PRIMARY GONADAL INSUFFICIENCY IN A GIRL AND A BOY: METABOLIC EFFECTS OF ESTROGEN AND TESTOSTERONE

Abstract

IN GIRLS ovarian development may apparently become arrested at an early stage and give rise to lack of normal sex characteristics. This phenomenon is usually accompanied by certain congenital abnormalities. The syndrome to be discussed, recently reviewed by del Castillo and Argonz (1), was first presented by Funke (2) under the title of “Pterygium colli.” Subsequently a series of reports appeared in the continental literature and cases in which there were autopsy or biopsy records have been reviewed by Wilkins and Fleischmann (3). These authors presented 6 patients from the literature who were the subjects of autopsy reports and 2 on whom biopsy had been reported. These 8 all showed vestigial streaks in place of ovaries and no evidence of germinal epithelium or primordial follicles. In 1938 Turner (4) described 7 girls aged 15 to 23 years and since that time study of the condition has been greatly accelerated. The following titles have been used to describe the syndrome: 1. “Syndrome of infantilism, congenital webbed neck and cubitus valgus”; Turner (4). 2. “Primary ovarian insufficiency and decreased stature”; Albright, Smith and Fraser (5). 3. “Association of short stature, retarded sexual development and high urinary gonadotropin titers in women; ovarian dwarfism”; Varney, Kenyon and Koch (6). 4. “Ovarian agenesis”; Wilkins and Fleischmann (3). 5. “Syndrome of rudimentary ovaries with estrogenic insufficiency and increased gonadotropins”; del Castillo, de la Baize and Argonz (7). 6. “Syndrome of congenitally aplastic ovaries with sexual infantilism, high urinary gonadotropins, short stature and other congenital abnormalities”; Lisser, Curtis, Escamilla and Goldberg (8). These titles serve to show the variation of description used for this syndrome since Turner's paper and to indicate important contributions to its under-standing.