生长激素治疗Turner综合征成年终身高的疗效及其影响因素分析  

Effects of recombinant human growth hormone on final adult height in children with Turner＇s syndrome and analysis of the influencing factors

作　　者：王瑞芳[1] 董治亚[1] 王伟[1] 倪继红[1] 陈凤生[1] 孙文鑫[1] 王秀民[1] 王德芬[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院儿科,200025

出　　处：《中华内分泌代谢杂志》2011年第11期901-905,共5页Chinese Journal of Endocrinology and Metabolism

摘　　要：目的观察重组人生长激素（rhGH）治疗Turner综合征（TS）的疗效并综合分析改善其成年终身高（FAH）相关的影响因素。方法达FAH的TS患儿以往经rhGH治疗组30例、未治疗组16例，比较分析经治组与未治组、治疗前后的预测成年身高的标准差分值（PAHSDS）、按年龄的身高标准差分值（HtSDSCA）、按骨龄的身高标准差分值（HtSDSBA）和生长速率（GV）等生长参数的变化，并分析影响FAH的因素。结果rhGH治疗组较未治组FAH有明显改善[（149．5±6．3对142．4±5．2）cm，P〈0．01]，相关性分析显示治疗组FAH与初诊时HtSDSCA、HtSDSBA、初诊时的身高别年龄、rhGH治疗的疗程、性激素治疗前单纯rhGH治疗的疗程和初诊时PAHSDS（PAH0SDS）呈正相关；逐步回归显示性激素治疗前单纯rhGH治疗的疗程和PAH0SDS是影响治疗组患者FAH的独立因素（F分别为11．56和86．91，均P〈0．01）；且单体型组（45，XO）和嵌和型组（46，XX／45，XO）TS患儿的FAH差异有统计学意义（P=0．038）。结论rhGH治疗能显著改善TS患儿的FAH，但疗效存在个体差异，以按性激素治疗前单纯rhGH治疗的疗程及PAH。SDS为FAH的最主要影响因素，核型也可能对FAH有所影响。故对于TS的患儿采用rhGH治疗起始年龄宜早，性激素启动青春发育前的rhGH疗程宜长，FAH才更获益。Objective To observe the final adult height （FAH） outcome and influencing factors in Turner＇s Syndrome（TS） children treated with recombinant human growth hormone （rhGH）. Methods Thirty TS children treated with rhGH were compared with 16 TS children without rhGH treatment and were followed up to achieve their FAH. Comparisons were made regarding predicted adult height （ PAH ）, height standard deviation score for chronological age （HtSDScs）, height SDS for BA （HtSDSBA）, and growth velocity （GV）between rhGH treatment and without treatment groups and between the onset and by the end of rhGH treatment group. The factors determining FAH were also evaluated. Results FAH in rhGH treatment group was obviously improved as compared with untreatment group[ （ 149. 5±6. 3 vs 142. 4±5.2） cm, P〈0. 01 ]. FAH in treatment group was positively correlated with height standard deviation score for chronological age （ Ht0 SDScA ）, Hto SDS for BA （ Ht0 SDSBA ）, height age （ HA0 ） at preliminary diagnosis, and correlated with duration of rhGH therapy, duration of estrogen-free rhGH therapy, and PAHoSDS at preliminary diagnosis. Stepwise regression analysis indicated that duration of estrogen-free rhGH therapy and PAH0 SDS were the variables with the greatest identified influence on FAH （F = 11.56 and F = 86. 91, P〈 0. 01 ）. FAH in the 45, XO group was significantly different from the mosaicism group （45, XO/46, XX） [ （ 147.2 ± 6. 3 vs 153.3±6.4） cm,P =0. 038]. Conclusion rhGH treatment is efficacious in improving FAH of TS children, but a variability in the magnitude of the response to rhGH is recognized. Duration of estrogen-free rhGH therapy and PAHoSDS are the variables with the greatest identified influence on FAH, and karyotype may be one of the influence factors, rhGH treatment should be initiated as early as possible and sufficient course of estrogen-free rhGH therapy is needed to yield a satisfactory FAH.

关 键 词：重组人生长激素 TURNER综合征 成年终身高 影响因素 

分 类 号：R725.8[医药卫生—儿科]