SYNDROME PRODUCED BY ABSENCE OF THE GERMINAL EPITHELIUM WITHOUT IMPAIRMENT OF THE SERTOLI OR LEYDIG CELLS

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Abstract
Five cases are presented of a new syndrome, the chief clinical features of which are small testes and sterility in otherwise normal-appearing d* d[male][male]. Tes-ticular biopsies showed complete lack of germinal epithelium, normal appearing Leydig cells, and tubules with normal-appearing walls and containing normal-appearing Sertoli cells. To explain the absence of the germinal epithelium it is hypothesized that the primary gonocyte failed to penetrate the embryonic testis. Assays of urinary follicle stimulating hormone were normal. 17-ketosteroid excretions were somewhat low. It is suggested that the Sertoli cells are the source of a hormone, similar to estrogen, which regulates the follicle-stimulating hormone production by the pituitary.