Syndrome of Rudimentary Ovaries With Estrogenic Insufficiency and Increase in Gonadotropins

The Symmetrical Form of Status Bonnevie-Ullrich (Turner's Syndrome)

“…Numerous similar cases have since been reported by others (Sharpey-Schafer, 1941;Albright et al, 1942;Varney et al, 1942;Wilkins and Fleischmann, 1944;Lisser et al, 1947;Del Castillo et al, 1947;Dorff et al, 1947;Jackson, 1950;Jackson and Sougin-Mibashan, 1953). Congenital anomalies observed by these authors, in addition to the webbed neck, cubitus valgus, and short stature emphasized by Turner, included the low hair-line at the nape of the neck, dystrophies of the ears, " shield " chest, arched palate, scoliosis of the spine, and coarctation of the aorta.…”

Cytogenetic and Clinical Study on 100 Cases of Primary Amenorrhoea

“…Thus, the primary insufficiency was not pituitary but ovarian. Histological studies also showed (Wilkins andFleischman 1944, del Castillo andArgonz 1957) that, instead of normal ovaries, these patients had light-coloured ))streak, gonads composed of connective tissue similar to the ovarian stroma but containing no germinal elements.…”

Gonadal Dysgenesis in Normal-looking Females

“…It has been recognized that patients may lack many of the classical features which have been associated with the syndrome. There are numerous reports of tall patients and even of attractive ones (Wilkins and Fleischmann, 1944;Lisser et al, 1947;del Castillo et al, 1947;Kerkhof and Stolte, 1956;Greenblatt et al, 1956;Hoffenberg and Jackson, 1957), axillary and pubic hair are often normal in amount or even luxuriant (Hoffenberg and Jackson, 1957). The congenital anomalies of Turner's syndrome need not be present (del Castillo et al, 1947;Grumbach et al, 1955;Greenblatt et al, 1956) and good breast development has been reported (Varney et al, 1942;Wilkins and Fleischmann, 1944;Lisser et al, 1947;Hertz et al, 1950;Hoffenberg and Jackson, 1957).…”

“…There are numerous reports of tall patients and even of attractive ones (Wilkins and Fleischmann, 1944;Lisser et al, 1947;del Castillo et al, 1947;Kerkhof and Stolte, 1956;Greenblatt et al, 1956;Hoffenberg and Jackson, 1957), axillary and pubic hair are often normal in amount or even luxuriant (Hoffenberg and Jackson, 1957). The congenital anomalies of Turner's syndrome need not be present (del Castillo et al, 1947;Grumbach et al, 1955;Greenblatt et al, 1956) and good breast development has been reported (Varney et al, 1942;Wilkins and Fleischmann, 1944;Lisser et al, 1947;Hertz et al, 1950;Hoffenberg and Jackson, 1957). The urinary gonadotrophin, while generally high, may be normal or low (Dorff et al, 1947;Hertz et al, 1950;Sternlieb et al, 1954;Carpentier et al, 1956;Hoffenberg and Jackson, 1957); cases have been described with androgenic manifestations (Gordan et al, 1955;Greenblatt et al, 1956), and one proved case showed pseudohermaphroditic external genitals (Grumbach et al, 1955); finally, we have encountered two patients who claimed to have menstruated for five and six years respectively (Hoffenberg et al, 1957).…”