Research Institute of McGill University Health Centre, Montreal, Quebec, Canada.
Research Institute of McGill University Health Centre, Montreal, Quebec, Canada; Division of Pediatric Respiratory Medicine, Department of Pediatrics, Montreal Children's Hospital-McGill University Health Centre.
Paediatr Respir Rev. 2014 Jun;15(2):154-62. doi: 10.1016/j.prrv.2013.11.002. Epub 2013 Nov 14.
Cystic fibrosis (CF) is characterized by chronic infection and inflammation in the airways that lead to progressive lung damage and early death. Current anti-inflammatory therapies are limited by extensive adverse effects or insufficient efficacy. There is a large body of studies indicating beneficial anti-microbial and anti-inflammatory properties of vitamin D. Since most patients with CF present with vitamin D deficiency, and serum vitamin D levels demonstrate a positive correlation with lung function and negative correlation with airway inflammation and infection, correcting vitamin D deficiency may be an attractive therapeutic strategy in CF. The function of vitamin D is intricately tied to its metabolism, which may be impaired at multiple steps in patients with CF, with a potential to limit the efficacy of vitamin D supplementation. It is likely that the aforementioned beneficial properties of vitamin D require supplementation with doses of vitamin D markedly higher than those recommended to maintain proper bone function. This review will illustrate the potential for supplementation with vitamin D or its metabolites to modulate inflammation and improve defence against chronic infection in CF lung, as well as appropriate vitamin D supplementation strategies for improving lung function in CF.
囊性纤维化（CF）的特征是气道中的慢性感染和炎症，导致进行性肺损伤和早逝。目前的抗炎治疗受到广泛的不良反应或疗效不足的限制。有大量研究表明维生素 D 具有有益的抗微生物和抗炎特性。由于大多数 CF 患者存在维生素 D 缺乏，血清维生素 D 水平与肺功能呈正相关，与气道炎症和感染呈负相关，因此纠正维生素 D 缺乏可能是 CF 的一种有吸引力的治疗策略。维生素 D 的功能与其代谢密切相关，在 CF 患者中，其代谢可能在多个步骤受到损害，从而限制了维生素 D 补充的疗效。很可能需要补充维生素 D 或其代谢物来调节炎症并改善 CF 肺部对慢性感染的防御，以发挥上述有益的维生素 D 特性，同时还需要制定适当的维生素 D 补充策略来改善 CF 患者的肺功能。
