Testicular Biopsy and Hormonal Study in a Male with Noonan's Syndrome

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Abstract
The testicular biopsy study of a 17 yr old male with Noonan''s syndrome revealed seminiferous tubules of reduced diameter with hypospermatogenesis. Many spermatocytes underwent degeneration and many spermatids developed abnormally. The Sertoli cells were similar to immature Sertoli cells. Fully differentiated Leydig cells were rare while precursor Leydig cells were numerous. Both gonadotropin and testosterone levels were low, and a lack of response to LHRH and clomiphene was found. Testicular biopsy performed at 20 yr of age revealed a certain maturation of the seminiferous tubules which increased the germ cell number. Abnormalities in spermatogenesis and the immature appearance of Sertoli cells continued. Leydig cells were more numerous and showed a certain development without reaching the normal pattern. Gonadotropin levels were normal while testosterone levels were low. The response to LHRH was increased and the absence of response to clomiphene persisted. These features suggest a delayed puberty.