Haeusler G, Schmitt K, Blümel P, Plöchl E, Waldhör T, Frisch H
Paediatric Department, University of Vienna, Austria.
Acta Paediatr. 1996 Dec;85(12):1408-14.
Twenty patients with Turner syndrome (CA 7.3-16.4 years) were treated with growth hormone (GH) alone (12-18 IU/m2/week) for 0.9-2.5 years. Subsequently, all patients received GH 18 IU/m2/week in combination with oxandrolone (Ox) (0.0625 mg/kg/day po) or low dose testosterone (5 mg every 2 weeks, i.m.). Ethinylestradiol (50 ng/kg/day po) was started with a bone age of 12.5 "years", and the dose was increased stepwise to 200 ng/kg/day during the next 18 months. Final height (FH) after 4-7.7 years of therapy was 152.9 +/- 3.5 cm (range 145.0-158.9 cm). When compared to projected adult height (PAH) at start of therapy (143.7 +/- 4.0, range 137.5-151.0 cm), the estimated benefit from therapy (FH minus PAH) is 9.3 +/- 4.9 cm (range 1.4-21.4 cm). The wide range in individual responses may be due to over- or underestimation of PAH before therapy due to variable delay in bone age at start of therapy. FH did not differ between starting therapy before 11.5 years (n = 9; 152.0 +/- 3.4 cm) and after the age of 11.5 years (n = 11; 153.7 +/- 3.6 cm), due to the fact that a better short-term response to therapy in the younger group of patients was compensated for by a faster progression in bone age. The good result in terms of final height may be due in part to the late start (BA 13.3 +/- 0.4 years; range 12.7-14.5 years) of estrogen therapy in low doses.
20名特纳综合征患者（年龄7.3 - 16.4岁）先单独使用生长激素（GH）（12 - 18 IU/m²/周）治疗0.9 - 2.5年。随后，所有患者接受GH 18 IU/m²/周联合氧雄龙（Ox）（0.0625 mg/kg/天，口服）或低剂量睾酮（每2周5 mg，肌肉注射）治疗。在骨龄达到12.5“岁”时开始口服乙炔雌二醇（50 ng/kg/天），并在接下来的18个月内逐步增加剂量至200 ng/kg/天。治疗4 - 7.7年后的最终身高（FH）为152.9±3.5 cm（范围145.0 - 158.9 cm）。与治疗开始时的预测成人身高（PAH）（143.7±4.0，范围137.5 - 151.0 cm）相比，治疗的估计获益（FH减去PAH）为9.3±4.9 cm（范围1.4 - 21.4 cm）。个体反应差异较大可能是由于治疗开始时骨龄延迟不同导致治疗前PAH估计过高或过低。在11.5岁之前开始治疗的患者（n = 9；152.0±3.4 cm）和11.5岁之后开始治疗的患者（n = 11；153.7±3.6 cm）之间，FH没有差异，这是因为较年轻患者组对治疗的短期反应较好，但被骨龄更快进展所抵消。最终身高方面的良好结果可能部分归因于低剂量雌激素治疗开始较晚（骨龄13.3±0.4岁；范围12.7 - 14.5岁）。
