抄録
A rare autopsy case of congenital adrenal hyperplasia combined with agenesis of the gonad and adnexa was reported. The patient was a newborn with negative sex chromatin and the normal external female genitalia. The hyperplastic adrenocortical tissues were generally composed of spongy cells with tremendous accumulation of birefringent crystaline materials. Small lesions containing large crystaline clefts occasionally surrounded by a granulomatous cell-infiltration were additional findings. The adrenal findings were different from those of usual congenital adrenal hyperplasia but similar to those of the case with male pseudohermaphroditism reported by Gurtner (1955). Multifocal adenomatous hyperplasia of the basophils was evident in the anterior pituitary.
