Normal bone mineral content but unfavourable muscle/fat ratio in Klinefelter syndrome
Normal bone mineral content but unfavourable muscle/fat ratio in Klinefelter syndrome
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DOI：
10.1136/adc.2007.120675
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发表时间：
2008-01-01
影响因子：
5.2
通讯作者：
Juul, A.
中科院分区：
文献类型：
--
作者：
Aksglaede, L.;Molgaard, C.;Juul, A.
目的：评价儿童和青少年Klinefelter综合征（KS）的体成分和骨矿物质含量（BMC）。设计：回顾性横断面研究。地点：哥本哈根大学医院三级内分泌诊所。患者：18名未经治疗的KS男孩和6名接受雄激素替代治疗的KS男孩参加了这项研究，中位年龄为11.0岁（范围4.3-18.6）。干预：分析双能x线吸收测量和人体测量。主要结局指标：比较治疗和未治疗的KS男孩腰部和全身BMC、瘦体重（LBM）、体脂质量（BFM）、体脂百分比（BF%）、身高和体重指数（BMI），并与正常年龄匹配的男孩进行比较。结果：LBM（未经治疗-0.3（-2.4 ~ +2.1）和治疗+1.1(-1.6 ~ +2.1)）正常，BFM(未经治疗+0.5 （-1.0 ~ +2.3),p = 0.02和治疗+1.6 (-0.2 ~ +2.4),p =0.01）显著升高，均以标准差评分表示。腰椎骨密度（BMD：未治疗-0.4（-3.1至+0.9）和治疗+1.0(-1.4至+3.0)）和全身BMC（未治疗+0.1（-1.8至+3.3）和治疗+1.5(-1.1至+2.5)）正常。结论：我们发现尽管LBM正常，但BFM和BF%明显增加，表明存在不利的肌脂比。腰椎骨密度及全身骨密度正常。这些发现表明，在成人KS中看到的不利代谢特征可能已经存在于儿童时期，如脂肪量增加所证明的那样，而报道的低骨密度似乎是在青春期后发展起来的。
Objective: To evaluate body composition and bone mineral content (BMC) in children and adolescents with Klinefelter syndrome (KS).Design: Retrospective cross-sectional study.Setting: Tertiary endocrine clinic at the University Hospital, Copenhagen.Patients: Eighteen untreated boys with KS and six boys with KS receiving androgen substitution with a median age of 11.0 years (range 4.3-18.6) participated in the study.Intervention: Dual energy x ray absorptiometry and anthropometric measurements were analysed.Main outcome measures: Lumbar and whole body BMC, lean body mass (LBM), body fat mass (BFM), body fat percentage (BF%), height and body mass index (BMI) were compared between treated and untreated boys with KS and compared to normal age-matched boys.Results: LBM (untreated -0.3 (-2.4 to +2.1) and treated +1.1 (-1.6 to +2.1)) was normal, while BFM (untreated +0.5 (-1.0 to +2.3), p = 0.02 and treated +1.6 (-0.2 to +2.4), p =0.01) was significantly increased, all expressed as standard deviation scores. Lumbar bone mineral density (BMD; untreated -0.4 (-3.1 to +0.9) and treated +1.0 (-1.4 to +3.0)) and whole body BMC (untreated +0.1 (-1.8 to +3.3) and treated +1.5 (-1.1 to +2.5)) were normal.Conclusion: We found significantly increased BFM and BF% despite normal LBM, suggesting the presence of an unfavourable muscle/ fat ratio. Lumbar BMD and whole body BMC were normal. These findings suggest that the unfavourable metabolic profile seen in adult KS may already be present in childhood as evidenced by the increased fat mass, whereas the reported low BMD seems to develop after puberty.