重组人生长激素对不同生长激素分泌状态青春前期矮小患儿近期疗效分析

Short term responses to recombinant human growth hormone(rhGH)treatment in prepubertal children with short stature and various GH secretary status

目的探讨部分性生长激素缺乏症(pGHD)患儿在重组人生长激素(rhGH)治疗后早期追赶性生长的规律。方法回顾性分析62例青春前期不同生长激素(GH)分泌状态矮小患儿用rhGH治疗后,近期(1.5年)追赶性生长指标(生长速度和身高Z分增值)和促生长素轴实验室指标的变化。其中,完全性生长激素缺乏症(cGHD)27例;非GH缺乏性矮小(NGHD)12例;pGHD 23例,按GH激发峰值7 ng/ml分为pGHD-1(12例)和pGHD-2(11例)两个亚组。结果cGHD和NGHD初始追赶性生长的幅度相似,但NGHD组持续时间较短。pGHD和cGHD以同一rhGH生理替代量治疗后,促生长的应答(生长速度和ΔIGFBP-3SDS)pGHD-1和cGHD差异无统计学意义,但pGHD-2却低于cGHD,而与NGHD差异无统计学意义。结论GH激发试验的诊断界值选用7 ng/ml有更合理的依据,诊断pGHD时尤应审慎。pGHD-2组治疗早期的生长追赶不完全可能与rhGH剂量相对不足有关。[临床儿科杂志,2007,25(12):988-992]

Objectives To explore the pattern of early catch-up growth of children with partial growth hormone deficiency （pGHD）, following rhGH treatment. Methods Sixty-two prepubertal children with short stature and various GH secretary status were analyzed retrospectively. Among them, 27 had complete GH deficiency （cGHD）, 23 with pGHD and 12 with non-GH deficiency （NGHD）. The group of pGHD was further divided into pGHD-1 （5 - 6.9 ng/ml） and pGHD-2 （7 - 9.9 ng/ml） according to the peak GH value in GH provocative test. Height velocity and increase in height SDS were studied. Serum levels of somatotrophic axis indexes were examined. Results Early quick catch-up growth was observed in children with cGHD and NGHD, while the response persisted for shorter period in NGHD. After being treated with same dose of rhGH, the difference was not significant in the responses to rhGH, height velocities, and AIGFBP-3SDS between pGHD-1 and cGHD. Children with pGHD-2 presented similar responses with NGHD, while the response was weaker than cGHD. Conclusions The cut-off value of GH provocative test in diagnosing GHD would be more reasonable at the level of 7 ng/ml. Caution should be taken when making diagnosis of pGHD. The incomplete ,catch-up growth of pGHD-2 might be associated with relatively inadequate rhGH dose.