Features of Menstruation and Menstruation Management in Individuals with Rett Syndrome,Journal of Pediatric and Adolescent Gynecology

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Features of Menstruation and Menstruation Management in Individuals with Rett Syndrome
Journal of Pediatric and Adolescent Gynecology ( IF 1.8 ) Pub Date : 2020-11-10 , DOI: 10.1016/j.jpag.2020.11.002
Kristen N Humphrey ₁ , Paul S Horn ₂ , Leanne Olshavsky ₃ , Lisa Reebals ₄ , Shannon M Standridge ₅

Affiliation

Study Objective

To describe features of menstruation, menstrual-related symptoms, and menstrual management in females with Rett syndrome (RTT) to help develop a clinical approach to these parameters in RTT.

Design

Retrospective cross-sectional chart review and prospective survey.

Setting

Cincinnati Children's Hospital Medical Center, Rett Syndrome and Related Spectrum Disorders Clinic.

Participants

Females with RTT (12-55 years of age) and their caregivers.

Main Outcome Measures

Descriptive data on features of menstruation and menstrual-related symptoms in individuals with Rett syndrome; prevalence, types, reason for use/discontinuation, and efficacy of hormonal treatment in females with RTT.

Results

Age at menarche, menstrual cycle length, and menstrual period length in females with RTT are comparable to those in typically developing females and females with other neurodevelopmental disabilities. Dysmenorrhea and emotional lability are common menstrual cycle−related changes among females with RTT; 22.1% of participants also reported catamenial seizures. Oral progestin, combined oral contraceptive pill, and depot-medroxyprogesterone acetate (DMPA) were effectively used to suppress or regulate menstruation and to manage menstrual-related symptoms.

Conclusions

Characteristics of menstruation in females with RTT are comparable to those of typically developing females, with the exception of increase in catamenial seizure activity. Hormonal treatments are used for management of menstruation, dysmenorrhea, and seizures. Choice of hormonal treatment is influenced by bone health and immobility in females with RTT.

中文翻译：

Rett综合征患者的月经特征和月经管理

学习目标

描述 Rett 综合征 (RTT) 女性的月经特征、月经相关症状和月经管理，以帮助开发针对 RTT 中这些参数的临床方法。

设计

回顾性横截面图表审查和前瞻性调查。

环境

辛辛那提儿童医院医疗中心，雷特综合症及相关谱系障碍诊所。

参加者

患有 RTT 的女性（12-55 岁）及其照顾者。

主要观察指标

关于雷特综合征患者月经特征和月经相关症状的描述性数据；RTT 女性激素治疗的患病率、类型、使用/停药原因和疗效。

结果

患有 RTT 的女性的月经初潮年龄、月经周期长度和月经期长度与正常发育女性和患有其他神经发育障碍的女性相当。痛经和情绪不稳是 RTT 女性常见的与月经周期相关的变化；22.1% 的参与者还报告了经期癫痫发作。口服孕激素、复方口服避孕药和长效醋酸甲羟孕酮 (DMPA) 可有效抑制或调节月经，并控制月经相关症状。