Pediatric Pulmonary Institute and CF Center, Rappaport Children's Hospital, Rambam Health Care Campus, Haifa, Israel.
Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa, Israel.
Pediatr Pulmonol. 2020 Oct;55(10):2667-2673. doi: 10.1002/ppul.24925. Epub 2020 Jul 3.
Multiple factors affect bone mineral density (BMD) in cystic fibrosis (CF). Our aim was to perform comprehensive analyses of parameters potentially contributing to BMD.
A prospective single-center study assessing BMD, and correlations with multiple parameters including pancreatic status, lung functions, 6-minute walk test (6MWT), clinical score (modified Shwachman-Kulczycki [SK] score), vitamin D, nutritional intake, hand grip strength (HGS), habitual physical activity (smart watches), and quality of life (SF-36 questionnaire).
Forty CF patients, mean age 18.3 ± 8.1 years, forced expiratory volume in 1 second 74.7% ± 17.9% predicted. Fifteen (37.5%) and 11 (27.5%) had osteopenia and osteoporosis, respectively. BMD was similar in pancreatic sufficient (pancreatic sufficient [PS], n = 15) and insufficient (pancreatic insufficient [PI], n = 25); median hip z score -1.5 ((-2.7)-(+0.2)) vs -1.5 ((-3.5)-(+0.7)), P = .79; spine -0.8 ((-2.2)-(+2)) vs -1.2 ((-4.4)-(+1.5)), P = .39 in PS vs PI, respectively. BMD correlated with HGS (r = .72, P < .001 hip; r = .52, P = .001 spine) and fat-free mass index (r = .81, P < .001 hip; r = .63, P < .001 spine). BMD z score correlated weakly with SK score and moderately with SF-36 general health. Data from smart watches, nutrition questionnaires, and 6MWT did not correlate with BMD. In a multivariate model, age and SK score predicted spine z score BMD.
A substantial number of CF patients have low BMD. Similar rates in PS and PI suggest that other factors, such as disease severity, may contribute to low BMD. SK and age, which can easily be obtained even with limited resources, were the best predictors of low BMD. Further larger multicenter studies are warranted to evaluate the contribution of multifactorial etiologies to low BMD in CF.
多种因素会影响囊性纤维化(CF)患者的骨矿物质密度(BMD)。本研究旨在对可能影响 BMD 的多种参数进行综合分析。
前瞻性单中心研究评估了 BMD,并与多个参数相关联,包括胰腺状态、肺功能、6 分钟步行测试(6MWT)、临床评分(改良的 Shwachman-Kulczycki [SK] 评分)、维生素 D、营养摄入、手握力(HGS)、习惯性体力活动(智能手表)和生活质量(SF-36 问卷)。
40 名 CF 患者,平均年龄 18.3±8.1 岁,第一秒用力呼气量占预计值的 74.7%±17.9%。15 名(37.5%)和 11 名(27.5%)患者分别患有骨质疏松和骨质疏松症。胰腺功能充足(胰腺功能充足 [PS],n=15)和不足(胰腺功能不足 [PI],n=25)的 BMD 相似;髋部 z 评分中位数-1.5(-2.7-0.2)vs-1.5(-3.5-0.7),P=0.79;脊柱-0.8(-2.2-2)vs-1.2(-4.4-1.5),P=0.39,分别在 PS 与 PI 之间。BMD 与 HGS(r=0.72,P<0.001 髋部;r=0.52,P=0.001 脊柱)和无脂肪质量指数(r=0.81,P<0.001 髋部;r=0.63,P<0.001 脊柱)相关。BMD z 评分与 SK 评分弱相关,与 SF-36 总体健康中度相关。智能手表、营养问卷和 6MWT 的数据与 BMD 不相关。在多变量模型中,年龄和 SK 评分预测脊柱 z 评分 BMD。
相当数量的 CF 患者存在低 BMD。PS 和 PI 中的相似比率表明,其他因素(如疾病严重程度)可能导致低 BMD。SK 和年龄,即使在资源有限的情况下也很容易获得,是低 BMD 的最佳预测指标。需要进一步进行更大规模的多中心研究,以评估多因素病因对 CF 中低 BMD 的贡献。