Pediatric Cystic Fibrosis Center, Hospices Civils de Lyon, Lyon, France.
University Claude-Bernard Lyon 1, Lyon, France.
Osteoporos Int. 2017 Nov;28(11):3179-3188. doi: 10.1007/s00198-017-4179-9. Epub 2017 Aug 9.
We investigated whether bone microstructure assessed by high-resolution peripheral quantitative tomography (HR-pQCT) could be altered in children and teenagers with cystic fibrosis (CF). In comparison to their healthy counterparts, bone microstructure was mildly affected at the tibial level only.
Cystic fibrosis-related bone disease (CFBD) may alter bone health, ultimately predisposing patients to bone fractures. Our aim was to assess bone microstructure using high-resolution peripheral quantitative tomography (HR-pQCT) in a cohort of children and teenagers with CF in comparison to age-, puberty-, and gender-matched healthy volunteers (HVs).
In this single-center, prospective, cross-sectional study, we evaluated the HR-pQCT bone parameters of CF patients and compared them to those of the healthy volunteers.
At a median age of 15.4 [range, 10.5-17.9] years, 37 CF patients (21 boys) with 91% [range, 46-138%] median forced expiratory volume in 1 s were included. At the ultradistal tibia, CF patients had a smaller bone cross-sectional area (579 [range, 399-1087] mm) than HVs (655 [range, 445-981] mm) (p = 0.027), related to a decreased trabecular area, without any significant differences for height. No other differences were found (trabecular number, separation, thickness, or distribution) at the radial or tibial levels. Bone structure was different in patients receiving ursodeoxycholic acid and those bearing two F508del mutations.
In our cohort of children and teenagers with good nutritional and lung function status, bone microstructure evaluated with HR-pQCT was not severely affected. Minimal microstructure abnormalities observed at the tibial level may be related to the cystic fibrosis transmembrane conductance regulator defect alone; the long-term consequences of such impairment will require further evaluation.
我们旨在使用高分辨率外周定量计算机断层扫描（HR-pQCT）评估囊性纤维化（CF）患儿和青少年的骨微结构，并将其与年龄、青春期和性别匹配的健康对照者（HCs）进行比较。
在这项单中心、前瞻性、横断面研究中，我们评估了 CF 患者的 HR-pQCT 骨参数，并将其与 HCs 进行了比较。
在中位年龄为 15.4 岁（范围，10.5-17.9 岁）的 37 例 CF 患者（21 名男性）中，有 91%（范围，46-138%）的患者有中位数用力呼气量 1 秒（FEV1）。在超远端胫骨处，CF 患者的骨横截面积（579 [范围，399-1087] mm）小于 HCs（655 [范围，445-981] mm）（p = 0.027），这与骨小梁面积减少有关，而身高无显著差异。在桡骨或胫骨水平未发现其他差异（骨小梁数量、分离、厚度或分布）。接受熊去氧胆酸治疗的患者和携带两个 F508del 突变的患者的骨结构不同。
在我们的儿童和青少年队列中，营养和肺功能状况良好，使用 HR-pQCT 评估的骨微结构未受到严重影响。在胫骨水平观察到的微小微结构异常可能仅与囊性纤维化跨膜电导调节因子缺陷有关；这种损害的长期后果需要进一步评估。
