重组人生长激素对Turner综合征患儿身高的疗效

Therapeutic Effect of Recombinant Human Growth Hormone on the Stature of Children with Turner Syndrome

目的探讨重组人生长激素（rhGH）对Turner综合征（TS）患者身高的疗效,分析影响其治疗效果的因素。方法2004年1月-2007年6月四川大学华西第二医院儿科门诊12例TS患儿。年龄4.9-16.9（11.28±3.64）岁。均进行染色体核型分析确诊。常规行肝肾功能、血常规和头颅磁共振检查均正常。所有患儿尚未接受过生长激素或雌激素治疗。患儿有不同的临床表现,患儿均身材矮小,肘外翻9例,盾状胸8例,发际低4例,颈蹼2例,乳房未发育11例,无腋毛、阴毛11例,均无初潮,均无智力落后和其他畸形。rhGH为每日睡前皮下注射,注射部位为脐周3~5cm的环形区域。rhGH治疗平均剂量0.16IU/（kg·d）[0.15-0.18IU/（kg·d）],计算和比较治疗前后TS患儿身高标准差积分（HtSDS）,观察有无rhGH不良反应发生。结果持续治疗时间12个月9例,15个月2例,18个月1例。12例患者治疗前身高（122.77±15.98）cm,治疗后身高（129.92±14.59）cm,身高增长速度（0.55±0.15）cm/月,治疗前HtSDS为（-2.99±1.13）,治疗后为（-2.36±0.76）,治疗前后HtSDS比较有显著统计学意义（t=2.87P〈0.05）。3例出现一过性头痛,8例出现一过性膝关节疼痛。结论rhGH对TS患者有明显的促生长作用,可提高患者的生活质量。

Objective To explore the therapeutic effect of recombinant human growth hormone （rhGH） on the stature of children with Turner syndrome （TS） and analyze its influencing factors in treatment. Methods Twelve children with TS were selected in the outpatient department of West China second hospital of siehuan university between Jan. 2004 to Jun. 2007. They were diagnosed as TS with karyotypieally. The average age was 4.9 - 16.9 （ 11.28 ± 3.64） years old. Every patient must be made sure to have normal liver function, kidney funtion and MRI for hypothalamus and pituitary before enrollment. All eases were never treated with growth hormone or estrin. Not all the patients had the same manifestations： 12 eases with short stature ,9 cases with eubitus valgue, 8 cases with broad chest ,4 eases with low posterior hairline ,2 eases with webbed neck, 11 eases without pubes and armpit hair, none with menarche. Every patient must be made sure to have normal liver function ,kidney funtion and MRI for hypothalamus and pituitary before enrollment, rhGH was used subcutaneously 3 -5 em around navel every night before sleep. Average rhGH dose was 0.16 IU/（kg · d） [0.15 -0.18 IU/（kg · d） ] ,and height standard deviation score （HtSDS） was calculated and compared before and after rhGH treatment, and adverse reactions were observed. Results The duration of treatment was different ：9 eases for 12 cases months ,2 eases for 15 eases months and 1 ease for 18 eases months. The average height increase rat was （0.55 ± 0. 15 ） cm / month. Difference of HtSDS existed before and after treatment by t test （ t = 2.87 P 〈 0.05 ）. A little and mild adverse events occurred such as headache and knee joint ache. Conclusion rhGH can play a significant role in promoting growth of TS and it＇s necessary to observe long - term safety of rhGH in TS.