Putman Melissa S, Baker Joshua F, Uluer Ahmet, Herlyn Karen, Lapey Allen, Sicilian Leonard, Tillotson Angela Pizzo, Gordon Catherine M, Merkel Peter A, Finkelstein Joel S
Endocrine Unit, Department of Medicine, Massachusetts General Hospital, Boston, MA, United States; Division of Endocrinology, Boston Children's Hospital, Boston, MA, United States.
Division of Rheumatology, University of Pennsylvania, Philadelphia, PA, United States.
J Cyst Fibros. 2015 Jul;14(4):526-32. doi: 10.1016/j.jcf.2015.01.011. Epub 2015 Feb 16.
Improvements in clinical care have led to increased life expectancy in patients with cystic fibrosis (CF) over the past several decades. Whether these improvements have had significant effects on bone health in patients with CF is unclear.
This is a cross-sectional study comparing clinical characteristics and bone mineral density (BMD) measured by dual energy X-ray absorptiometry (DXA) in adults with CF evaluated in 1995-1999 to age-, race-, and gender-matched patients with CF evaluated in 2011-2013 at the same center on calibrated DXA machines.
The cohorts were similar in terms of age, BMI, pancreatic insufficiency, presence of F508del mutation, and reproductive history. In the most recent cohort, pulmonary function was superior, and fewer patients had vitamin D deficiency or secondary hyperparathyroidism. Areal BMD measures of the PA spine, lateral spine, and distal radius were similarly low in the two cohorts.
Although pulmonary function and vitamin D status were better in patients in the present-day cohort, areal BMD of the spine was reduced in a significant number of patients and was no different in patients with CF today than in the late 1990s. Further attention to optimizing bone health may be necessary to prevent CF-related bone disease.
在过去几十年中,临床护理的改善使囊性纤维化(CF)患者的预期寿命有所延长。目前尚不清楚这些改善是否对CF患者的骨骼健康产生了显著影响。
这是一项横断面研究,比较了1995年至1999年接受评估的成年CF患者与2011年至2013年在同一中心使用校准后的双能X线吸收仪(DXA)进行评估的年龄、种族和性别匹配的CF患者的临床特征和骨密度(BMD)。
两组患者在年龄、体重指数、胰腺功能不全、F508del突变的存在情况以及生育史方面相似。在最近的队列中,肺功能更好,维生素D缺乏或继发性甲状旁腺功能亢进的患者更少。两组患者的腰椎正位、腰椎侧位和桡骨远端的面积骨密度测量值同样较低。
尽管当今队列中的患者肺功能和维生素D状况更好,但仍有相当数量的患者脊柱面积骨密度降低,且如今CF患者的这一情况与20世纪90年代末相比并无差异。可能需要进一步关注优化骨骼健康,以预防与CF相关的骨病。