Hopkins S
Nurs Times. 1995;91(39):40-1.
Cystic fibrosis is an autosomal recessive disease, characterised by pancreatic insufficiency, abnormal viscous mucus secretions and chronic respiratory tract infections. Treatment is with pancreatin preparations to improve intestinal digestion of food, but the use of high-potency products requires care. Postural drainage and other physiotherapeutic measures are essential to the relief of respiratory obstruction. A new approach to the respiratory problems of cystic fibrosis is dornase alpha, a mucolytic enzyme given by inhalation. Gene therapy may eventually provide the definitive answer to treatment and is already on the therapeutic horizon, but many practical problems remain to be solved.
囊性纤维化是一种常染色体隐性疾病，其特征为胰腺功能不全、异常黏稠的黏液分泌以及慢性呼吸道感染。治疗方法是使用胰酶制剂来改善肠道对食物的消化，但使用高效产品时需谨慎。体位引流和其他物理治疗措施对于缓解呼吸道梗阻至关重要。一种针对囊性纤维化呼吸问题的新方法是使用雾化吸入的黏液溶解酶——脱氧核糖核酸酶α。基因治疗最终可能为治疗提供决定性答案，并且已经处于治疗前景之中，但仍有许多实际问题有待解决。
