Massarano A A, Brook C G, Hindmarsh P C, Pringle P J, Teale J D, Stanhope R, Preece M A
Endocrine Unit, Middlesex Hospital, London.
Arch Dis Child. 1989 Apr;64(4):587-92. doi: 10.1136/adc.64.4.587.
We investigated 24 hour growth hormone secretion by intermittent 20 minute blood sampling in 34 prepubertal patients with Turner's syndrome, aged 4.3-12.4 years. Growth hormone profiles were analysed by the PULSAR programme and results expressed as the sum of growth hormone pulse amplitudes. Six patients had abnormal growth hormone pulse frequencies. In the remaining 28, growth hormone pulse amplitudes declined significantly with increasing age, but there was no correlation between growth hormone pulse amplitudes and growth rates. Concentrations of insulin like growth factor-1 (IGF-1) rose with age but did not correlate with either growth rates or growth hormone secretion. Fifteen patients were given oxandrolone and 11 low dose ethinyl oestradiol. Both agents increased height velocity without increasing growth hormone secretion. We conclude that the relation between growth hormone secretion and growth in Turner's syndrome is less certain than in normal children. End organ resistance is probably due to a skeletal dysplasia. Both oxandrolone and low dose ethinyl oestradiol improve the growth of girls with Turner's syndrome, but their mechanism of action remains uncertain.
我们通过对34名4.3至12.4岁青春期前特纳综合征患者每隔20分钟进行一次采血,研究其24小时生长激素分泌情况。采用PULSAR程序分析生长激素谱,结果以生长激素脉冲幅度总和表示。6名患者生长激素脉冲频率异常。在其余28名患者中,生长激素脉冲幅度随年龄增长显著下降,但生长激素脉冲幅度与生长速率之间无相关性。胰岛素样生长因子-1(IGF-1)浓度随年龄升高,但与生长速率或生长激素分泌均无相关性。15名患者接受了氧雄龙治疗,11名患者接受了低剂量炔雌醇治疗。两种药物均提高了身高增长速度,但未增加生长激素分泌。我们得出结论,特纳综合征患者生长激素分泌与生长之间的关系不如正常儿童明确。终末器官抵抗可能是由于骨骼发育异常所致。氧雄龙和低剂量炔雌醇均可改善特纳综合征女孩的生长,但它们的作用机制仍不确定。