Massa G, Maes M, Heinrichs C, Vandeweghe M, Craen M, Vanderschueren-Lodeweyckx M
Department of Paediatrics, University of Leuven, Belgium.
Clin Endocrinol (Oxf). 1993 Mar;38(3):253-60. doi: 10.1111/j.1365-2265.1993.tb01003.x.
The aim was to evaluate the effect of 3 years treatment with recombinant human growth hormone (rhGH) on height velocity and height in girls with Turner's syndrome (TS) and to study to influence of spontaneous or induced puberty on the growth promoting effect of rhGH.
The investigation was performed in 36 girls with Turner's syndrome treated for 3 years with rhGH in a dose of 1 IU/kg week, administered as daily subcutaneous injections. Fifteen patients remained prepubertal throughout the observation period (Group 1). During the first 2 years of rhGH therapy, four girls developed puberty spontaneously (Group 2). During the 3rd year of rhGH treatment puberty was induced with 100 ng/kg day ethinyl oestradiol orally in 17 girls requesting pubertal development and with a bone age of at least 11 'years' (Group 3).
During the first year of rhGH therapy height velocity increased significantly in all patients. Mean +/- SD height velocity was higher in the four patients with Turner's syndrome who developed spontaneous puberty than in 17 age-matched girls with Turner's syndrome without puberty (8.9 +/- 1.2 vs 7.4 +/- 1.2 cm/year; P < 0.05). During the second and third year of rhGH treatment height velocity decreased in all patients but remained above baseline levels. The induction of puberty with 100 ng/kg day ethinyl oestradiol in the patients of Group 3 did not lead to an acceleration of height velocity, but seemed in contrast to decelerate height velocity. After 3 years of rhGH treatment, 21 out of 36 patients have obtained a height at or above the initially calculated projected adult height and five girls are already taller than 150 cm.
The onset of spontaneous puberty during the first years of rhGH treatment seems to have an additive effect to rhGH on height velocity. Induction of puberty with oral administration of 100 ng/kg day ethinyl oestradiol did not have any beneficial effect on height velocity and seems therefore not to be the optimal way to induce puberty with an adequate pubertal growth spurt in girls with Turner's syndrome under rhGH therapy. Different doses and routes of oestrogen administration have to be evaluated in order to mimic the growth promoting effect of spontaneous puberty as well as possible.
本研究旨在评估重组人生长激素（rhGH）治疗3年对特纳综合征（TS）女孩身高增长速度及身高的影响，并研究自然青春期或诱导青春期对rhGH促生长作用的影响。
本研究对36例接受rhGH治疗3年的特纳综合征女孩进行了调查，rhGH剂量为1IU/kg周，每日皮下注射。15例患者在整个观察期内仍处于青春期前状态（第1组）。在rhGH治疗的前2年，4例女孩自然进入青春期（第2组）。在rhGH治疗的第3年，17例要求青春期发育且骨龄至少为11岁的女孩口服100ng/kg日乙炔雌二醇诱导青春期（第3组）。
在rhGH治疗的第1年，所有患者的身高增长速度均显著增加。4例自然进入青春期的特纳综合征患者的平均±标准差身高增长速度高于17例年龄匹配的未进入青春期的特纳综合征女孩（8.9±1.2 vs 7.4±1.2cm/年；P<0.05）。在rhGH治疗的第2年和第3年，所有患者的身高增长速度均下降，但仍高于基线水平。第3组患者口服100ng/kg日乙炔雌二醇诱导青春期并未导致身高增长速度加快，反而似乎使其减慢。rhGH治疗3年后，36例患者中有21例达到或超过最初计算的预计成人身高，5例女孩身高已超过150cm。
在rhGH治疗的最初几年自然青春期的开始似乎对rhGH的身高增长速度有累加作用。口服100ng/kg日乙炔雌二醇诱导青春期对身高增长速度没有任何有益影响，因此似乎不是在rhGH治疗下诱导特纳综合征女孩出现足够青春期生长突增的最佳方法。必须评估不同剂量和途径的雌激素给药，以尽可能模拟自然青春期的促生长作用。
