Haeusler G, Frisch H
Department of Pediatrics, University of Vienna, Austria.
Clin Endocrinol (Oxf). 1992 Mar;36(3):247-53. doi: 10.1111/j.1365-2265.1992.tb01440.x.
The effect of GH administration on various metabolic parameters and on growth and bone age development was studied in patients with Turner's syndrome.
Patients were treated with daily s.c. GH (20 IU/m2/week) and ethinyloestradiol p.o. (100 ng/kg/day) during the first year and with additional oxandrolone (0.125 mg/kg/day) during the second year. The responses of free fatty acids (FFA), urinary excretion of hydroxyproline (HP) and IGF-I were evaluated after short-term GH application. Glucose tolerance was investigated before any therapy, during treatment with GH and oestradiol and after adding oxandrolone, respectively. The course of growth, bone age and IGF-I levels was followed throughout the study.
Eleven patients with Turner's syndrome aged 12.6 +/- 1.9 years (mean +/- SD) were included.
Free fatty acids increased significantly 4 hours after one s.c. injection of GH (0.7 +/- 0.2-1.1 +/- 0.3 mmol/l; mean +/- SD). Mean urinary hydroxyproline excretion remained unchanged after 6 weeks of GH therapy (337 +/- 206-299 +/- 145 mumol/m2/24 h), but there was a significant negative correlation between individual hydroxyproline values and the peak serum GH followed stimulation. IGF-I was in the prepubertal range and increased significantly after 3 days of GH injection (30.0 +/- 10.0-42.5 +/- 10.0 nmol/l). Growth velocity (in Turner's syndrome related SD) increased from 0.0 +/- 0.3 SD before treatment to 0.9 +/- 0.8 SD after the first year and to 3.4 +/- 1.3 SD during the second year of treatment. There was no undue acceleration of bone age. During long-term treatment, IGF-I increased significantly only when oxandrolone was added. Two patients had impaired glucose tolerance prior to GH therapy and three additional children developed impaired or abnormal glucose tolerance after GH therapy. Insulin concentrations increased significantly only after introduction of oxandrolone.
Patients with Turner's syndrome who had lower basal IGF-I levels had significantly higher responses of IGF-I, free fatty acids and hydroxyproline (P less than 0.01 for all parameters) after short-term GH application. The data indicate adequate endocrine and metabolic responses in patients with Turner's syndrome which are the basis for growth promoting action. A considerable number of patients had impaired glucose tolerance during GH treatment.
研究生长激素（GH）治疗对特纳综合征患者各种代谢参数以及生长和骨龄发育的影响。
患者在第一年接受每日皮下注射GH（20 IU/m²/周）和口服乙炔雌二醇（100 ng/kg/天）治疗，第二年额外加用氧雄龙（0.125 mg/kg/天）。短期应用GH后评估游离脂肪酸（FFA）、羟脯氨酸（HP）尿排泄量和IGF-I的反应。分别在任何治疗前、GH和雌二醇治疗期间以及加用氧雄龙后研究葡萄糖耐量。在整个研究过程中跟踪生长、骨龄和IGF-I水平的变化。
纳入11例年龄为12.6±1.9岁（均值±标准差）的特纳综合征患者。
一次皮下注射GH后4小时，游离脂肪酸显著增加（0.7±0.2 - 1.1±0.3 mmol/L；均值±标准差）。GH治疗6周后，平均尿羟脯氨酸排泄量保持不变（337±206 - 299±145 μmol/m²/24小时），但个体羟脯氨酸值与刺激后血清GH峰值之间存在显著负相关。IGF-I处于青春期前范围，GH注射3天后显著增加（30.0±10.0 - 42.5±10.0 nmol/L）。生长速度（以特纳综合征相关标准差表示）从治疗前的0.0±0.3标准差增加到第一年治疗后的0.9±0.8标准差，第二年治疗期间增加到3.4±1.3标准差。骨龄没有过度加速。在长期治疗期间，仅在加用氧雄龙时IGF-I显著增加。2例患者在GH治疗前葡萄糖耐量受损，另外3例儿童在GH治疗后出现葡萄糖耐量受损或异常。仅在加用氧雄龙后胰岛素浓度显著增加。
基础IGF-I水平较低的特纳综合征患者在短期应用GH后，IGF-I、游离脂肪酸和羟脯氨酸的反应显著更高（所有参数P均小于0.01）。数据表明特纳综合征患者有适当的内分泌和代谢反应，这是生长促进作用的基础。相当数量的患者在GH治疗期间葡萄糖耐量受损。
