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孤立性生长激素缺乏症患儿:空蝶鞍与正常蝶鞍。

Children with isolated growth hormone deficiency: Empty sella versus normal sella.

作者信息

Ismail Nagwa Abdallah, Metwaly Nermeen Salah Eldin, El-Moguy Fatma Ahmed, Hafez Mona Hassan, El Dayem Soha M Abd, Farid Tarek Mohamed

机构信息

Department of Pediatrics, National Research Centre, Faculty of Medicine, Cairo University, Cairo, Egypt.

出版信息

Indian J Hum Genet. 2013 Apr;19(2):130-5. doi: 10.4103/0971-6866.116102.

Abstract

BACKGROUND

Empty sella (ES) may be associated with variable clinical conditions ranging from the occasional discovery of a clinically asymptomatic pouch within the sella turcica to severe intracranial hypertension and rhinorrhea. The need for replacement hormone therapy in ES, as in other syndromes that may cause hypopituitarism, must be assessed for every single hormone, including growth hormone (GH).

AIM

To determine whether or not the presence of ES could allow some changes in the GH responses of the isolated growth hormone deficiency (GHD) patients.

MATERIALS AND METHODS

We included a cohort of 59 short stature children and adolescents with isolated GHD. According to computed tomography finding, they were classified into 2 groups: Group 1 included 40 children with normal sella and 19 children with ES in Group 2. All patients received recombinant human growth hormone (rhGH) with a standard dose of 20 IU/m(2)/week.

RESULTS

The baseline results were not significantly different for all variables except weight standard deviation was smaller with statistical significant difference (P = 0.02). We identified no significant differences when comparing both groups, except for height standard deviation (HTSD) after the first year of therapy which revealed significant difference in favor of group 1. When comparing pre- and the two post-treatments HTSD results of the studied cases, all showed significant changes after GH therapy. The results of related variables pre-and post-treatment in both the groups showed significant improvement in all variables of the two groups of the study.

CONCLUSION

Our study showed a similar stature outcome in the two treatment groups.

摘要

背景

空蝶鞍(ES)可能与多种临床情况相关,从蝶鞍内偶尔发现无症状的囊袋到严重的颅内高压和鼻漏。与其他可能导致垂体功能减退的综合征一样,对于ES患者,必须针对包括生长激素(GH)在内的每一种激素评估其替代激素治疗的必要性。

目的

确定ES的存在是否会使孤立性生长激素缺乏(GHD)患者的GH反应发生某些变化。

材料与方法

我们纳入了一组59名身材矮小的孤立性GHD儿童和青少年。根据计算机断层扫描结果,他们被分为两组:第1组包括40名蝶鞍正常的儿童,第2组包括19名患有ES的儿童。所有患者均接受标准剂量为20 IU/m²/周的重组人生长激素(rhGH)治疗。

结果

除体重标准差较小且有统计学显著差异(P = 0.02)外,所有变量的基线结果无显著差异。比较两组时,我们未发现显著差异,但治疗第一年后的身高标准差(HTSD)显示第1组有显著差异。比较研究病例治疗前和治疗后的两个HTSD结果时,所有结果在GH治疗后均显示出显著变化。两组治疗前后相关变量的结果显示,两组的所有变量均有显著改善。

结论

我们的研究表明两个治疗组的身高结局相似。

相似文献

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