Lamas Adelaida, Máiz Luis, Ruiz de Valbuena Marta, González-Casbas José Manuel, Suárez Lucrecia
Department of Paediatric Pulmonology and Cystic Fibrosis Unit, Ramón y Cajal University Hospital, Carretera de Colmenar Km, 9,1, 28034 Madrid, Spain.
BMC Pulm Med. 2014 Oct 24;14:165. doi: 10.1186/1471-2466-14-165.
Cystic Fibrosis (CF) is a genetic disease with equal prevalence across sexes. However, women present worse lung function with faster function decline, earlier onset of bacterial colonization, more frequent pulmonary exacerbations (PE), greater bronchial hyper-responsiveness, and higher mortality rates after puberty than men. The etiology of this gender disparity remains elusive but female hormones have been implicated in several studies.
A 20-year-old female with CF with severe recurrent PE, always related to the menstrual cycle since menarche, combined with lung function decline requiring multiple courses of intravenous antibiotics. We report the cessation of PE and recovery of pulmonary function following the insertion of a subcutaneous implant with 68 mg of etonogestrel (Implanon®, Organon Española S.A. Laboratories, Madrid, Spain).
Our case report supports the key role of female hormones in the development of PE and in the decline of lung function in a woman with CF. When appropriate, hormonal manipulation through contraceptive methods should be considered as potential treatment.
囊性纤维化(CF)是一种男女患病率相等的遗传性疾病。然而,女性的肺功能较差,功能下降更快,细菌定植更早,肺部加重(PE)更频繁,支气管高反应性更高,青春期后死亡率也高于男性。这种性别差异的病因尚不清楚,但多项研究表明女性激素与之有关。
一名20岁患有CF的女性,严重反复发生PE,自初潮起就一直与月经周期有关,同时肺功能下降,需要多次静脉使用抗生素治疗。我们报告了在皮下植入68毫克依托孕烯(Implanon®,西班牙奥加农公司实验室,马德里,西班牙)后PE停止且肺功能恢复的情况。
我们的病例报告支持女性激素在患有CF的女性发生PE及肺功能下降中起关键作用。在适当情况下,应考虑通过避孕方法进行激素调节作为潜在治疗手段。