Nilsson K O, Albertsson-Wikland K, Alm J, Aronson S, Gustafsson J, Hagenäs L, Häger A, Ivarsson S A, Karlberg J, Kriström B, Marcus C, Moell C, Ritzen M, Tuvemo T, Wattsgård C, Westgren U, Westphal O, Aman J
Department of Pediatrics, University Hospital Malmo, Sweden.
J Clin Endocrinol Metab. 1996 Feb;81(2):635-40. doi: 10.1210/jcem.81.2.8636281.
The spontaneous growth process in Turner's syndrome is characterized by a progressive decline in height velocity during childhood and no pubertal growth spurt. Therefore, therapy aimed at improving height during childhood as well as increasing final height is desirable for most girls with Turner's syndrome. Forty-five girls with Turner's syndrome, 9-16 yr of age (mean age, 12.2 yr), were allocated to three study groups. Group 1 (n = 13) was initially treated with oxandrolone alone; after 1 yr of treatment, GH without (group 1a; n = 6) or with (group 1b; n = 7) ethinyl estradiol was added. Group 2 (n = 17) was treated with GH plus oxandrolone. Group 3 (n = 15) was treated with GH, oxandrolone, and ethinyl estradiol. The dosage were: GH, 0.1 IU/kg.day; oxandrolone, 0.05 mg/kg.day; and ethinyl estradiol, 100 ng/kg.day. A height of 150 cm or more was achieved in 61%, 75%, and 60% of the girls in groups 1, 2, and 3, respectively. The most impressive increase in height was seen in group 2. In this group the mean final height was 154.2 cm (SD = 6.6), which is equivalent to a mean net gain of 8.5 cm (SD = 4.6) over the projected final height. In group 3, in which ethinyl estradiol was included from the start of therapy, the initially good height velocity decelerated after 1-2 yr of treatment. Their mean final height was 151.1 (SD = 4.6) cm, equivalent to a mean net gain of 3.0 cm (SD = 3.8). A similar growth-decelerating effect of ethinyl estradiol was seen in group 1b. We conclude that in girls with Turner's syndrome who are older than 9 yr of age, treatment with GH in combination with oxandrolone results in significant growth acceleration, imitating that in normal puberty, leading to a more favorable height during childhood. This mode of treatment also results in a significantly increased final height, permitting a great number of the girls to attain a final height of more than 150 cm. However, early addition of estrogen decelerates the height velocity and reduces the gain in height.
特纳综合征的自然生长过程的特点是儿童期身高增长速度逐渐下降，且没有青春期生长突增。因此，对于大多数特纳综合征女孩来说，旨在改善儿童期身高以及增加最终身高的治疗是很有必要的。45名9至16岁（平均年龄12.2岁）的特纳综合征女孩被分配到三个研究组。第1组（n = 13）最初仅用氧雄龙治疗；治疗1年后，添加生长激素（GH），其中不添加乙炔雌二醇的为第1a组（n = 6），添加乙炔雌二醇的为第1b组（n = 7）。第2组（n = 17）用生长激素加氧雄龙治疗。第3组（n = 15）用生长激素、氧雄龙和乙炔雌二醇治疗。剂量分别为：生长激素0.1 IU/kg·天；氧雄龙0.05 mg/kg·天；乙炔雌二醇100 ng/kg·天。第1、2、3组分别有61%、75%和60%的女孩身高达到150厘米或更高。身高增长最显著的是第2组。该组的平均最终身高为154.2厘米（标准差 = 6.6），比预计最终身高平均净增8.5厘米（标准差 = 4.6）。在第3组中，从治疗开始就加入了乙炔雌二醇，治疗1至2年后，最初良好的身高增长速度减缓。她们的平均最终身高为151.1（标准差 = 4.6）厘米，平均净增3.0厘米（标准差 = 3.8）。在第1b组中也观察到了乙炔雌二醇类似的生长减速作用。我们得出结论，对于9岁以上的特纳综合征女孩，生长激素与氧雄龙联合治疗可显著加速生长，模拟正常青春期的生长情况，使儿童期身高更理想。这种治疗方式还能显著增加最终身高，使大量女孩最终身高达到150厘米以上。然而，早期添加雌激素会减缓身高增长速度并减少身高增加量。
