Kamp G A, Kuilboer M M, Wynne H J, Rongen-Westerlaken C, Johnson M L, Veldhuis J D, Wit J M
Department of Pediatrics, Wilhelmina Children's Hospital, Utrecht, The Netherlands.
J Clin Endocrinol Metab. 1993 Jun;76(6):1604-9. doi: 10.1210/jcem.76.6.8501169.
Spontaneous growth and growth responses to GH therapy vary considerably among girls with Turner's syndrome. In an attempt to clarify this variability, we assessed growth parameters, 24-h GH profiles, arginine-stimulated serum GH levels, and plasma insulin-like growth factor-I (IGF-I) concentrations in a group of 41 girls with Turner's syndrome with a mean (+/- SD) age of 13 +/- 3 yr (range, 6.7-18.9). We subsequently treated all girls with biosynthetic GH (24 IU/m2 x week) and documented the growth response after 1 yr of therapy. GH profiles were analyzed according to Pulsar and Cluster, and GH secretion rates were calculated by waveform-independent deconvolution (Pulse). Factor analysis selected the mean 24-h GH secretion rate and number of GH peaks according to Cluster and Pulse as the principal GH profile variables to be used for further analysis. The mean (+/- SD) daily pituitary GH secretion rate was 127 +/- 47 micrograms/L.24 h (range, 37-232). The GH secretion rate correlated inversely with body mass index (r = -0.45; P < 0.01; n = 41). There was no relationship between the GH secretion rate and the growth parameters before or after GH therapy. However, the number of GH peaks (Pulse) correlated negatively with baseline height velocity (r = -0.53; P = 0.03) and was a positive predictor for height velocity increment during the first year of GH therapy (r = 0.71, P = 0.001). The mean (+/- SD) IGF-I level was 217 +/- 91 ng/mL (range, 87-413). There was no relationship between GH secretion rate or growth parameters and IGF-I. However, the number of GH peaks correlated negatively with IGF-I (r = -0.49; P = 0.04; n = 17). We conclude that an elevated spontaneous GH pulse frequency pattern is associated with relatively low IGF-I levels and slow baseline growth in girls with Turner's syndrome and that girls with such a pulse pattern may benefit most from exogenous GH therapy.
特纳综合征女孩的自然生长及对生长激素(GH)治疗的生长反应差异很大。为了阐明这种变异性,我们评估了一组41名特纳综合征女孩的生长参数、24小时GH谱、精氨酸刺激后的血清GH水平以及血浆胰岛素样生长因子-I(IGF-I)浓度,这些女孩的平均(±标准差)年龄为13±3岁(范围6.7 - 18.9岁)。随后我们用生物合成GH(24 IU/m²×周)治疗所有女孩,并记录治疗1年后的生长反应。根据Pulsar和Cluster方法分析GH谱,并通过波形独立去卷积(Pulse)计算GH分泌率。因子分析根据Cluster和Pulse方法选择平均24小时GH分泌率和GH峰次数作为主要的GH谱变量用于进一步分析。平均(±标准差)每日垂体GH分泌率为127±47μg/L·24小时(范围37 - 232)。GH分泌率与体重指数呈负相关(r = -0.45;P < 0.01;n = 41)。GH分泌率与GH治疗前后的生长参数均无关联。然而,GH峰次数(Pulse)与基线身高增长速度呈负相关(r = -0.53;P = 0.03),并且是GH治疗第一年身高增长速度增加的阳性预测指标(r = 0.71,P = 0.001)。平均(±标准差)IGF-I水平为217±91 ng/mL(范围87 - 413)。GH分泌率或生长参数与IGF-I之间均无关联。然而,GH峰次数与IGF-I呈负相关(r = -0.49;P = 0.04;n = 17)。我们得出结论,自发GH脉冲频率模式升高与特纳综合征女孩相对较低的IGF-I水平和缓慢的基线生长相关,并且具有这种脉冲模式的女孩可能从外源性GH治疗中获益最大。