Kalnins Daina, Wilschanski Michael
Clinical Dietetics, Respiratory Medicine, The Hospital for Sick Children, Toronto, ON, Canada.
Drug Des Devel Ther. 2012;6:151-61. doi: 10.2147/DDDT.S9258. Epub 2012 Jun 20.
Poor clinical outcomes in cystic fibrosis are often associated with undernutrition. Normal growth and development should be achieved in cystic fibrosis, and nutritional counseling is paramount at all ages. Prevention and early detection of growth failure is the key to successful nutritional intervention. The advance in nutritional management is certainly one factor that has contributed to the improved survival in recent decades. This review outlines the major nutritional parameters in the management of the patient with cystic fibrosis, including recent advances in pancreatic enzyme replacement therapy and fat-soluble vitamin therapy. There are sections on complicated clinical situations which directly affect nutrition, for example, before and after lung transplantation, cystic fibrosis-related diabetes, and bone health.
囊性纤维化患者不佳的临床预后常与营养不良相关。囊性纤维化患者应实现正常的生长发育，营养咨询在各个年龄段都至关重要。预防和早期发现生长发育迟缓是成功进行营养干预的关键。营养管理的进步无疑是近几十年来患者生存率提高的一个因素。本综述概述了囊性纤维化患者管理中的主要营养参数，包括胰酶替代疗法和脂溶性维生素疗法的最新进展。文中还设有章节讨论直接影响营养状况的复杂临床情况，例如肺移植前后、囊性纤维化相关糖尿病以及骨骼健康等。
