Høiby N
Department of Clinical Microbiology, Rigshospitalet, Copenhagen, Denmark.
Curr Opin Pediatr. 1993 Jun;5(3):247-54. doi: 10.1097/00008480-199306000-00002.
The discovery of the cystic fibrosis gene has provided new approaches to gene therapy and drug therapy. At the present time, however, we are still faced with the morbidity and mortality due to lung infections caused by Pseudomonas aeruginosa and Pseudomonas cepacia. The present survey of some of the recently published articles shows that applied science related to the clinical care of cystic fibrosis (CF) patients is also progressing. Cross-infection in CF centers and during social contacts between CF patients out of the hospital environment has been described in some countries. Prophylactically, cohort isolation procedures are effective. The individual clinical course of the chronic pulmonary infection is adversely influenced during prediabetic mellitus periods and by development of high titers of specific IgG2 and IgG3 antibodies against P. aeruginosa. A vaccine against P. aeruginosa is currently being tested in CF patients. The induced antibodies have much higher affinity and opsonophagocytic activity than infection-induced antibodies. Ciprofloxacin, which is widely used in CF, easily gives rise to resistance in P. aeruginosa and Staphylococcus aureus. Restricting the use of this drug in CF is therefore necessary. Aerosolized recombinant DNase is a promising and efficient new drug for management of airflow limitation due to purulent airway secretion in CF. For end-stage CF, double-lung and heart-lung transplantation show encouraging intermediate-term results, the two main obstacles being donor organ shortage and obliterative bronchiolitis.
囊性纤维化基因的发现为基因治疗和药物治疗提供了新方法。然而,目前我们仍面临由铜绿假单胞菌和洋葱伯克霍尔德菌引起的肺部感染所导致的发病率和死亡率问题。对近期发表的一些文章的调查显示,与囊性纤维化(CF)患者临床护理相关的应用科学也在不断发展。在一些国家,已经描述了CF中心内以及医院外CF患者社交接触期间的交叉感染情况。预防性地采用群组隔离程序是有效的。在糖尿病前期以及针对铜绿假单胞菌产生高滴度特异性IgG2和IgG3抗体时,慢性肺部感染的个体临床病程会受到不利影响。目前正在CF患者中测试一种针对铜绿假单胞菌的疫苗。诱导产生的抗体比感染诱导产生的抗体具有更高的亲和力和调理吞噬活性。在CF治疗中广泛使用的环丙沙星很容易导致铜绿假单胞菌和金黄色葡萄球菌产生耐药性。因此,有必要在CF治疗中限制这种药物的使用。雾化重组脱氧核糖核酸酶是一种有前景且有效的新药,可用于治疗CF患者因脓性气道分泌物导致的气流受限。对于终末期CF患者,双肺移植和心肺移植显示出令人鼓舞的中期结果,两个主要障碍是供体器官短缺和闭塞性细支气管炎。