Neri A S, Lori I, Festini F, Masi L, Brandi M L, Galici V, Braggion C, Taccetti G
Cystic Fibrosis Center of Tuscany, Meyer Hospital, Florence, Italy.
Minerva Pediatr. 2008 Apr;60(2):147-54.
The increase in life expectancy of cystic fibrosis (CF) patients has brought about a rise in new clinical problems in these patients, such as a decrease in bone mineral density (BMD). The cause of diminished BMD in CF is multi-factorial.
The aim of this cross-sectional study, conducted on 39 CF patients under the age of 18 years, was to evaluate the degree of bone mineralization and the prevalence of low BMD in these patients during a follow-up at the Cystic Fibrosis Regional Center of Tuscany, using a dual energy X-ray absorptiometry (DXA) scan, and to then study the factors correlated with low BMD.
Areas BMD values (g/cm2) and Z-score values were determined. Eighteen patients (46%) out of the our sample had decreased BMD, while 21 patients (54%) had normal values. A statistically significant association was found between BMD Z-score values and pancreatic insufficiency, BMI<5th percentile and DeltaF508 homozygosis. Subjects treated with oral steroid therapy had a 3.9 times greater risk of developing osteoporosis compared to non-treated subjects (95% C.I.: 1.07-22.6; R.R. 4.9). An association was found between BMD Z-score values and FEV1 values (r=0.29; P=0.06), physical activity total score values (r=0.22; P=0.19) and the Chrispin-Norman chest radiographic score (r=-0.31; P=0.06).
Early identification of reduced bone mass values would permit early intervention to prevent the development of osteoporosis. Maintaining pulmonary function, guaranteeing optimal nutritional status, following an adequate program of physical activity and controlling steroid intake could maintain BMD over time.
囊性纤维化（CF）患者预期寿命的增加导致这些患者出现了新的临床问题，如骨矿物质密度（BMD）降低。CF患者BMD降低的原因是多因素的。
这项横断面研究对39名18岁以下的CF患者进行，目的是在托斯卡纳囊性纤维化区域中心的随访期间，使用双能X线吸收测定法（DXA）扫描评估这些患者的骨矿化程度和低BMD的患病率，然后研究与低BMD相关的因素。
测定了各区域的BMD值（g/cm²）和Z评分值。我们样本中的18名患者（46%）BMD降低，而21名患者（54%）值正常。发现BMD Z评分值与胰腺功能不全、BMI<第5百分位数和ΔF508纯合性之间存在统计学上的显著关联。接受口服类固醇治疗的受试者患骨质疏松症的风险是非治疗受试者的3.9倍（95%置信区间：1.07 - 22.6；相对风险4.9）。发现BMD Z评分值与FEV1值（r = 0.29；P = 0.06）、体力活动总分值（r = 0.22；P = 0.19）和克里斯平 - 诺曼胸部X线评分（r = -0.31；P = 0.06）之间存在关联。
早期识别骨量降低值将有助于早期干预以预防骨质疏松症的发生。随着时间的推移，维持肺功能、保证最佳营养状况、遵循适当的体力活动计划以及控制类固醇摄入量可以维持BMD。
