BMD and body composition in children and young patients affected by cystic fibrosis
BMD and body composition in children and young patients affected by cystic fibrosis
复制标题
DOI：
10.1359/jbmr.051023
复制
发表时间：
2006-03-01
影响因子：
6.2
通讯作者：
Colombo, C
中科院分区：
文献类型：
--
作者：
Bianchi, ML;Romano, G;Colombo, C
囊性纤维化患者较长的生存期导致了更多的骨并发症。136名年轻患者被研究了12-24个月。66%的患者骨密度低。脂肪量和瘦肉量也减少了。肺功能受损和类固醇总剂量对骨骼的负面影响最大。导读：据报道，低骨密度是囊性纤维化（CF）成人患者的常见并发症，但现有数据在年轻患者中不太一致。材料和方法：本研究旨在纵向评估136例年轻患者（3-24岁）12-24个月的骨密度，并调查其主要决定因素。同时对BMC和体成分进行评价。结果：66%的患者脊柱和全身骨密度（以Z评分表示）降低。低骨密度的患病率在儿童、青少年和年轻人中是相同的。青春期血小板计数和骨密度的主要决定因素是用力呼气量（FEV1，作为肺功能的一项指标）。（作为门静脉高压症的指标）和累积类固醇剂量。FEV1随时间的变化影响BMD的变化。CF患者全身和亚区域（躯干、四肢）的骨量、脂肪量（FM）和无脂（瘦）质量（FFM）均降低。瘦质量影响骨密度。全身和下肢，而脂肪量（和BMI）影响脊柱骨密度。FEV1也影响FFM。结论：低骨密度存在于相当比例的CF患者中，与性别和年龄无关。骨密度取决于肺功能、类固醇剂量和晚期肝病的存在。随着时间的推移，肺功能和青春期是骨密度增加的主要刺激因素。CIF也改变了机体成分，FFM受肺功能的影响。
Longer survival in cystic fibrosis has led to more bone complications. One hundred thirty-six young patients were studied for 12-24 months. Low BMD was found in 66%. Fat mass and lean mass were also reduced. Impaired pulmonary function and total steroid dose had the greatest negative influence on bone.Introduction: Low BMD is reported as a frequent complication in adult patients affected by cystic fibrosis (CF), but the available data are less consistent for younger patients.Materials and Methods: This study was designed to evaluate BMD longitudinally over 12-24 months in a sample of 136 young patients (3-24 years of age) and to investigate its major determinants. BMC and body composition were also evaluated.Results: BMD (expressed as Z score) of spine and of total body was reduced in 66% of patients. The prevalence of low BMD was the same in children, adolescents, and young adults. The main determinants of platelet count puberty, BMD were forced expiratory volume in 1s (FEV1; as an of pulmonary function). (as an index of portal hypertension), and cumulative steroid dose. Changes of FEV1 over time influenced BMD changes. Bone mass, fat mass (FM) and fat-free (lean) mass (FFM) were reduced in CF patients at both total body and subregions (trunk, limbs). Lean mass influenced BMD of. total body and lower limbs, whereas fat mass (and BMI) influenced spine BMD. FEV1 also influenced FFM.Conclusions: Low BMD was present in a significant proportion of CF patients, independent of sex and age. BMD depended oil pulmonary function, steroid dose, and presence of advanced liver disease. Pulmonary function and puberty were the main stimuli for the increase of BMD over time. CIF also altered body composition, and FFM was influenced by pulmonary function.