Anthropometry of patients with osteogenesis imperfecta
Anthropometry of patients with osteogenesis imperfecta
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DOI：
10.1136/adc.80.6.524
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发表时间：
1999-06-01
影响因子：
5.2
通讯作者：
Skovby, F
中科院分区：
文献类型：
--
作者：
Lund, AM;M端ller, J;Skovby, F
站立高度 测定了 86 名成骨不全症患者的站立高度、坐高、臂展、坐骨下腿长、头围和生长激素-胰岛素样生长因子 I (IGF-T) 轴。本研究的目的是确定站立高度和身体比例及其在成骨不全类型和胶原缺陷之间的变异性。所有组患者的平均站立高度均最大程度地降低，并且 III/IV 型成骨不全症和胶原质缺陷患者的变异性也有所降低。成骨不全症患者的平均站立高度低于未患病的一级家庭成员。成骨不全症患者躯干高度降低；头部尺寸增大，并且在成骨不全型和定性胶原缺陷的患者中比在 I 型成骨不全和定量胶原缺陷的患者中更为明显。 IGF-I 和 IGF 结合蛋白 3 (IGFBP-3) 的平均浓度较低，但大多数值在年龄特定参考值内。 的减少似乎与成骨不全类型和胶原缺陷类型相关。典型的躯干相对较短，头围和身体长度不成比例。
standing height Standing height, sitting height, armspan, subischial leg length, head circumference, and growth hormone-insulin-like growth factor I (IGF-T) axis were determined in 86 patients with osteogenesis imperfecta, The aim of this study was to determine standing height and body proportions and their variability among osteogenesis imperfecta types and collagen defects. Mean standing height was reduced in all groups of patients, to the greatest extent and variability in osteogenesis imperfecta type III/IV and in those with qualitative collagen defects. The mean standing height of patients with osteogenesis imperfecta was lower than that of their unaffected first degree family members. Truncal height of patients with osteogenesis imperfecta was reduced; head size was increased, and this was more pronounced in patients with osteogenesis imperfecta type and qualitative collagen defects than in patients with osteogenesis imperfecta type I and quantitative collagen defects, Mean concentrations of IGF-I and IGF binding protein 3 (IGFBP-3) were low, but most values were within age specific referencevalues, The reduction of appears to correlate with osteogenesis imperfecta type and the type of collagen defect. A relatively short trunk is typical and head circumference and body length are disproportionate.