Steroidal puberty in girls with Turner's syndrome

Abstract

To study the role of the adrenal cortex versus the ovaries in steroidal puberty, we determined serum steroids in (125 samples from) 60 girls with Turner's syndrome (aged 6-20 years) and in 200 healthy girls. Pregnenolone, dehydroepiandrosterone (DHEA), progesterone, 17-hydroxyprogesterone, androstenedione, testosterone and dihydrotestosterone were measured with a radioimmunoassay after chromatography on Lipidex-5000. The levels of pregnenolone, progesterone and 17-hydroxyprogesterone were similar in the two groups. Testosterone and androstenedione levels were also not different in girls below age 10 years. But later the girls with Turner's syndrome had clearly lower levels, about 50% of the reference levels for testosterone and 60% for androstenedione. The early prepubertal rise in DHEA level was similar in the two groups, but at age 14.5 years the girls with Turner's syndrome had only about 80% of the reference levels. The ovaries thus seem responsible for most of the pubertal increase in circulating testosterone and androstenedione, and possibly a part of the late pubertal increase in DHEA.