Biomedical subjects
A A Massarano
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Growth hormone secretion in patients with Turner's syndrome as determined by time series analysis.
Twenty-four-hour growth hormone (GH) profiles in 26 girls with Turner's syndrome were compared with those of 26 normally growing short children and 24 slowly growing short children. All children were prepubertal and below 12 years of age. A subgroup of 13 girls was treated with ethinyl estradiol and a 24-h GH profile was reassessed. In an additional group of 45 girls with Turner's syndrome (aged 6.7-18.9 years) the effect of age, spontaneous breast development and ethinyl estradiol treatment was studied. The profiles were assessed by Fourier analysis. The oscillatory activity and the mean 24-h GH concentration were similar in children with Turner's syndrome and the normally growing short children, in contrast to lower levels in the slowly growing short children. The periodicity of GH secretion was similar in all groups. In the longitudinal study, ethinyl estradiol treatment resulted in a significant increase in pulse amplitude, but not in periodicity. In the cross-sectional study there was no significant difference between the subgroups of girls with either presence or absence of breast development or ethinyl estradiol treatment. GH secretion was not significantly related to age, height in standard deviation score or height velocity. These data imply that there is no abnormality in GH secretion in girls with Turner's syndrome.
Growth hormone therapy and final height in girls with Turner's syndrome.
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Ovarian ultrasound appearances in Turner syndrome.
We performed pelvic ultrasound assessment in 104 patients with Turner syndrome aged 0.2 to 17.4 years; 69 had the 45,X karyotype and 35 had variant karyotypes. Ovarian appearances were classified as "streak" (n = 70, including 30 patients in whom no ovary could be seen) or "nonstreak" (n = 34). The nonstreak ovaries ranged from small glands, sometimes containing minute cysts, to ovaries indistinguishable from those which are normal for age. Nonstreak ovaries retained a range of function, as evidenced in some cases by spontaneous breast development and uterine enlargement. The proportion of nonstreak ovaries followed a U-shaped pattern, with a nadir from 4 to 10 years; this follows the known biphasic pattern of luteinizing hormone and follicle-stimulating hormone secretion. Only those patients with karyotype variants in which the long arm of the X chromosome was retained fared better than those with the 45,X karyotype.
Growth hormone secretion in Turner's syndrome and influence of oxandrolone and ethinyl oestradiol.
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.