Barak Asher, Dulitzki Mordechai, Efrati Ori, Augarten Arie, Szeinberg Amir, Reichert Nira, Modan Dalit, Weiss Batia, Miller Mervin, Katzanelson Daniel, Yahav Yaacov
National Cystic Fibrosis Center, Safra Children's Hospital, Sheba Medical Center, Tel Hashomer, Israel.
Isr Med Assoc J. 2005 Feb;7(2):95-8.
Along with the increased life expectancy in cystic fibrosis and the remarkable progress in its management and therapy, issues of female fertility and pregnancy are frequently raised. These include infertility, severity of lung disease, pancreatic insufficiency, poor nutritional status, glucose intolerance and diabetes, drug safety, and long-term maternal and neonatal outcome.
To describe the experience of our CF center in the management of CF pregnant woman from 1977 to 2004.
We analyzed 27 years of records (1977-2004) of the national CF registry of all CF women who wished to conceive and became pregnant.
Eight CF women (mean age 24 +/- 4.5 years) who wished to conceive had 11 pregnancies and delivered 12 neonates. The pregestational results of forced expiratory volume per 1 second varied significantly among patients (59 +/- 23%), yet most (10/11) stayed stable throughout the pregnancy course. Maternal deterioration in CF condition occurred in only one mother, necessitating cesarean section. In 9 of the 11 pregnancies the women were pancreatic-insufficient. Of the 11 pregnancies, 2 CF women had diabetes mellitus and 3 developed gestational diabetes. One pregnancy occurred in a mother with a transplanted lung. Of the 12 neonates, 3 were preterm and one was born with esophageal atresia. No miscarriages, terminations or neonatal mortalities occurred. Although most of the CF mothers had FEV1 below 55% before pregnancy, the maternal and neonatal outcome was favorable and lung function tests generally remained stable.
We conclude that pregnancy in CF is feasible with a positive maternal and neonatal outcome. Early participation of the CF physician in the wish of the CF woman to reproduce is required. The integration of an intensive multidisciplinary approach during pregnancy, which includes close follow-up of maternal and fetal condition by the various specialists, should ensure an optimal outcome.
随着囊性纤维化患者预期寿命的延长以及其管理和治疗方面的显著进展，女性生育和妊娠问题经常被提及。这些问题包括不孕、肺部疾病的严重程度、胰腺功能不全、营养状况差、葡萄糖不耐受和糖尿病、药物安全性以及母婴长期结局。
描述我们的囊性纤维化中心在1977年至2004年期间管理囊性纤维化孕妇的经验。
我们分析了全国囊性纤维化登记处27年（1977 - 2004年）中所有希望怀孕并成功怀孕的囊性纤维化女性的记录。
8名希望怀孕的囊性纤维化女性（平均年龄24±4.5岁）共怀孕11次，分娩12名新生儿。患者的一秒用力呼气量孕前结果差异显著（59±23%），但大多数（10/11）在整个孕期保持稳定。仅1名母亲的囊性纤维化病情出现恶化，需要剖宫产。11次怀孕中有9名女性存在胰腺功能不全。11次怀孕中，2名囊性纤维化女性患有糖尿病，3名出现妊娠期糖尿病。1次怀孕发生在一名接受过肺移植的母亲身上。12名新生儿中，3名早产，1名出生时患有食管闭锁。未发生流产、终止妊娠或新生儿死亡。尽管大多数囊性纤维化母亲孕前的第一秒用力呼气量低于55%，但母婴结局良好，肺功能测试总体保持稳定。
我们得出结论，囊性纤维化患者怀孕是可行的，母婴结局良好。囊性纤维化医生需要尽早参与到囊性纤维化女性的生育愿望中。孕期采用强化多学科方法，包括各专科医生对母婴状况进行密切随访，应能确保获得最佳结局。
