Pure gonadal dysgenesis is diagnosed in the presence of:

Patients with pure gonadal dysgenesis have bilateral streak gonads without any potentiality to produce hormones. In pure gonadal dysgenesis patients usually presents as primary amenorrhea with delayed development of secondary sex characters. They have an average stature. Vagina, uterus and tubes are present but infantile. As these patents have no gonads a female phenotype is expected regardless of the chromosomal complement. Their karyotype is either 46 XX or 46 XY. Ref: Textbook of Gynecology By D.C. Dutta, 4th Edition, Page 40 ; Harisson's Internal Medicine, 18th Edition, Page 3051