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囊性纤维化患者骨质减少的相关因素

Correlates of osteopenia in patients with cystic fibrosis.

作者信息

Bhudhikanok G S, Lim J, Marcus R, Harkins A, Moss R B, Bachrach L K

机构信息

Department of Pediatrics, Stanford University School of Medicine, California, USA.

出版信息

Pediatrics. 1996 Jan;97(1):103-11.

PMID:8545201
Abstract

OBJECTIVE

As the expected survival improves for individuals with cystic fibrosis, these individuals face myriad medical complications. The goals of this study were to examine the prevalence of osteopenia in children and adults with cystic fibrosis and to elucidate the risk factors associated with deficits in bone mineral.

METHODOLOGY

We compared bone mineral levels in 49 patients (30 female and 19 male) ages 8 through 48 years with those of age- and sex-matched control subjects. Lumbar spine, femoral neck, and whole-body bone mineral were measured by dual-energy radiographic absorptiometry and expressed in terms of bone mineral content, areal bone density (BMD), and bone mineral apparent density (BMAD), which corrects for differences in bone size. Clinical variables were evaluated as potential correlates of bone mineral.

RESULTS

Patients with cystic fibrosis had significantly less bone mineral than did control subjects at all sites using all expressions of bone mass. Mean BMD z scores were -1.7 (lumbar spine), -1.9 (femoral neck), and -1.2 (whole body). BMAD z scores also were significantly low for age and gender. Twenty-six of the 49 patients (8 adolescents) had significant osteopenia according to their BMD z scores; 14 of the 45 patients (5 adolescents) with available BMAD z scores had significantly low values at one or more sites. Age, pubertal stage, body mass, caloric expenditure, illness severity, glucocorticoid therapy, and gonadal function predicted bone mineral status. Serum parathyroid hormone and calcium, carbohydrate intake, and weight-bearing activity had limited predictive value. Daily calcium intake and cystic fibrosis transmembrane regulator genotype did not predict bone mineral status.

CONCLUSIONS

Osteopenia is common at all ages in cystic fibrosis, suggesting that inadequate bone mineral accretion as well as increased bone loss contribute to the deficits in bone mineral observed. Several clinical factors seem to contribute to these deficits.

摘要

目的

随着囊性纤维化患者预期生存期的延长,这些患者面临着众多医学并发症。本研究的目的是调查儿童和成人囊性纤维化患者中骨质减少的患病率,并阐明与骨矿物质缺乏相关的危险因素。

方法

我们将49例年龄在8至48岁之间的患者(30例女性和19例男性)的骨矿物质水平与年龄和性别匹配的对照受试者进行比较。通过双能X线吸收法测量腰椎、股骨颈和全身骨矿物质,并以骨矿物质含量、面积骨密度(BMD)和骨矿物质表观密度(BMAD)表示,后者校正了骨大小的差异。评估临床变量作为骨矿物质的潜在相关因素。

结果

使用所有骨量表达方式,囊性纤维化患者在所有部位的骨矿物质均明显少于对照受试者。平均BMD z评分分别为-1.7(腰椎)、-1.9(股骨颈)和-1.2(全身)。BMAD z评分按年龄和性别也显著降低。根据BMD z评分,49例患者中有26例(8例青少年)存在明显的骨质减少;在45例有可用BMAD z评分的患者中,14例(5例青少年)在一个或多个部位的值显著降低。年龄、青春期阶段、体重、热量消耗、疾病严重程度、糖皮质激素治疗和性腺功能可预测骨矿物质状态。血清甲状旁腺激素和钙、碳水化合物摄入量和负重活动的预测价值有限。每日钙摄入量和囊性纤维化跨膜调节因子基因型不能预测骨矿物质状态。

结论

骨质减少在囊性纤维化患者的所有年龄段都很常见,这表明骨矿物质积累不足以及骨丢失增加导致了观察到的骨矿物质缺乏。几个临床因素似乎导致了这些缺乏。

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