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生长激素释放激素试验对矮小儿童的诊断价值

Diagnostic value of growth hormone-releasing hormone tests in short children.

作者信息

Butenandt O

机构信息

Department of Paediatric Endocrinology, Children's Hospital, University of Munich, West Germany.

出版信息

Acta Paediatr Scand Suppl. 1989;349:93-9; discussion 100. doi: 10.1111/j.1651-2227.1989.tb17176.x.

Abstract

The growth hormone-releasing hormone (GHRH) test was applied to more than 230 children. Twenty-five out of 61 patients with proven growth hormone (GH) deficiency responded to GHRH with a GH increase of greater than 10 ng/ml. In most of the patients with idiopathic GH deficiency, a priming procedure using daily injections of GHRH improved the secretory response to GHRH. Nearly all children with familial shortness of stature showed a prompt increase in GH levels, with a mean peak level of 34.9 ng/ml (range 0.5-144 ng/ml). A second test was performed in five children with familial short stature because of failure to respond to the first test. Children with constitutional delay of growth and development did not differ in their GH response from patients with familial shortness of stature. Ten girls with Ullrich-Turner's syndrome responded with a mean increase of 22 ng/ml GH (range 10.1-34.0 ng/ml). Therapy with glucocorticoids, as well as endogenous hypersecretion of cortisol, suppressed the responsiveness of the pituitary gland to GHRH. Suppression was also observed following a single dose of dexamethasone during the steroid-suppression test in eight obese children. Low responsiveness of the pituitary gland was also seen in patients with thalassaemia and transfusion-induced haemosiderosis. It is concluded that it is not possible to detect GH deficiency with a single GHRH test. A full endocrinological evaluation is necessary to prove the diagnosis.

摘要

生长激素释放激素(GHRH)试验应用于230多名儿童。61名经证实生长激素(GH)缺乏的患者中,有25名对GHRH有反应,GH升高超过10 ng/ml。在大多数特发性GH缺乏的患者中,采用每日注射GHRH的激发程序可改善对GHRH的分泌反应。几乎所有家族性身材矮小的儿童GH水平均迅速升高,平均峰值水平为34.9 ng/ml(范围0.5 - 144 ng/ml)。由于对首次试验无反应,对5名家族性身材矮小的儿童进行了第二次试验。生长发育体质性延迟的儿童与家族性身材矮小的患者在GH反应方面没有差异。10名患有乌尔里希 - 特纳综合征的女孩GH平均升高22 ng/ml(范围10.1 - 34.0 ng/ml)。糖皮质激素治疗以及皮质醇的内源性分泌过多会抑制垂体对GHRH的反应性。在8名肥胖儿童的类固醇抑制试验中,单次给予地塞米松后也观察到了抑制现象。地中海贫血和输血引起的血色素沉着症患者也出现垂体反应性降低。结论是单次GHRH试验无法检测出GH缺乏。必须进行全面的内分泌评估以证实诊断。

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