[Fertility and cystic fibrosis. Management at pregnancy]

Life expectancy of patients with cystic fibrosis has regularly increased in recent years, currently exceeding the age of 30 years. The disease now concerns adolescents and young adults more than children. Thus problems linked to fertility have to be considered by physicians caring for adult patients. In males, sterility is quite constant. Changes are related to bilateral agenesis of the efferent ducts leading to excretory azospermia which is one of the major diagnostic criteria leading to the diagnosis of cystic fibrosis in adults. This form of sterility may be relieved by removal of sperm from the epididymis to achieve in vitro fertilization. In the female, fertility is sometimes diminished either by disease-specific abnormalities (tubal epithelium and cervical mucus) or impaired by respiratory failure or nutritional factors. Pregnancy is however possible and is observed in an increasing number of cases. Pregnancy does not appear to alter the general prognosis of the disease as long as cardiorespiratory function is preserved. In all cases, a prognostic evaluation and genetic advice are indispensable.