Bone mineral, histomorphometry, and body composition in adults with growth hormone receptor deficiency

Bone mineral, histomorphometry, and body composition in adults with growth hormone receptor deficiency
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DOI:
10.1359/jbmr.1998.13.3.415
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发表时间:
1998-03-01
影响因子:
6.2
通讯作者:
Guevara-Aguirre, J
Guevara-Aguirre, J
中科院分区:
医学1区
文献类型:
--
作者:
Bachrach, LK;Marcus, R;Guevara-Aguirre, J

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生长激素(GH)和胰岛素样生长因子I (IGF-I)缺乏与儿童和成人的骨质减少有关。为了研究生长激素抵抗对骨矿物质和身体成分的影响,我们研究了来自厄瓜多尔南部的11名(平均年龄30岁)生长激素受体缺乏(GHRD, Laron综合征)的成年人和11名年龄和性别匹配的对照组。采用双能x线吸收仪测定骨矿物质和体成分。用骨转换生化指标和动态骨组织形态测定法评估骨生理学,GHRD患者的骨大小和体组成与对照组有明显差异,GHRD患者比对照组矮40厘米,瘦体重明显减少,体脂百分比增加,脊柱、股骨颈和全身的骨矿物质含量和密度(BMD)明显低于对照组。受影响的女性所有部位的平均BMD Z评分为-1.5至-1.6,患有GHRD的男性为-2.2至-2.3。然而,GHRD患者脊柱和股骨颈的估计体积骨密度(BMAD)并未降低。女性GHRD患者脊柱BMAD为0.210 +/- 0.025,对照组为0.177 +/- 0.021 (p < 0.05);男性GHRD患者脊柱BMAD为0.173 +/- 0.018,对照组为0.191 +/- 0.025 (p = 0.31)。成人GHRD患者尿吡啶啉浓度明显高于对照组,而I型胶原c端肽分解产物和骨形成标志物没有差异。组织形态学的差异仅限于小梁连性的降低;这些数据证实了GH/IGF轴在调节骨大小和身体组成方面的重要性,这些肽对骨矿物质的获取和维持的贡献不太确定,因为尽管与GH抵抗相关的IGF- 1和IGFBP-3水平低,但体积骨密度仍然保持不变。
Growth hormone (GH) and insulin-like growth factor I (IGF-I) deficiencies have been associated with osteopenia in both children and adults, To examine the effects of growth hormone resistance on bone mineral and body composition, we studied 11 adults (mean age 30 years) with growth hormone receptor deficiency (GHRD, Laron syndrome) and 11 age-and gender-matched controls from Southern Ecuador. Bone mineral and body composition were determined by dual-energy X-ray absorptiometry. Bone physiology was assessed with biochemical markers of bone turnover and dynamic bone histomorphometry, Bone size and body composition differed markedly between subjects with GHRD and controls, Affected adults were 40 cm shorter than controls, had significantly less lean body mass, and had increased percent body fat, Bone mineral content and density (BMD) at the spine, femoral neck, and whole body were significantly lower in adults with GHRD than in controls, Mean BMD Z scores were -1.5 to -1.6 at all sites in affected women and -2.2 to -2.3 in men with GHRD. Estimated volumetric bone density (BMAD) at the spine and femoral neck, however, was not reduced in GHRD. Spine BMAD was 0.210 +/- 0.025 versus 0.177 +/- 0.021 for affected women versus controls (p < 0.05) and 0.173 +/- 0.018 versus 0.191 +/- 0.025 for men with GHRD versus normals (p = 0.31). Urinary pyridinoline concentrations were significantly greater in adults with GHRD than in controls, while type I collagen C-telopeptide breakdown products and markers of bone formation did not differ, Differences in histomorphometry were limited to a reduction in trabecular connectivity; bone volume and formation rate were similar to controls, These data confirm the importance of the GH/IGF axis in regulating bone size and body composition, The contribution of these peptides to the acquisition and maintenance of bone mineral is less certain since volumetric bone density was preserved despite low levels of IGF-I and IGFBP-3 associated with GH resistance.