Haeusler G, Frisch H, Schmitt K, Blümel P, Plöchl E, Zachmann M, Waldhör T
Paediatric Department, University of Vienna, Austria.
Eur J Pediatr. 1995 Jun;154(6):437-44. doi: 10.1007/BF02029351.
Thirty-nine girls with Ullrich-Turner syndrome (UTS) (median age 9.5 years) were treated with growth hormone (GH) with either 12 or 18 IU/m2 per week for 12 months followed by combination therapy with either oxandrolone (Ox) (0.0625 mg/kg/day po) or low-dose testosterone (T) (5 mg in every 2 weeks). Growth velocity improved significantly after 12 IU/m2 per week (6.4 +/- 1.7 cm/year vs 4.0 +/- 1.3 cm/year, x +/- SD, P < 0.001) and 18 IU/m2 per week of GH (6.5 +/- 1.3 cm/year vs 4.5 +/- 1.4 cm/year, P < 0.001). Ox, but not T was effective in maintaining growth velocity during the 2nd year of therapy (6.9 +/- 1.3 vs 5.3 +/- 1.5 cm/year). Basal insulin-like growth factor-I (IGF-I) concentrations were in the lower normal range and increased significantly in patients treated with 18 IU/m2 per week (357 +/- 180 ng/ml vs 160 +/- 84 ng/ml) and 12 IU/m2 per week (273 +/- 121 ng/ml vs 140 +/- 77 ng/ml). IGF-I concentrations increased further after addition of Ox (533 +/- 124 ng/ml, P < 0.001) or T (458 +/- 158, P < 0.05). IGFBP-3 concentrations were in the upper normal range before therapy and increased only moderately in both GH dosage groups. However, IGF binding protein-3 (IGFBP-3) concentrations were not affected by additional Ox or T treatment.
39名患有乌尔里希-特纳综合征（UTS）的女孩（中位年龄9.5岁）接受了生长激素（GH）治疗，每周剂量为12或18 IU/m²，持续12个月，随后分别接受氧雄龙（Ox）（口服0.0625 mg/kg/天）或低剂量睾酮（T）（每2周5 mg）的联合治疗。每周12 IU/m²（6.4±1.7 cm/年 vs 4.0±1.3 cm/年，x±标准差，P<0.001）和每周18 IU/m²的GH治疗后生长速度显著提高（6.5±1.3 cm/年 vs 4.5±1.4 cm/年，P<0.001）。在治疗的第二年，Ox可有效维持生长速度，但T无效（6.9±1.3 vs 5.3±1.5 cm/年）。基础胰岛素样生长因子-I（IGF-I）浓度处于较低正常范围，在每周接受18 IU/m²（357±180 ng/ml vs 160±84 ng/ml）和每周12 IU/m²治疗的患者中显著升高（273±121 ng/ml vs 140±77 ng/ml）。添加Ox（533±124 ng/ml，P<0.001）或T（458±158，P<0.05）后IGF-I浓度进一步升高。IGFBP-3浓度在治疗前处于较高正常范围，在两个GH剂量组中仅适度升高。然而，IGF结合蛋白-3（IGFBP-3）浓度不受额外Ox或T治疗的影响。
