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囊性纤维化性骨病的治疗:现有知识和未来方向。

Cystic fibrosis bone disease treatment: Current knowledge and future directions.

机构信息

Divisions of Endocrinology, Boston Children's Hospital and Massachusetts General Hospital, Boston, MA, USA.

Department of Internal Medicine, Division of Endocrinology, Diabetes and Metabolism, University of Kansas Medical Center, Kansas City, KS, USA.

出版信息

J Cyst Fibros. 2019 Oct;18 Suppl 2:S56-S65. doi: 10.1016/j.jcf.2019.08.017.

DOI:10.1016/j.jcf.2019.08.017
PMID:31679730
Abstract

Bone disease is a frequent complication in adolescents and adults with cystic fibrosis (CF). Early detection and monitoring of bone mineral density and multidisciplinary preventive care are necessary from childhood through adolescence to minimize CF-related bone disease (CFBD) in adult CF patients. Approaches to optimizing bone health include ensuring adequate nutrition, particularly intake of calcium and vitamins D and K, addressing other secondary causes of low bone density such as hypogonadism, encouraging weight bearing exercise, and avoiding bone toxic medications. Of the currently available anti-resorptive or anabolic osteoporosis medications, only bisphosphonates have been studied in individuals with CF. Future studies are needed to better understand the optimal approach for managing CFBD.

摘要

骨骼疾病是囊性纤维化(CF)青少年和成人的常见并发症。从儿童期到青春期,需要早期检测和监测骨矿物质密度,并进行多学科预防保健,以最大限度地减少成年 CF 患者的 CF 相关骨骼疾病(CFBD)。优化骨骼健康的方法包括确保充足的营养,特别是钙、维生素 D 和 K 的摄入,解决其他导致骨密度降低的继发性原因,如性腺功能减退,鼓励负重运动,避免使用有骨骼毒性的药物。在目前可用的抗吸收或合成代谢骨质疏松症药物中,只有双磷酸盐类药物在 CF 患者中进行了研究。需要进一步的研究来更好地了解管理 CFBD 的最佳方法。

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