Four cases of XX males: endocrine and clinical features

G-banded chromosome preparations from four phenotypically male patients resulted azoospermic at semen analysis, showed no evidence of Y chromosome in 46,XX female constitution. At the andrological examination, the only common finding in them was the presence of very small testes. Serum levels of basal LH, FSH, prolactin and testosterone were evaluated. A subnormal response by testicular Leydig cells to HCG was observed. The LH and FSH responses to LHRH were similar to those of XXY controls, and the variation of prolactin levels after sulpiride stimulus was in the normal range. Seminal, histological and endocrine investigations show the primitive origin of the hypogognadism.