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两个家族性低钙血症高钙血症患者对刺激物的生长激素分泌受损。

Impaired GH secretion to provocative stimuli in two families with hypocalciuric hypercalcaemia.

作者信息

Cecconi Elisabetta, Bogazzi Fausto, Cetani Filomena, Grasso Lucia, Marcocci Claudio, Genovesi Maura, Procopio Massimo, Gasperi Maurizio, Pinchera Aldo, Ghigo Ezio, Martino Enio

机构信息

Department of Endocrinology and Metabolism, University of Pisa, Pisa, Italy.

出版信息

Clin Endocrinol (Oxf). 2003 Nov;59(5):604-6. doi: 10.1046/j.1365-2265.2003.01891.x.

Abstract

OBJECTIVE

To determine whether hypercalcemia per se might be responsible for an impairment in GH secretion.

DESIGN

Prospective study.

PATIENTS

Six subjects of two unrelated families with familial hypocalciuric hypercalcaemia (FHH), an autosomal dominant disorder due to inactivating mutations in the calcium receptor gene, leading to an increase in serum calcium levels and inappropriately normal serum PTH concentrations. Forty normal subjects, matched for sex and age served as controls.

MEASUREMENTS

Serum GH concentrations were measured after GHRH-Arginine (GHRH-Arg) stimulation test; serum IGF-I, ACTH, cortisol, FT4, FT3, TSH, PRL, LH, FSH levels were measured under basal conditions.

RESULTS

All subjects (two male, four female, age range 24-74 years) had increased serum ionized calcium levels (range 1.36-1.56 mmol/l) and five of six patients had normal PTH levels (range for all patients was 14-68 ng/l). Basal serum GH concentrations ranged from 0.1 to 7.0 micro g/l. Mean serum GH secretory peak after GHRH-Arg stimulation test was reduced in five subjects (mean 9.3 +/- 3.6 microg/l, P < 0.006 vs. Controls, mean 67.0 +/- 44.0 microg/l, cut-off, 16.0 microg/l) and normal in one subject (38.7 microg/l). However, serum IGF-I levels were reduced only in two patients (29 and 57 microg/l) and normal in four subjects (range 127-208 microg/l). The basal secretion of the other anterior pituitary hormones was within their normal ranges.

CONCLUSIONS

The results of the present study support the concept that elevated serum calcium levels impair GH secretion. However, the clinical relevance of GH deficiency in FHH remains to be elucidated.

摘要

目的

确定高钙血症本身是否可能导致生长激素(GH)分泌受损。

设计

前瞻性研究。

患者

两个无血缘关系的家族中6名患有家族性低钙血症性高钙血症(FHH)的受试者,这是一种常染色体显性疾病,由钙受体基因的失活突变引起,导致血清钙水平升高且血清甲状旁腺激素(PTH)浓度异常正常。40名年龄和性别匹配的正常受试者作为对照。

测量

在生长激素释放激素 - 精氨酸(GHRH - Arg)刺激试验后测量血清GH浓度;在基础条件下测量血清胰岛素样生长因子 - I(IGF - I)、促肾上腺皮质激素(ACTH)、皮质醇、游离甲状腺素(FT4)、游离三碘甲状腺原氨酸(FT3)、促甲状腺激素(TSH)、催乳素(PRL)、黄体生成素(LH)、卵泡刺激素(FSH)水平。

结果

所有受试者(2名男性,4名女性,年龄范围24 - 74岁)血清离子钙水平均升高(范围1.36 - 1.56 mmol/L),6名患者中有5名PTH水平正常(所有患者的范围为14 - 68 ng/L)。基础血清GH浓度范围为0.1至7.0 μg/L。在GHRH - Arg刺激试验后,5名受试者的平均血清GH分泌峰值降低(平均9.3±3.6 μg/L,与对照组相比P < 0.006,对照组平均67.0±44.0 μg/L,临界值16.0 μg/L),1名受试者正常(38.7 μg/L)。然而,仅2名患者的血清IGF - I水平降低(29和57 μg/L),4名受试者正常(范围127 - 208 μg/L)。其他垂体前叶激素的基础分泌在正常范围内。

结论

本研究结果支持血清钙水平升高会损害GH分泌这一概念。然而,FHH中GH缺乏的临床相关性仍有待阐明。

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