Amin Reshma, Ratjen Felix
Division of Respiratory Medicine, Department of Pediatrics, The Hospital for Sick Children, University of Toronto, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
Adv Pediatr. 2008;55:99-121. doi: 10.1016/j.yapd.2008.07.015.
In summary, there is a significant interplay between the pulmonary manifestations and nutritional status of CF patients. The advances in CF clinical care in the past 2 decades are mainly attributed to anti-infective therapy as well as aggressive nutritional management. Currently, there are multiple therapeutic agents that are in clinical trial that target either the underlying CFTR defect or the downstream effects of CFTR. The broad spectrum of therapeutic agents being studied as well as the advances in therapies that target the underlying CFTR defect are exciting, making it likely that at least one of the treatments will make a major difference in how we will treat CF in the future.
总之，囊性纤维化（CF）患者的肺部表现与营养状况之间存在显著的相互作用。过去20年中CF临床护理的进展主要归功于抗感染治疗以及积极的营养管理。目前，有多种治疗药物正处于临床试验阶段，这些药物要么针对潜在的CFTR缺陷，要么针对CFTR的下游效应。正在研究的治疗药物种类繁多，以及针对潜在CFTR缺陷的治疗进展令人振奋，这使得至少有一种治疗方法很可能会在未来我们治疗CF的方式上产生重大影响。
