Background: Pregnancies in patients with cystic fibrosis (CF) are subject to an increased risk of complications.
Methods: We have made a systematic review of the literature concerning pregnancies in women with CF to evaluate prognostic factors of pregnancy outcome such as lung function and nutritional status, also including and describing the case of a woman suffering from CF who had two pregnancies in her late thirties, both with a good outcome.
Results: Thirteen case series and 22 case reports involving 523 pregnancies in 401 women were extracted. 83.1% of 516 pregnancies whose outcome was known resulted in the delivery of live births, with preterm birth rate of 24%. Miscarriage occurred in 6.3% and therapeutic abortion in 10% of pregnancies. Indirect mother's death occurred in seven cases. In our case report, the course of the second pregnancy proved to be much more difficult than the first, with preterm delivery probably related to a worsening of lung disease in the third trimester of pregnancy.
Conclusions: Pre-pregnant lung function as well as lung function deterioration, CF-related diabetes mellitus and weight gain in pregnancy, are parameters to consider in the counseling about the outcome of pregnancy.