retrospective chart review was performed of outpatient treatment of HMB in female patients age 9-25 years who were diagnosed with IPFDs and referred to gynecology and/or hematology at a tertiary care hospital between 2006 and 2018.

Interventions

The study sample was identified using billing codes for IPFDs. Data on HM and NHM treatments and outcomes over a one- to two-year period were collected. Initial treatment was defined as the first treatment prescribed after referral. Descriptive statistics, Pearson χ², and t tests were used for analysis.

Main Outcome Measures

Treatment failure was defined as a change in treatment method because of continued bleeding.

Results

Thirty-four girls met inclusion criteria. After their initial visit, 19/34 (56%) were treated with HM, 12/34 (35%) with NHM, 2/34 (6%) with a combination of methods, and 1/34 (3%) were untreated. Initial treatment failed in 19/34 (56%) and those patients subsequently required a mean of 2 additional treatments during follow-up. Of the 34 included, 6/34 (18%) remained uncontrolled despite numerous treatment changes and 2/34 (6%) because of noncompliance. When control was achieved, 7/26 (27%) of patients were receiving combined oral contraceptives and 6/26 (23%) desmopressin acetate.

Conclusion

HMB in girls with IPFDs can be difficult to control despite ongoing follow-up and treatment changes. Although the most effective treatment for HMB in young women with IPFDs was not identified, these findings will help providers and patients with setting expectations. Prospective studies are needed to develop recommendations on best practices.

中文翻译：

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具有遗传性血小板功能障碍的青春期和年轻女性的重度月经出血的门诊治疗。

研究目的

在患有遗传性血小板功能障碍（IPFD）的年轻女性中，评估重度月经出血（HMB）的激素（HM）和非HM（NHM）处理的模式和疗效。

设计，设置和参与者

回顾性图表审查了2006年至2018年间在三级护理医院诊断为IPFD并转诊至妇科和/或血液科的9-25岁女性患者中HMB的门诊治疗。

干预措施

使用IPFD的计费代码来确定研究样本。收集有关HM和NHM治疗以及一到两年期间结果的数据。初始治疗定义为转诊后开出的第一种治疗方法。描述性统计，皮尔逊χ ²，和牛逼用于分析测试。

主要观察指标

治疗失败被定义为由于持续出血而改变治疗方法。

结果

共有34名女孩符合入选标准。初次就诊后，用HM治疗19/34（56％），使用NHM治疗12/34（35％），使用多种方法治疗的2/34（6％）和未经治疗的1/34（3％） 。最初的治疗失败率为19/34（56％），随后这些患者在随访期间平均需要再接受2次治疗。在包括的34例患者中，尽管进行了多次治疗更改，但仍有6/34例（18％）不受控制，而由于不合规而有2/34例（6％）。达到控制后，7/26（27％）的患者正在接受联合口服避孕药和6/26（23％）的醋酸去氨加压素。

结论

尽管有后续的随访和治疗方法改变，但IPFD女孩的HMB仍难以控制。尽管尚未确定IPFD年轻女性中HMB的最有效治疗方法，但这些发现将有助于提供者和患者设定期望。需要进行前瞻性研究，以提出有关最佳做法的建议。