1 Department of Pediatrics, Division of Pediatric Endocrinology, Cornell University Medical College, New York
In 1938, Turner1 described the syndrome of short stature and sexual infantilism. Since that report, the association of streak gonads and a variety of other developmental defects has become well recognized.2(p286) Psychosexually, girls with Turner's syndrome are said to be "the epitome of femininity and markedly maternal in their play and child care interests."3 Administration of hormones to induce breast development and menstrual cycles maintains and promotes their feminine identity4 and is recommended for the psychological well-being of the girl with Turner's syndrome.5
Continuous estrogen therapy to induce breast development has been recommended for initial treatment, followed by cyclical estrogen administration to permit periodic shedding of the endometrium and bleeding.5-7 The addition of a progestational drug has been recommended if the response to estrogen alone was unsatisfactory.5,7 More recently, however, the use of cyclic estrogen-progesterone regimens has been considered safer.8-10
Although there have been a number of reports on the effects of estrogens on bone age and growth,11-14 there have been few systematic studies of the hormonal effects of replacement therapy in Turner's syndrome.15-18 Rosenfield et al.17 measured the changes in circulating levels of estradiol, estrone, follicle-stimulating hormone (FSH), and luteinizing hormone (LH), sex hormone-binding globulin capacity, thyroxine-binding globulin capacity, and vaginal cytological pattern in five girls with Turner's syndrome and one girl with multiple endocrine deficiency syndrome following a single intramuscular injection of estradiol cypionate, 1.5 to 2.0 mg (Fig. 1). Plasma estradiol levels reached a peak three to seven days after the injection and fell steadily thereafter, reaching control levels at 18 to 20 days.