| Abstrakt: |
The term gonadal dysgenesis has recently been suggested for a condition previously called ovarian agenesis since sex chromatin studies revealed that this disorder frequently occurs in genetic males (Polani et al., 1954, Wilkins et al., 1954, and Grumbach et al., 1955). This syndrome consists of stunted growth, primary amenorrhea, absence of secondary sex characteristics and other anomalies such as webbed neck, cubitus valgus and coarctation of the aorta. The gonads are absent or represented by vestigial streaks consisting histologically of stroma-like cells and fibrous tissue only. The most significant laboratory finding is a consistently high urinary gonadotrophin excretion (Albright et al., 1942). The patients appear to be females and their external genitalia are of a distinctly feminine pattern: vulva, vagina, uterus and Fallopian tubes are present, though hypoplastic.Two patients with gonadal dysgenesis are reported. In both the possibility of a male genetic sex was suspected because of a congenitally |
