Vinatier D, Buvat J, Tonnel B, Monnier J C, Maunoury-Lefebvre C
J Gynecol Obstet Biol Reprod (Paris). 1986;15(2):195-200.
We have seen a patient who became pregnant. She was suffering from cystic fibrosis diagnosed late in life at the age of 26 years. Her case has allowed the authors to make some observations on this association of pregnancy with cystic fibrosis which will become increasingly common. 5% of individuals are heterozygous for this condition. The prospect for these patients surviving gets better and better. Although their fertility seems to be lowered it is possible for them to become pregnant. They are caught in the web of almost constant episodes of broncho-pulmonary infection with lasting effects on their respiratory capacities in 50% of cases. About 83% of these patients will deliver prematurely because of the respiratory distress they suffer. It is difficult to predict the long-term effects of pregnancy on the survival of these patients since the mortality rate anyhow is about 10% per year. According to the literature pregnancy will cause a permanent deterioration in respiratory function in about 37% of cases. The history of this particular case should be able to give new hope to some women suffering from this condition. All the same, there are a large number of problems that still have to be dealt with.
我们接诊了一位怀孕的患者。她患有囊性纤维化,26岁时才被确诊,属于晚发性。她的病例让作者得以对妊娠与囊性纤维化的这种关联进行一些观察,而这种情况会越来越常见。5%的人是这种疾病的杂合子。这些患者存活的前景越来越好。尽管他们的生育能力似乎有所下降,但仍有可能怀孕。他们深陷几乎持续不断的支气管肺部感染的困境,50%的病例会对其呼吸功能产生持久影响。这些患者中约83%会因所遭受的呼吸窘迫而早产。由于无论如何死亡率约为每年10%,所以很难预测妊娠对这些患者存活的长期影响。根据文献,约37%的病例中妊娠会导致呼吸功能永久性恶化。这个特殊病例的病史应该能给一些患有这种疾病的女性带来新的希望。尽管如此,仍有大量问题有待解决。