LONGITUDINAL STUDY OF PLASMA TESTOSTERONE IN MALE PSEUDOHERMAPHRODITES DURING EARLY INFANCY

Plasma testosterone was longitudinally studied during the first months of life in 7 XY infants with male pseudohermaphroditism. In all, the 2 testes were palpable in normal or inguinal position. In 1 case the defect of masculinization was complete and associated with a salt loss syndrome. The 6 others had ambiguous genitalia with perineal hypospadias and micropenis. In 2, the physiological rise of testosterone was absent or blunted, and a combined adrenal and testicular enzymatic defect was demonstrated (3 β hydroxysteroid dehydrogenase and 20-22 desmolase deficiencies). In 5, a normal postnatal testosterone rise with peak values ranging between 3.4 and 6.4 ng/ml suggested an abnormal peripheral responsiveness to androgens.