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The effect of hormone replacement therapy on cardiovascular hemodynamics in women with Turner's syndrome

JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM 2000年第2期85卷 614-618页

作者： Elsheikh, M Bird, R Casadei, B Conway, GS Wass, JAH Radcliffe Infirm Dept Endocrinol Oxford OX2 6HE England John Radcliffe Hosp Dept Cardiovasc Med Oxford England Middlesex Hosp Dept Endocrinol London OX3 7XP England

Women with Turner's syndrome, the majority of whom are estrogen deficient, have an increased incidence of coronary artery disease. The aim of this study was to assess the effects of hormone replacement therapy (HRT) on central arterial hemodynamics, insulin sensitivity, and lipids in adults with Turner's syndrome. Twenty-one women with Turner's syndrome were studied prospectively, on and off 3 months of estradiol valerate in combination with levonorgestrel. The following measurements were made: body mass index, waist/hip ratio, serum lipids, fasting insulin and glucose, and mean arterial blood pressure. Aortic root pressure and waveforms were estimated noninvasively and the augmentation index (AI), a measure of aortic stiffness, was calculated. The AT was significantly lower during estrogen therapy (22% vs. 15%;P = 0.008), suggesting a reduction in central arterial stiffness. Fasting insulin and glucose concentrations were also significantly lower during HRT (P = 0.01 and P = 0.0004, respectively). There was no difference in body mass index, serum Lipids, or brachial and aortic blood pressures on and off treatment. Total cholesterol was correlated with the AI (r = 0.4;P = 0.03). These results suggest that HRT in women with Turner's syndrome has a favorable effect on central arterial hemodynamics and insulin sensitivity. The lack of effect on serum lipids suggests that the effects of HRT on aortic compliance may be mediated by an improvement in endothelial function.

关键词：主动脉/病理生理学血压心血管系统/病理生理学弹性雌二醇/治疗应用雌激素替代疗法心率左炔诺孕酮/治疗应用脂类/血液心肌缺血/预防和控制孕酮同源物/治疗应用回顾性研究特纳综合征/病理学特纳综合征/病理生理学青少年成年人女(雌)性人类中年人

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Longitudinal analysis of growth over the first 3 years of life in Turner's syndrome

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JOURNAL OF PEDIATRICS 2000年第4期137卷 460-464页

作者： Even, L Cohen, A Marbach, N Brand, M Kauli, R Sippell, W Hochberg, Z Bnai Zion Med Ctr Dept Pediat Haifa Israel Technion Israel Inst Technol Fac Ind Engn & Management IL-32000 Haifa Israel Univ Kiel Dept Paediat Kiel Germany Schneider Pediat Med Ctr Dept Endocrinol Petah Tiqwa Israel Rambam Med Ctr Dept Pediat Haifa Israel

Objective: To evaluate longitudinal growth in Turner's syndrome (TS) over the first 3 years of life. Methods: Growth of 47 patients with TS was compared with that of 40 age-matched control girls by using an analysis according to the Infancy-Childhood-Puberty and bi-exponential models. Results: A mean of 1.2 SDs were lost before birth and a total of 3.0 SDs were lost by age 3 years. According to the Infancy-Childhood-puberty model, intrauterine growth retardation contributed -1.24 SDs, a 5-month delay in childhood growth spurt contributed -0.96 SDs, and slow childhood growth contributed an additional -0.8 SDs by age 3 years. The bi-exponential analysis disclosed a quasi-linear first exponent and a confining second exponent, which merged at age 18 months in control subjects and 24 months in patients with TS. The first exponent confers an average annual growth rate of 8.4 cm/y in control subjects and 6.7 cm/y in patients with TS. Conclusions: Intrauterine growth retardation and the initial 3 Sears of life contribute most of the deficit in the final height of patients with TS. These data provide a reference of standards for longitudinal growth in patients with TS at age 3 months to 3 years.

关键词：生长纵向研究模型统计学特纳综合征/病理生理学儿童学龄前女(雌)性人类婴儿婴儿新生

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Osteoporosis and fractures in Turner syndrome - importance of growth promoting and oestrogen therapy

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CLINICAL ENDOCRINOLOGY 1999年第4期51卷 497-502页

作者： Landin-Wilhelmsen, K Brymant, I Windh, M Wilhelmsens, L Sahlgrens Univ Hosp Res Ctr Endocrinol & Metab S-41345 Gothenburg Sweden Sahlgrens Univ Hosp Dept Gynaecol S-41345 Gothenburg Sweden Sahlgrens Univ Hosp Sect Prevent Cardiol S-41345 Gothenburg Sweden

OBJECTIVE Turner syndrome (TS) is a chromosomal aberration (45,X) characterized by endogenous oestrogen deficiency and short stature. The aim was to study body composition, bone mineral density, fracture frequency, social and life style factors and biochemical bone markers, as well as hormones, in adults with TS in comparison with a female random population sample. PATIENTS Seventy women with TS responded to questionnaires. They underwent physical examination, bone mineral density measurement with Dual Energy X-ray Absorptiometry (DEXA) and blood sampling. Mean age was 31 +/- 12 (range 16-71) years. A random population sample of women from the WHO MONICA Project, Goteborg (25-64 years) served as controls (n = 740). RESULTS Women with TS were shorter than the controls and had lower body weight and lean body mass (P < 0.0001). Body mass index and waist/hip circumference ratio were higher in TS (P < 0.0001). Osteoporosis was present in seven TS women, six above 45 years of age. None of these had received oestrogen substitution continuously. Fractures tall types) were reported by 11 (16%) TS women (six (50%) above 45 years) compared with 5% in the population sample (P < 0.001). Four TS women with fractures had osteoporosis, all above 45 years of age. Osteoporosis and fractures did not differ between women with the 45,X karyotype and those with mosaicism. Impaired hearing was reported by 40%, and 73% wore glasses. Six percent among TS were smokers compared with 25% in the population (P < 0.001). TS women reported a lower degree of leisure time physical activity than controls (P < 0.001). Parathyroid hormone and osteocalcin were higher among TS (P < 0.02 and 0.001). Insulin-like growth factor-1 was similar. Ninety-one percent of all TS had oestrogen substitution and 98% of TS below 25 years of age had received growth hormone treatment. CONCLUSION Osteoporosis and fractures were common above, but not below, 45 years of age in Turner syndrome. It is probable that modern the

关键词：身体成分身高人体质量指数体重骨密度病例对照研究雌激素替代疗法骨折/血液骨折/病因学骨折/病理生理学生长激素/治疗应用听力障碍/血液听力障碍/并发症听力障碍/病理生理学胰岛素样生长因子Ⅰ/分析骨钙素/血液骨质疏松/血液骨质疏松/并发症骨质疏松/病理生理学甲状旁腺素/血液特纳综合征/血液特纳综合征/并发症特纳综合征/病理生理学青少年成年人老年人女(雌)性人类中年人

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Combination growth hormone and estrogen increase bone mineralization in girls with Turner Syndrome

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PEDIATRIC RESEARCH 1999年第5期45卷 709-713页

作者： Beckett, PR Copeland, KC Flannery, TK Sherman, LD Abrams, SA Texas Childrens Hosp Dept Pediat Endocrinol & Metab Sect Houston TX 77030 USA Baylor Univ USDA ARS Childrens Nutr Res Ctr Houston TX 77030 USA

Turner syndrome is characterized by osteopenia and impaired skeletal growth. Neither feature is normalized by current modes of hormone therapy. The purpose of this study was to determine whether GH would increase protein anabolism and provide additional benefit to a regimen of estrogen replacement on calcium metabolism in girls and women with Turner syndrome. Using stable isotopes of calcium and leucine, we determined calcium absorption, urinary calcium loss, calcium retention, deposition into bone, leucine rate of appearance from protein, leucine incorporation into protein, and leucine oxidation in seven girls (10-17 y of age) and four adult females (16-34 y of age) with Turner syndrome, before and after 3 mo of GH treatment. All adults were treated with estrogen (ethinyl estradiol, 50 mu g/d) and progesterone before and throughout the study. Three girls received no estrogen, and four girls were treated with low-dose estrogen (ethinyl estradiol, 5 mu g/d) in combination with GH. The addition of estrogen to GH treatment resulted in a significant increase in calcium absorption and deposition in girls. GH did not affect calcium kinetics in adults already receiving estrogen/progesterone replacement therapy, nor did GH alone affect calcium kinetics in girls, and neither GH nor estrogen affected protein metabolism. These data suggest that the addition of low-dose estrogen to a regimen of GH improves bone deposition and calcium metabolism in girls with Turner syndrome and that estrogen is facultative for GH effects on bone.

关键词：钙化生理性/药物作用钙/代谢药物疗法联合雌二醇同源物/治疗应用炔雌醇/治疗应用激素替代疗法人生长激素/治疗应用胰岛素样生长因子结合蛋白质1/血液胰岛素样生长因子结合蛋白质3/血液胰岛素样生长因子Ⅰ/代谢孕酮/治疗应用特纳综合征/病理生理学特纳综合征/治疗青少年成年人儿童女(雌)性人类

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ABNORMALITIES OF OVARIAN-FUNCTION

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CLINICS IN OBSTETRICS AND GYNAECOLOGY 1976年第1期3卷 181-194页

作者： MACNAUGHTON, MC UNIV GLASGOW GLASGOW G12 8QQ SCOTLAND GLASGOW ROY MATERN HOSP GLASGOW SCOTLAND ROY INFIRM GLASGOW G4 0SF SCOTLAND

This chapter deals with some of the abnormalities of function of the human ovary. The ovary is an intermediate organ in the preparation of the endometrium for implantation of the ovum and it is also an end organ as far as the process of ovulation is concerned. It is acted upon by gonadotrophic hormones secreted by the anterior pituitary gland, the stimulus from these hormones resulting in follicle growth and ovulation. Abnormalities of function of the ovary may therefore occur in two ways: by the presence of a particular ovarian abnormality which makes it unable to respond to the stimulus coming from the anterior pituitary gland (primary ovarian failure), or by a failure of the stimulus to the ovary resulting in failure of growth of the graafian follicle and ovulation, so that there is no stimulus to the endometrium (secondary ovarian failure).

关键词：雄激素类/代谢氯米芬/治疗应用雌激素类/代谢雌激素类/治疗应用炔雌醇/治疗应用不育女(雌)性卵巢疾病卵巢功能试验卵巢/代谢卵巢/病理生理学多囊卵巢综合征/药物疗法多囊卵巢综合征/外科学孕酮/代谢综合征特纳综合征/药物疗法特纳综合征/病理生理学女(雌)性人类

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GONADOTROPIN-SECRETION IN PREPUBERTAL AND PUBERTAL PRIMARY HYPOGONADISM - RESPONSE TO LHRH

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JOURNAL OF CLINICAL ENDOCRINOLOGY & METABOLISM 1975年第6期41卷 1070-1075页

作者： DEBEHAR, BR MENDILAHARZU, H RIVAROLA, MA BERGADA, C CTR INVEST ENDOCRINOL BUENOS AIRES ARGENTINA HOSP NINOS BUENOS AIRES ARGENTINA

Plasma LH and FSH were measured before and after LHRH administration in 10 patients with Turner's syndrome, in 7 with anorchia, in 2 castrates, in 18 with Klinefelter's syndrome, and in 11 prepubertal subjects with unilateral cryptochidism used as a control group. Basal LH was elevated in 4 and basal FSH in 8 of 10 patients with Turner's syndrome. Four patients with anorchia showed elevations of LH while FSH had increased in all of them. The two castrates had normal or slightly increased basal LH and definite elevations of FSH. Prepubertal subjects with Klinefelter's syndrome had normal plasma LH and FSH levels, but showed a marked elevation when they developed puberty. After LHRH administration, mean LH increased by 297% and FSH by 81% in Turner's syndrome, while in anorchia LH increased 757% and FSH 104%. After LHRH administration, patients with unilateral cryptorchidism had an LH increment of 316% and a FSH increment of 164%. Patients with prepubertal Klinefelter's syndrome showed elevations of 261% for LH and 221% for FSH after LHRH treatment. Adolescent subjects with Klinefelter's syndrome had an increment of 352% for LH and only 13% for FSH after LHRH administration. We have concluded that patients without functioning gonads fail to suppress gonadotropin secretion even before puberty while the gonads of the prepubertal Klinefelter's syndrome are able to control LH and FSH release. After puberty, in spite of the hypersection of LH and FSH observed in all subjects with agonadism there is a large pituitary reserve of the gonadotropins. We suggest that the relative inability of pubertal patients with Klinefelter's syndrome to increase FSH after LHRH treatment might be due to the presence of an abnormal compound secreted by the gonads.

关键词：卵泡刺激素/血液卵泡刺激素/代谢促性腺素释放激素性腺功能减退症/病理生理学下丘脑-垂体系统/代谢 Klinefelter综合征/病理生理学促黄体激素/血液促黄体激素/代谢垂体/代谢青春期特纳综合征/病理生理学青少年成年人儿童女(雌)性人类男(雄)性

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