Gilljam M, Antoniou M, Shin J, Dupuis A, Corey M, Tullis D E
Department of Respiratory Medicine and Allergology, Sahlgrenska University Hospital, Göteborg University, Göteborg, Sweden.
Chest. 2000 Jul;118(1):85-91. doi: 10.1378/chest.118.1.85.
To assess the effect of pregnancy on pulmonary function and survival in women with cystic fibrosis (CF) and to assess the fetal outcome.
Cohort study. The data analyzed were collected from the Toronto CF database, chart review, and patient questionnaire.
Tertiary-care center.
All women with CF who, at the time of diagnosis or pregnancy, attended the Toronto Cystic Fibrosis Clinics between 1961 and 1998.
From 1963 to 1998, there were 92 pregnancies in 54 women. There were 11 miscarriages and 7 therapeutic abortions. Forty-nine women gave birth to 74 children. The mean follow-up time was 11 +/- 8 years. One patient was lost to follow-up shortly after delivery, and one was lost after 12 years. The overall mortality rate was 19% (9 of 48 patients). Absence of Burkholderia cepacia (p < 0.001), pancreatic sufficiency (p = 0.01), and prepregnancy FEV(1) > 50% predicted (p = 0.03) were associated with better survival rates. When adjusted for the same parameters, pregnancy did not affect survival compared to the entire adult female CF population. The decline in FEV(1) was comparable to that in the total CF population. Three women had diabetes mellitus, and seven developed gestational diabetes. There were six preterm infants and one neonatal death. CF was diagnosed in two children.
The maternal and fetal outcome is good for most women with CF. Risk factors for mortality are similar to those for the nonpregnant CF population. Pregnancies should be planned so that there is opportunity for counseling and optimization of the medical condition. Good communication between the CF team and the obstetrician is important.
评估妊娠对囊性纤维化（CF）女性肺功能和生存的影响，并评估胎儿结局。
队列研究。分析的数据来自多伦多CF数据库、病历回顾和患者问卷。
三级医疗中心。
1961年至1998年间在多伦多囊性纤维化诊所就诊的所有CF女性，包括诊断时或妊娠时的患者。
1963年至1998年，54名女性中有92次妊娠。有11次流产和7次治疗性流产。49名女性生下74名儿童。平均随访时间为11±8年。1名患者在分娩后不久失访，1名在12年后失访。总死亡率为19%（48名患者中的9名）。无洋葱伯克霍尔德菌（p<0.001）、胰腺功能正常（p=0.01）和孕前FEV（1）>预测值的50%（p=0.03）与较高的生存率相关。在对相同参数进行调整后，与整个成年女性CF人群相比，妊娠并不影响生存。FEV（1）的下降与整个CF人群相当。3名女性患有糖尿病，7名患妊娠期糖尿病。有6名早产儿和1名新生儿死亡。2名儿童被诊断为CF。
大多数CF女性的母婴结局良好。死亡的危险因素与非妊娠CF人群相似。应计划妊娠，以便有机会进行咨询和优化医疗状况。CF团队与产科医生之间的良好沟通很重要。
