Moustaki Maria, Loukou Ioanna, Priftis Kostas N, Douros Konstantinos
Maria Moustaki, Ioanna Loukou, Cystic Fibrosis Unit, "Aghia Sophia" Children's Hospital, 11527 Athens, Greece.
World J Clin Pediatr. 2017 Aug 8;6(3):132-142. doi: 10.5409/wjcp.v6.i3.132.
Bronchiectasis is usually classified as cystic fibrosis (CF) related or CF unrelated (non-CF); the latter is not considered an orphan disease any more, even in developed countries. Irrespective of the underlying etiology, bronchiectasis is the result of interaction between host, pathogens, and environment. Vitamin D is known to be involved in a wide spectrum of significant immunomodulatory effects such as down-regulation of pro-inflammatory cytokines and chemokines. Respiratory epithelial cells constitutively express 1α-hydroxylase leading to the local transformation of the inactive 25(OH)-vitamin D to the active 1,25(OH)-vitamin D. The latter through its autocrine and paracrine functions up-regulates vitamin D dependent genes with important consequences in the local immunity of lungs. Despite the scarcity of direct evidence on the involvement of vitamin D deficiency states in the development of bronchiectasis in either CF or non-CF patients, it is reasonable to postulate that vitamin D may play some role in the pathogenesis of lung diseases and especially bronchiectasis. The potential contribution of vitamin D deficiency in the process of bronchiectasis is of particular clinical importance, taking into consideration the increasing prevalence of vitamin D deficiency worldwide and the significant morbidity of bronchiectasis. Given the well-established association of vitamin D deficiency with increased inflammation, and the indicative evidence for harmful consequences in lungs, it is intriguing to speculate that the administration of vitamin D supplementation could be a reasonable and cost effective supplementary therapeutic approach for children with non-CF bronchiectasis. Regarding CF patients, maybe in the future as more data become available, we have to re-evaluate our policy on the most appropriate dosage scheme for vitamin D.
支气管扩张通常分为与囊性纤维化（CF）相关或与CF无关（非CF）；后者即使在发达国家也不再被视为罕见病。无论潜在病因如何，支气管扩张都是宿主、病原体和环境之间相互作用的结果。已知维生素D具有广泛的重要免疫调节作用，如下调促炎细胞因子和趋化因子。呼吸道上皮细胞组成性表达1α-羟化酶，导致无活性的25（OH）-维生素D在局部转化为活性的1,25（OH）-维生素D。后者通过其自分泌和旁分泌功能上调维生素D依赖性基因，对肺部局部免疫产生重要影响。尽管缺乏关于维生素D缺乏状态在CF或非CF患者支气管扩张发展中作用的直接证据，但推测维生素D可能在肺部疾病尤其是支气管扩张的发病机制中发挥一定作用是合理的。考虑到全球维生素D缺乏患病率的上升以及支气管扩张的高发病率，维生素D缺乏在支气管扩张过程中的潜在作用具有特别的临床重要性。鉴于维生素D缺乏与炎症增加之间已确立的关联以及肺部有害后果的指示性证据，推测补充维生素D可能是一种合理且具有成本效益的非CF支气管扩张患儿辅助治疗方法很有意思。对于CF患者，也许未来随着更多数据的出现，我们必须重新评估我们关于维生素D最合适剂量方案的政策。
