CAJ | 학술논문

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( PAH.SDS)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PAHoSDS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PAHD 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.
목적 관찰중조인생장격소(rhGH)치료Turner종합정(TS)적료효병종합분석개선기성년종신고(FAH)상관적영향인소.방법 체FAH적TS환인이왕경rhGH치료조30례、미치료조16례,비교분석경치조여미치조、치료전후적예측성년신고적표준차분치( PAHSDS)、안년령적신고표준차분치( HtSDScA)、안골령적신고표준차분치(HtSDSBA)화생장속솔(GV)등생장삼수적변화,병분석영향FAH적인소.결과 rhGH치료조교미치조FAH유명현개선[(149.5±6.3대142.4±5.2)cm,P＜0.01],상관성분석현시치료조FAH여초진시HtSDScA、HtSDSBA、초진시적신고별년령、rhGH치료적료정、성격소치료전단순rhGH치료적료정화초진시PAHSDS( PAH.SDS)정정상관；축보회귀현시성격소치료전단순rhGH치료적료정화PAHoSDS시영향치료조환자FAH적독립인소(F분별위11.56화86.91,균P＜0.01)；차단체형조(45,XO)화감화형조(46,XX/45,XO)TS환인적FAH차이유통계학의의(P=0.038).결론 rhGH치료능현저개선TS환인적FAH,단료효존재개체차이,이안성격소치료전단순rhGH치료적료정급PAHD SDS위FAH적최주요영향인소,핵형야가능대FAH유소영향.고대우TS적환인채용rhGH치료기시년령의조,성격소계동청춘발육전적rhGH료정의장,FAH재경획익.
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( HtSDScA ),height SDS for BA( HtSDSRA ),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 ( Hto SDSBA ),height age ( HA0 ) at preliminary diagnosis,and correlated with duration of rhGH therapy,duration of estrogen-free rhGH therapy,and PAH0SDS 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 PAH0SDS 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.