MALE PSEUDOHERMAPHRODITISM SECONDARY TO PANHYPOPITUITARISM

An infant with a 46XY karyotype was born with ambiguous genitalia, inc luding microphallus and perineal hypospadias. A female gender was assi gned due to extreme failure of development of the external genitalia. Subsequent investigations demonstrated panhypopituitarism, and it is b elieved that severe gonadotrophin deficiency was responsible for the i ntersex state. This case illustrates the need to evaluate the hypothal amic-pituitary axis in selected cases of intersex, and also questions the prevailing assumption that testosterone secretion during embryogen esis is largely pituitary gonadotrophin independent, under the control of human chorionic gonadotrophin.