Coutant R, Rouleau S, Despert F, Magontier N, Loisel D, Limal J M
Department of Pediatrics, University Hospital, 4 rue Larrey, 49000 Angers, France.
J Clin Endocrinol Metab. 2001 Oct;86(10):4649-54. doi: 10.1210/jcem.86.10.7962.
We analyzed the final height of 146 short children with either nonacquired GH deficiency or idiopathic short stature. Our purpose was 1) to assess growth according to the pituitary magnetic resonance imaging findings in the 63 GH-treated children with GH deficiency and 2) to compare the growth of the GH-deficient patients with normal magnetic resonance imaging (n = 48) to that of 32 treated and 51 untreated children with idiopathic short stature (GH peak to provocative tests >10 microg/liter). The mean GH dose was 0.44 IU/kg.wk (0.15 mg/kg.wk), given for a mean duration of 4.6 yr. Among the GH-deficient children, 15 had hypothalamic-pituitary abnormalities (stalk agenesis), all with total GH deficiency (GH peak <5 microg/liter). They were significantly shorter and younger at the time of diagnosis than those with normal magnetic resonance imaging, had better catch-up growth (+2.7 +/- 0.9 vs. +1.3 +/- 0.8 SD score; P < 0.01), and reached greater final height (-1.1 +/- 1.0 vs. -1.7 +/- 1.0 SD score; P < 0.05). Among patients with normal magnetic resonance imaging, there was no difference in catch-up growth and final height between partial and total GH deficiencies. GH-deficient subjects with normal magnetic resonance imaging and treated and untreated patients with idiopathic short stature had comparable auxological characteristics, age at evaluation, and target height. Although they had different catch-up growth (+1.3 +/- 0.8, +0.9 +/- 0.6, and +0.7 +/- 0.9 SD score, respectively; P < 0.01, by ANOVA), these patients reached a similar final height (-1.7 +/- 1.0, -2.1 +/- 0.8, and -2.1 +/- 1.0 SD score, respectively; P = 0.13). Pituitary magnetic resonance imaging findings show the heterogeneity within the group of nonacquired GH deficiency and help to predict the response to GH treatment in these patients. The similarities in growth between the GH-deficient children with normal magnetic resonance imaging and those with idiopathic short stature suggest that the short stature in the former subjects is at least partly due to factors other than GH deficiency.
我们分析了146名患有非获得性生长激素缺乏症或特发性身材矮小的矮小儿童的最终身高。我们的目的是:1)根据垂体磁共振成像结果评估63名接受生长激素治疗的生长激素缺乏症儿童的生长情况;2)比较磁共振成像正常的生长激素缺乏症患者(n = 48)与32名接受治疗和51名未接受治疗的特发性身材矮小儿童(生长激素激发试验峰值>10μg/升)的生长情况。生长激素的平均剂量为0.44IU/kg·周(0.15mg/kg·周),平均给药持续时间为4.6年。在生长激素缺乏症儿童中,15名患有下丘脑 - 垂体异常(垂体柄发育不全),均为完全性生长激素缺乏(生长激素峰值<5μg/升)。他们在诊断时比磁共振成像正常的儿童明显更矮、年龄更小,追赶生长更好(标准差评分+2.7±0.9对+1.3±0.8;P<0.01),最终身高更高(标准差评分-1.1±1.0对-1.7±1.0;P<0.05)。在磁共振成像正常的患者中,部分性和完全性生长激素缺乏症患者在追赶生长和最终身高方面没有差异。磁共振成像正常的生长激素缺乏症患者以及接受治疗和未接受治疗的特发性身材矮小患者在体格学特征、评估时的年龄和靶身高方面具有可比性。尽管他们的追赶生长情况不同(分别为标准差评分+1.3±0.8、+0.9±0.6和+0.7±0.9;方差分析,P<0.01),但这些患者的最终身高相似(分别为标准差评分-1.7±1.0、-2.1±0.8和-2.1±1.0;P = 0.13)。垂体磁共振成像结果显示了非获得性生长激素缺乏症组内的异质性,并有助于预测这些患者对生长激素治疗的反应。磁共振成像正常的生长激素缺乏症儿童与特发性身材矮小儿童在生长方面的相似性表明,前者身材矮小至少部分归因于生长激素缺乏以外的因素。