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通过胰高血糖素刺激试验评估特发性生长激素缺乏症患儿的肾上腺轴。

Assessing the adrenal axis by the glucagon stimulation test in children with idiopathic growth hormone deficiency.

作者信息

Taieb Ach, Asma Ben Abdelkarim, Yosra Hasni, Amel Maaroufi, Maha Kacem, Molka Chaieb, Monia Zaouali, Koussay Ach

机构信息

Department of Endocrinology, University Hospital of Farhat Hached Sousse, Tunisia.

Department of Physiology, University Hospital of Farhat Hached Sousse, Tunisia.

出版信息

Pediatr Endocrinol Diabetes Metab. 2018;24(4):161-166. doi: 10.5114/pedm.2018.83361.

Abstract

Approximately 30% of children with idiopathic growth hormone deficiency (IGHD) also suffer from other pituitary hormone deficien-cies. Of children with IGHD, approximately 10% are unable to generate appropriate ACTH levels in response to stress. This study was prospectively designed to test the integrity of the adrenal axis in patients with an established diagnosis of IGHD using the glucagon stimulation test (GST). The study population comprised 39 patients with established childhood-onset IGHD. The diagnosis of GHD was established on the basis of failure of GH to increase over 10 ng/ml after two stimulation tests. The GST was performed by intra-muscular injection of 1 mg glucagon. The criteria followed to define adrenal deficiency was cortisol less than 167 ng/l in response to GST. The mean peak blood glucose level was 8.64 ±1.71 mmol/l. Analysing the cohort using the cut-off of 167 ng/ml to define adrenal insufficiency under GST, there were 25.64% of children diagnosed: 20% among males and 35.7% among females. Subjects with GH and ACTH deficiency had a mean peak GH of 2.07 ±1.79 ng/ml - significantly lower than GH peak of children with IGHD alone (p < 0,001). The frequency of children with combined somatotroph and corticotroph deficiencies with a GH peak < 3 ng/ml was 21% (p < 0,001). The current study identified a prevalence of adrenal insufficiency of 25.64%, which could predict greater risk for children if untreated, especially because a substantial proportion of patients do not present clinical symptoms.

摘要

约30%的特发性生长激素缺乏症(IGHD)患儿还伴有其他垂体激素缺乏。在IGHD患儿中,约10%在应激时无法产生适当的促肾上腺皮质激素(ACTH)水平。本研究前瞻性设计,使用胰高血糖素刺激试验(GST)来检测确诊为IGHD患者的肾上腺轴完整性。研究人群包括39例确诊为儿童期起病的IGHD患者。生长激素缺乏症的诊断基于两次刺激试验后生长激素未能升高超过10 ng/ml。GST通过肌肉注射1 mg胰高血糖素进行。定义肾上腺功能不全的标准是GST刺激后皮质醇低于167 ng/l。平均血糖峰值水平为8.64±1.71 mmol/l。以167 ng/ml为界值分析队列,以定义GST下的肾上腺功能不全,诊断出25.64%的儿童:男性中为20%,女性中为35.7%。生长激素和促肾上腺皮质激素缺乏的受试者平均生长激素峰值为2.07±1.79 ng/ml,显著低于单纯IGHD儿童的生长激素峰值(p<0.001)。生长激素峰值<3 ng/ml的生长激素和促肾上腺皮质激素联合缺乏儿童的发生率为21%(p<0.001)。本研究确定肾上腺功能不全的患病率为25.64%,如果不治疗,这可能预示着儿童面临更大风险,尤其是因为相当一部分患者没有临床症状。

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