Service de Pédiatrie, Pneumologie, Allergologie, Mucoviscidose, Hôpital Femme Mère Enfant, 59 boulevard Pinel, Bron cedex, France.
Pediatr Radiol. 2010 Mar;40(3):301-8. doi: 10.1007/s00247-009-1446-8. Epub 2009 Dec 3.
With the increased life span of cystic fibrosis (CF) patients, CF-related bone diseases could have an increased prevalence and morbidity in this group. In children, previous retrospective and prospective studies have yielded conflicting results on bone mineralization.
To monitor body composition and bone mineral status of children with CF.
We reviewed the dual-energy X-ray absorptiometry (DXA) data of 161 children with CF (age 10 +/- 4.8 years). Total body bone mineral content (BMCt), total lean tissue mass (LTMt) and total fat mass (FMt) were measured and compared to expected data calculated from ideal weight for height (Wi; e.g. BMCti, LTMti, FMti). The bt (BMCt/BMCti), lt (LTMt/LTMti) and ft (FMt/FMti) ratios were used as quantitative variables.
Low bt ratio was found at all ages (mean bt ratio 0.94 +/- 0.10; P < 0.001), even in children <6 years of age. However, the children's BMCt was satisfactorily adapted to their weight. lt and ft ratios were not constant across age groups. Children <10 years had 8% reduction of their lt ratio, maintaining normal levels thereafter. The opposite trend was found for ft ratio. Poor clinical, nutritional status and vitamin A levels were correlated with bt and lt ratios.
Our results indicate that children with CF could have early alterations in their bone status and that lt and ft ratios did not have constant values across ages. Interpreting DXA data using this approach is suitable in children with CF.
随着囊性纤维化（CF）患者寿命的延长，CF 相关骨病在该人群中的患病率和发病率可能会增加。在儿童中，先前的回顾性和前瞻性研究对骨矿化得出了相互矛盾的结果。
监测 CF 患儿的身体成分和骨矿物质状况。
我们回顾了 161 名 CF 患儿（年龄 10 ± 4.8 岁）的双能 X 射线吸收法（DXA）数据。测量了全身骨矿物质含量（BMCt）、全身瘦组织量（LTMt）和全身脂肪量（FMt），并与身高理想体重（Wi）计算的预期数据进行比较（例如 BMCti、LTMti、FMti）。bt（BMCt/BMCti）、lt（LTMt/LTMti）和 ft（FMt/FMti）比值用作定量变量。
在所有年龄段均发现低 bt 比值（平均 bt 比值 0.94 ± 0.10；P < 0.001），即使在<6 岁的儿童中也是如此。然而，儿童的 BMCt 与其体重相适应。lt 和 ft 比值在不同年龄组之间并不恒定。<10 岁的儿童其 lt 比值减少 8%，此后保持正常水平。ft 比值则呈现相反的趋势。较差的临床、营养状况和维生素 A 水平与 bt 和 lt 比值相关。
我们的结果表明，CF 患儿可能存在早期骨状况改变，且 lt 和 ft 比值在不同年龄组之间没有恒定值。使用这种方法解释 DXA 数据适用于 CF 患儿。
