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[下丘脑-垂体轴功能障碍儿童及特纳综合征女孩经生长激素释放激素(GHRH)刺激后生长激素的行为]

[Behavior of growth hormone after stimulation with growth hormone releasing hormone (GHRH) in children with disorders of the hypothalamo-hypophyseal axis and girls with Turner syndrome].

作者信息

Schmitt K, Schober E, Birnbacher R, Frisch H

机构信息

Univ. Kinderklinik, Wien.

出版信息

Klin Padiatr. 1996 Jan-Feb;208(1):29-34. doi: 10.1055/s-2008-1043989.

Abstract

BACKGROUND

To investigate the hypothalamic pituitary axis in children with various growth disorders stimulation tests with growth hormone releasing hormone (GHRH) were performed and compared to pharmacological stimulation tests.

PATIENTS

103 subjects were studied-15 healthy volunteers, 20 patients with isolated growth hormone deficiency (IGHD), 16 patients with multiple pituitary hormone deficiency (MPHD), 17 children with organic growth hormone deficiency (OGHD) and 35 Turner patients.

METHODS

1 microgram/kg GHRH was administered iv, blood samples were drawn before GHRH and after 5, 15, 30, 45, 60 and 90 minutes. In 53 patients a second GHRH-test was done after repeated GHRH injections (7 times 1 microgram/kg GHRH every 3 hours).

RESULTS

In the group of healthy probands we found wide variations of stimulated growth hormone (GH) levels. In 7 of the 20 patients with IGHD the stimulated GH level exceeded 10 ng/ml in one of the two GHRH-tests. The maximal GH response to the second GHRH-test (8.3 +/- 6.8 ng/ml) was significantly higher (p < or = 0.025) than after the first GHRH-test (5.0 +/- 4.0 ng/ml). In patients with MPHD stimulated GH values were significantly lower than in patients with IGHD (p < or = 0.005). GHRH stimulation tests in OWHM patients did not differ from the results in children with IGHD. The GHRH induced GH response in girls with Turner syndrome was significantly lower than in the healthy volunteers (p < or = 0.025). Basal (p < or = 0.05) and GHRH stimulated GH levels (p < 0.02) were lower after estrogen administration.

CONCLUSIONS

Pituitary GH secretion could be activated by repeated stimulation with GHRH (priming) in children with hypothalamic GH deficiency. Only in the patients with MPHD a correlation could be found between the result of the GHRH-stimulation and one pharmacological GH test. In children with OGHD the radiation dose was negatively correlated to the maximal GH concentrations after L-Dopa stimulation. Basal and GHRH stimulated GH levels in Turner syndrome were lower after estrogen administration.

摘要

背景

为研究各种生长障碍患儿的下丘脑 - 垂体轴,进行了生长激素释放激素(GHRH)刺激试验,并与药物刺激试验进行比较。

患者

共研究了103名受试者,包括15名健康志愿者、20名孤立性生长激素缺乏症(IGHD)患者、16名多种垂体激素缺乏症(MPHD)患者、17名器质性生长激素缺乏症(OGHD)患儿和35名特纳综合征患者。

方法

静脉注射1微克/千克GHRH,在注射前及注射后5、15、30、45、60和90分钟采集血样。53例患者在重复注射GHRH(每3小时注射7次,每次1微克/千克GHRH)后进行了第二次GHRH试验。

结果

在健康受试者组中,我们发现刺激后的生长激素(GH)水平存在很大差异。在20例IGHD患者中,有7例在两次GHRH试验中的一次中,刺激后的GH水平超过10纳克/毫升。第二次GHRH试验的最大GH反应(8.3±6.8纳克/毫升)显著高于第一次GHRH试验后(5.0±4.0纳克/毫升)(p≤0.025)。MPHD患者刺激后的GH值显著低于IGHD患者(p≤0.005)。OGHD患者的GHRH刺激试验结果与IGHD患儿的结果无差异。特纳综合征女孩中GHRH诱导的GH反应显著低于健康志愿者(p≤0.025)。雌激素给药后,基础(p≤0.05)和GHRH刺激后的GH水平降低(p<0.02)。

结论

下丘脑性GH缺乏患儿垂体GH分泌可通过GHRH重复刺激(启动)而被激活。仅在MPHD患者中,GHRH刺激结果与一种药物GH试验结果之间存在相关性。在OGHD患儿中,放射剂量与左旋多巴刺激后的最大GH浓度呈负相关。雌激素给药后,特纳综合征患者的基础和GHRH刺激后的GH水平降低。

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