[Gonadal dysgenesis]

The authors describe six patients, female phenotype with karyotype 45X/46XY. Two girls suffered from pure gonadal dysgenesis, four from mixed gonadal dysgenesis. The authors emphasize the intact axis hypothalamus-pituitary gonads. In the above diagnoses the authors recommend revision and extirpation of the gonads with regard to possible malignant growth in the dysgenetic gonad.