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特纳综合征的生长激素治疗:真实世界经验

Growth Hormone Treatment in Turner's Syndrome: A Real World Experience.

作者信息

Reddy Danda Vijay Sheker, Sreedevi P, Arun G, Rao P Srinivas

机构信息

Department of Endocrinology, Gandhi Medical College/Gandhi Hospital, Musheerabad, Hyderabad, Telangana, India.

出版信息

Indian J Endocrinol Metab. 2017 May-Jun;21(3):378-381. doi: 10.4103/ijem.IJEM_36_17.

DOI:10.4103/ijem.IJEM_36_17
PMID:28553590
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC5434718/
Abstract

OBJECTIVE

Short stature is a universal clinical feature of Turner's syndrome (TS). Growth failure begins in fetal life, and adults with TS are on an average 20 cm shorter than the normal female population. Since there is a paucity of data from India regarding the effect of growth hormone (GH) on TS patients, we retrospectively analyzed the data of TS patients who are on GH treatment.

METHODS

This hospital-based observational retrospective study was conducted in a tertiary care hospital of Hyderabad. The data such as height, weight, and bone age of 16 patients who are diagnosed with TS on GH therapy for at least 6 months were included in the study. All the patients were treated with human recombinant GH at the dose of 0.3 mg/kg/week administered as daily subcutaneous injections.

RESULTS

The mean age at diagnosis was 12.7 years. The mean height at the start of GH therapy was 1.26 m, and mean height standard deviation score (HSDS) was-0.61 when compared to Turner's specific reference data. With a mean duration of GH therapy of 25 months, the mean height at the end of therapy was 1.37 m and the mean height as per HSDS was + 0.37 resulting in a mean height gain of + 0.99 HSDS.

CONCLUSION

Our observation shows that girls with TS benefit from early diagnosis and initiation of treatment with GH.

摘要

目的

身材矮小是特纳综合征(TS)的一个普遍临床特征。生长发育迟缓始于胎儿期,患TS的成年人平均比正常女性群体矮20厘米。由于印度缺乏关于生长激素(GH)对TS患者影响的数据,我们回顾性分析了接受GH治疗的TS患者的数据。

方法

这项基于医院的观察性回顾性研究在海得拉巴的一家三级医疗医院进行。研究纳入了16例经诊断为TS且接受GH治疗至少6个月的患者的身高、体重和骨龄等数据。所有患者均接受重组人生长激素治疗,剂量为0.3mg/kg/周,每日皮下注射。

结果

诊断时的平均年龄为12.7岁。开始GH治疗时的平均身高为1.26米,与特纳综合征特定参考数据相比,平均身高标准差评分(HSDS)为-0.61。GH治疗的平均持续时间为25个月,治疗结束时的平均身高为1.37米,根据HSDS的平均身高为+0.37,平均身高增加了+0.99 HSDS。

结论

我们的观察表明,患TS的女孩可从早期诊断和开始GH治疗中获益。

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Growth Hormone Treatment in Turner's Syndrome: A Real World Experience.特纳综合征的生长激素治疗:真实世界经验
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Final height of growth hormone-treated GH-deficient children and girls with Turner's syndrome: the Dutch experience. The Dutch Advisory Group on Growth Hormone.生长激素治疗的生长激素缺乏儿童及特纳综合征女童的最终身高:荷兰的经验。荷兰生长激素咨询小组。
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Influence of spontaneous or induced puberty on the growth promoting effect of treatment with growth hormone in girls with Turner's syndrome.自然青春期或诱导青春期对特纳综合征女孩生长激素治疗生长促进作用的影响。
Clin Endocrinol (Oxf). 1993 Mar;38(3):253-60. doi: 10.1111/j.1365-2265.1993.tb01003.x.
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Growth hormone therapy of Turner's syndrome: beneficial effect on adult height.特纳综合征的生长激素治疗:对成人身高的有益影响。
J Pediatr. 1998 Feb;132(2):319-24. doi: 10.1016/s0022-3476(98)70452-4.
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Decreased metabolic clearance of endogenous growth hormone and specific alterations in the pulsatile mode of growth hormone secretion occur in prepubertal girls with Turner's syndrome. Genentech Collaborative Group.患有特纳综合征的青春期前女孩体内,内源性生长激素的代谢清除率降低,且生长激素分泌的脉冲模式出现特定改变。基因泰克协作组。
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Acta Endocrinol (Copenh). 1990 Sep;123(3):291-7. doi: 10.1530/acta.0.1230291.

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