Children with male pseudohermaphroditism: endocrine and metabolic studies

In 22 children with male pseudohermaphroditism, plasma concentrations of testosterone and dehydroandrosterone sulfate were measured before and after HCG stimulation. In ten of them, nitrogen retention was measured both before and during treatment with testosterone-propionate. The results of this investigation allow the patients to be classified in the following groups 1) nine in which both the testicular function and the end-organ sensitivity to androgens were normal; 2) nine patients had an abnormal testicular function; 3) four patients had the testicular feminization syndrome. In addition, in one adolescent patient with familial male pseudohermaphroditism and gynecomastia, the pattern of plasma androgens under basal conditions, after HCG and following gonadectomy, suggest that the patient had an incomplete 17-ketosteroid reductase defect.