Vitamin D deficiency is a common finding in individuals with cystic fibrosis (CF), despite routine supplementation. Hypovitaminosis D is often the result of fat malabsorption, but other contributors include increased latitude, poor nutritional intake, decreased sun exposure, impaired hydroxylation of vitamin D, and non-adherence to the prescribed vitamin D regimen. Vitamin D is critical for calcium homeostasis and optimal skeletal health, and vitamin D deficiency in CF can lead to skeletal complications of osteopenia and osteoporosis. Over time, our understanding of treatment regimens for vitamin D deficiency in CF has evolved, leading to recommendations for higher doses of vitamin D to achieve target levels of circulating 25-hydroxyvitamin D. There is also some evidence that vitamin D deficiency may have non-skeletal consequences such as an increase in pulmonary exacerbations. The exact mechanisms involved in the non-skeletal complications of vitamin D deficiency are not clearly understood, but may involve the innate immune system. Future clinical studies are needed to help address whether vitamin D has a role in CF beyond skeletal health.

译文

尽管常规补充,维生素d缺乏症是囊性纤维化 (CF) 患者的常见发现。维生素d缺乏通常是脂肪吸收不良的结果,但其他因素包括纬度增加,营养摄入差,日晒减少,维生素d的羟基化受损以及不遵守规定的维生素d方案。维生素d对于钙稳态和最佳骨骼健康至关重要,CF中的维生素d缺乏会导致骨质减少和骨质疏松症的骨骼并发症。随着时间的推移,我们对CF中维生素d缺乏症的治疗方案的理解不断发展,导致建议使用更高剂量的维生素d来达到循环25-羟基维生素d的目标水平。还有一些证据表明,维生素d缺乏可能会导致非骨骼后果,例如肺加重的增加。维生素d缺乏的非骨骼并发症的确切机制尚不清楚,但可能涉及先天免疫系统。未来的临床研究需要帮助解决维生素d是否在骨骼健康之外的CF中发挥作用。

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