Abstract
Gonadal function in four patients with mosaic XO or XX/XY Turner's syndrome was reviewed. Luteinizing hormone (LH) levels were determined by double antibody radioimmunoassay. Urinary testosterone (UT) and plasma testosterone (T) levels were determined by either double isotope or competitive protein binding methods. Three patients had ambiguous genitalia and exploratory surgery revealed only medullary elements in the gonads. Two virilized at puberty and were studied prior to surgery. One had normal LH levels (11 mIU/ml), a normal excretion of UT (0.5 μg/24 hrs) but responded to human chorionic gonadotropin (HCG) with an ten-fold increase in UT. The other had castrate levels of LH (≧ 100 mIU/ml) and adult male levels of plasma T (208 mμg%) which did not increase with HCG. The third patient at age 41/2 years was found to have an undifferentiated streak and, contralaterally, a testis and epididymis. The fourth patient, 14 years of age, has no evidence of any adoloscent development.
Prophylactic gonadectomy in mixed gonadal dysgenesis has been recommended because of the increased incidence of gonadal malignancy. Our patients support the recommendation for early surgery. It appears that waiting until post-puberty serves no purposes because if the gonads are to function at all, they are more likely to produce androgens.
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Moshang, T., Vallet, H., Cintron, C. et al. Gonadal function in mosaic XO or XX/XY Turner's syndrome. Pediatr Res 5, 423 (1971). https://doi.org/10.1203/00006450-197108000-00217
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DOI: https://doi.org/10.1203/00006450-197108000-00217