Growth hormone-binding protein levels: studies of children with short stature.
Growth hormone-binding protein levels: studies of children with short stature.
复制标题
生长激素结合蛋白水平：对身材矮小的儿童的研究。
DOI：
10.1016/0026-0495(94)90104-x
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发表时间：
1994
期刊：
影响因子：
--
通讯作者：
Merimee,TJ
中科院分区：
文献类型：
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作者：
Mauras,N;Carlsson,LM;Murphy,S;Merimee,TJ
血清中的高亲和力生长激素结合蛋白（GHBP）来源于GH受体的胞外结构域。在试图调查GHBP水平的差异，在各种条件下的不良增长，我们测量GHBP水平的两种方法-超凝胶色谱技术和配体介导的免疫功能测定（LIFA）。研究了以下三组儿童：特纳综合征（n = 7），特发性和/或家族性身材矮小（[ISS] n = 15）和器质性或特发性垂体功能减退症（n = 19）。所有组的年龄相似（特纳综合征，10.1 ± 0.9岁; ISS，10.0 ± 0.7;垂体功能减退症，11.5 ± 1.0）和身高SEM评分相似（特纳综合征，−2.9 ± 0.3; ISS，−3.0 ± 0.4;垂体功能减退症，−2.3 ± 0.4）。将其值与相似年龄的健康对照组的GHBP值进行比较。GHBP的免疫功能测定值如下：特纳综合征，235.4 ± 26.0 pmol/L; ISS，122.4 ± 11.0;垂体功能减退，157.1 ± 23.0。这些结果在患有ISS和垂体功能减退症的受试者中与9至12岁的健康对照组相比有显著差异（N = 255; GHBP = 287.9 ± 10.9 pMol/L;与ISS和垂体功能减退症相比P<0.001）。使用Ultrogel色谱法发现了类似的变化。即使采用更严格的标准来定义垂体功能减退症（即，对刺激的峰值GH反应<6.0 ng/mL，而不是最初的≤ 10 ng/mL），GHBP水平的这种差异仍然显著。对所有三组中的GHBP值和几个生长参数进行相关分析，包括年龄、身高、身高SD评分、体重、血浆胰岛素样生长因子-I（IGF-I）、对刺激的峰值GH反应和生长速度。在所有三组中，唯一一致的显著相关性是GHBP和体重之间的相关性（特纳综合征，r= 0.92; ISS，0.61;垂体功能减退，0.55;P<0.01）。总之，特纳综合征的女孩有正常的GHBP水平。然而，在ISS中存在GHBP的相对缺乏，类似于垂体功能减退症患者。这可能代表GH受体定量缺乏，这可能导致这些患者的线性生长不良。
A high-affinity growth hormone-binding protein (GHBP) in serum is derived from the extracellular domain of the GH receptor. In an attempt to investigate the differences in GHBP levels in various conditions of poor growth, we measured GHBP levels by two methods—an Ultrogel chromatographic technique and a ligand-mediated immunofunctional assay (LIFA). The following three groups of children were studied: Turner's syndrome (n = 7), idiopathic and/or familial short stature ([ISS] n = 15), and organic or idiopathic hypopituitarism (n = 19). All groups were similar in age (Turner's syndrome, 10.1 ± 0.9 years; ISS, 10.0 ± 0.7; hypopituitarism, 11.5 ± 1.0) and height SEM score (Turner's syndrome, −2.9 ± 0.3; ISS, −3.0 ± 0.4; hypopituitarism, −2.3 ± 0.4). Their values were compared with those values of GHBP in healthy controls of similar age. Immunofunctional assay values for GHBP were as follows: Turner's syndrome, 235.4 ± 26.0 pmol/L; ISS, 122.4 ± 11.0; and hypopituitarism, 157.1 ± 23.0. These results were significantly different in subjects with ISS and hypopituitarism as compared with a group of healthy controls between the ages of 9 and 12 years (N = 255; GHBP = 287.9 ± 10.9 pMol/L;P< .001 compared with both ISS and hypopituitarism). Similar changes were found using Ultrogel chromatography. This difference in GHBP levels is still significant even when more stringent criteria are applied to define hypopituitarism (ie, peak GH responses to stimuli <6.0 ng/mL, instead of ≤ 10 ng/mL originally). Correlation analysis was performed for GHBP values in all three groups and several parameters of growth, including age, height, height SD score, weight, plasma insulin-like growth factor-I (IGF-I), peak GH response to stimuli, and growth velocity. The only consistent significant correlation found was between GHBP and weight in all three groups (Turner's syndrome,r= .92; ISS, .61; hypopituitarism, .55;P< .01). In conclusion, girls with Turner's syndrome have normal GHBP levels. However, in ISS a relative deficiency of the GHBP is present, similar to patients with hypopituitarism. This may represent a quantitative GH receptor deficiency, which might contribute to these patients' poor linear growth.