125 Plasma consuntration of androgens, cortisol and its precursors in virilizing adrenal tumors in children

Abstract

Five children (3 girls and 2 boys) aged 1 to 3 years were investigated. Virilization was isolated in 2 (group A), associated with Cushing's syndrome in 2 (group B) and with feminization in 1 (group C). The individual patterns of androgens were variable. However, in 4 out of 5 cases, the relative rises above normal levels (N) were greater for androstenedione (Δ₄) 4 to 100 times, than for dehydroepiandrosterone (DHA) 1.4 to 25 times. 11 β-OH-androstenedione (11 β-OH-Δ₄) measured in groups B and C, was found low normal in C (0.9 ng/ml) and high in B (2.8 and > 10 ng/ml) (N : 0.9 - 2 ng/ml). 17-OH-Progesterone was elevated in group B : 3.5 and 4.7 ng/ml and normal in groups B and C (N : 0.2 - 0.9 ng/ml). Only one of B patients showed a high cortisol level (255 ng/ml) 11-deoxycortisol levels were increased in all groups A, B and C.

Conclusions: a) androstenedione is the androgen the most prominently elevated

b) the rise in 11-déoxycortisol is a common feature

c) 11 β-OH-androstenedione may be low in spite of high levels of Δ₄

d) a possible mild block on 11 β-hydroxylation could be suspected in these tumors

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