Effects of Recombinant Human Growth Hormone and Sex Hormone on Children with Turner Syndrome_West China Medical Journal

Authors：

CHEN Lina ¹ , XIANG Chengfa ²

1.Department of Pediatrics, Sichuan Academy of Medical Science & Provincial People’s Hospital, Chengdu, Sichuan 610072, P. R. China; 2. Department of Pediatrics, West China Second Hospital, Sichuan University, Chengdu, Sichuan 610041, P. R. China;

Corresponding author：

Keywords：

Turner综合征; 重组人生长激素; 雌激素/孕激素

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【摘要】　目的　探讨基因重组人生长激素（recombinant human growth hormone，rhGH）及雌/孕激素（estrogen/progestogem，E/P）治疗对Turner综合征（Turner syndrome，TS）患儿身高及性征发育的影响。　方法　 2005年1月—2009年6月四川大学华西第二医院门诊就诊TS患儿22例，12例患儿接受rhGH治疗，年龄（13.58±2.23）岁，剂量0.15 U/（kg•d），睡前皮下注射，疗程4~24个月。16例年龄≥13岁、骨龄≥11岁的患儿接受E/P治疗，疗程3~30个月。　结果　 rhGH治疗后患儿身高、身高的标准差积分提高，生长速率达（9.33±2.39）cm/年；E/P治疗可促进患儿乳房发育及规律月经出现。　结论　 rhGH和E/P治疗对TS患儿身高增长及性征发育有明显疗效。
【Abstract】　Objective　 To explore the therapeutic effects of recombinant human growth hormone (rhGH) and sex hormone on the stature and sex feature of children with Turner syndrome (TS). 　Methods　 A total of 22 children with TS were selected in the outpatients department of West China Second Hospital between January 2005 and June 2009. Twelve children with TS the average age of (13.58±2.23) years received rhGH [0.15 U/(kg•d)] every night before sleep for 4-24 months . Sixteen children with TS (age≥14 years old, bone age≥11 years old) underwent estrogen and progestogen (E/P) treatment for 3-30 months. 　Results　 The height and height standard deviation score increased significantly in children with rhGH therapy (P lt;0.01). The height velocity was (9.33±2.39) cm/year after the treatment. The treatment of estrogen and progestogen could promote the development of breast and establish menstrual cycle in children with TS. 　Conclusion　 rhGH and E/P can play a significant role in treatment of TS in children.

Citation： CHEN Lina,XIANG Chengfa. Effects of Recombinant Human Growth Hormone and Sex Hormone on Children with Turner Syndrome. West China Medical Journal, 2011, 26(5): 720-722. doi: Copy

1.	颜纯, 王慕逖. 小儿内分泌学[M]. 2版. 北京: 人民卫生出版社, 2006: 348-358.
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9.	江静, 傅曼芬, 王伟, 等. Turner综合征的实验临床综合研究[J]. 中国优生与遗传杂志, 2005, 13(11): 48-51.
10.	吴瑾, 向承发, 孙小妹, 等. 重组人生长激素治疗特纳氏综合征临床观察[J]. 四川医学, 2008, 29(7): 881-882.
11.	吕淑云. Turner综合征药物治疗进展[J]. 齐鲁医学杂志, 2007, 22(5): 467-468.
12.	Ranke MB, Lindberg A, Ferrández Lonqás A, et al. Major determinants of height development in Turner syndrome (TS) patients treated with GH: analysis of 987 patients from KIGS[J]. Pediatr Res, 2007, 61(1): 105-110.
13.	江静, 王伟, 邱定众, 等. 生长激素对Turner综合征的促身高作用[J]. 中国优生与遗传杂志, 2006, 14(2): 28-29.
14.	余涛, 向承发. 重组人生长激素对Turner综合征患儿身高的疗效[J]. 实用儿科临床杂志, 2008, 23(8): 624-625.
15.	张茵颀, 项蓉. 基因重组人生长激素治疗非生长激素缺乏性矮小的临床研究[J]. 中国妇幼保健, 2009, 24(34): 4854-4856.
16.	Bolar K, Hoffman AR, Maneatis T, et al. Long-term safety of recombinant human growth hormone in turner syndrome[J]. J Clin Endocrinal Metab, 2008, 93(2): 344-351.
17.	Bondy CA, Turner Syndrome Study Group. Care of girls and women with turner syndrome: A guide line of the Turner Syndrome Study Group[J]. J Clin Endocrinal Metab, 2007, 92(1): 10-25.
18.	梁艳, 罗小平. Turner综合征[J]. 中国实用儿科杂志, 2005, 20(8): 064-364.

1. 　颜纯, 王慕逖. 小儿内分泌学[M]. 2版. 北京: 人民卫生出版社, 2006: 348-358.
2. 　Morqan T. Turner syndrome: diagnosis and management[J]. Am Fam Physician, 2007, 76(3): 405-410.
3. 　王红英, 何亚香, 卲雪君, 等. 苏州地区67例儿童Turner 综合征临床特征与染色体分析[J]. 中国优生与遗传杂志, 2009, 17(10): 44.
4. 　梁燕, 程静, 蔡燕娜, 等. Turner综合征患儿染色体核型和盆腔超声分析[J]. 中国当代儿科杂志, 2008, 10(2): 158-160.
5. 　吴瑾, 向承发, 王栋. 1 038例矮小症病因及治疗的研究[J]. 中国妇幼保健, 2008, (7): 947-950.
6. 　刘楠, 佟彤, 陈艳玲, 等. 天津地区Turner 综合征的临床表现与核型分析[J]. 中国优生与遗传杂志, 2009, 17(12): 52-53.
7. 　中华医学会儿科学分会内分泌遗传代谢组, Turner综合征生长方式研究协作组. 253例中国Turner综合征患者的自然生长曲线[J]. 中华儿科杂志, 2001, 39(3): 141-144.
8. 　Quigley CA, Crown BJ, Anglin DG, et al. Growth hormone and low dose estrogen in Turner syndrome: results of a United States multi-center trial to near-final height [J]. J Clin Endocrinol Metab, 2002, 87(5): 2033-2041.
9. 　江静, 傅曼芬, 王伟, 等. Turner综合征的实验临床综合研究[J]. 中国优生与遗传杂志, 2005, 13(11): 48-51.
10. 　吴瑾, 向承发, 孙小妹, 等. 重组人生长激素治疗特纳氏综合征临床观察[J]. 四川医学, 2008, 29(7): 881-882.
11. 　吕淑云. Turner综合征药物治疗进展[J]. 齐鲁医学杂志, 2007, 22(5): 467-468.
12. 　Ranke MB, Lindberg A, Ferrández Lonqás A, et al. Major determinants of height development in Turner syndrome (TS) patients treated with GH: analysis of 987 patients from KIGS[J]. Pediatr Res, 2007, 61(1): 105-110.
13. 　江静, 王伟, 邱定众, 等. 生长激素对Turner综合征的促身高作用[J]. 中国优生与遗传杂志, 2006, 14(2): 28-29.
14. 　余涛, 向承发. 重组人生长激素对Turner综合征患儿身高的疗效[J]. 实用儿科临床杂志, 2008, 23(8): 624-625.
15. 　张茵颀, 项蓉. 基因重组人生长激素治疗非生长激素缺乏性矮小的临床研究[J]. 中国妇幼保健, 2009, 24(34): 4854-4856.
16. 　Bolar K, Hoffman AR, Maneatis T, et al. Long-term safety of recombinant human growth hormone in turner syndrome[J]. J Clin Endocrinal Metab, 2008, 93(2): 344-351.
17. 　Bondy CA, Turner Syndrome Study Group. Care of girls and women with turner syndrome: A guide line of the Turner Syndrome Study Group[J]. J Clin Endocrinal Metab, 2007, 92(1): 10-25.
18. 　梁艳, 罗小平. Turner综合征[J]. 中国实用儿科杂志, 2005, 20(8): 064-364.

West China Medical Journal

Effects of Recombinant Human Growth Hormone and Sex Hormone on Children with Turner Syndrome

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