86 INCREASED PLASMA DHEA AND DHEAS AND ABSENT OR VERY LOW URINARY DHEAS, A NEW CAUSE OF HIRSUTISM (H)

摘要

3 Hirsute,unrelated girls(14.6-17.8yrs)with normal clitoris and regular(1) or irregular(2) menstruations were studied.Cortisol(12.2-22.7 ug/dl),testosterone(68-172),17αOH-progesterone (107-479), and androstenedione(120-459ng/dl)in plasma were normal or moderately increased,but DHEA(1023-1384ng/dl) was high. Also DHEAS (2730-6080 ng/ml) was high,indicating intact sulfokinase. In urine (capillary column gas chromatography), DHEAS was undetectable after helicase, acid or glucuronidase hydrolysis,and low (0.4mg/d,lpt), or absent(2) after ACTH, but 16α-OH-DHEAS was detectable before and after ACTH. Other conjucates were normal or slightly increased, and dexamethasone-suppressible.When DHEAS was added to urine, a peak appeared after helicase, excluding hydrolysis inhibition. When DHEAS was injected iv (50mg,2pts), DHEAS appeared in urine (0.9-2.9 mg/d). With the same dose given to 2 normal girls (17-18.4 yrs) with normal basal plasma DHEA(285 and 549ng/dl),more DHEAS (4.6-6.4 mg/d),and also some 16αOH-DHEAS appeared.Unconjugated urinary DHEA in the pts.was similar as or higher than in the controls. This new type of H appears to be due to an increased renal threshold for DHEAS, but not for other steroids.Based on plasma steroid results alone, such pts.could be erroneously considered to have mild 3β-hydroxysteroid dehydrogenase deficiency. Supported by Swiss National Science Foundation (Grant 3874083).