Hiperplasia Suprarrenal de Iniciación Tardia

Abstract

Four female patients, 5 to 15 years of age, with late-onset adrenal hyperplasia are reported. One was referred because of premature pubarche, one because of post menarcheal hirsutism and two because of clitoromegaly. All of them had basal plasma 17 OHP above 2.0 ng/ml that increased over 10 ng/ml after ACTH stimulation. Basal plasma cortisol was normal in the four patients, but three of them showed a blunted response to ACTH. Plasma testosterone and DHEA sulfate were not informative for the diagnosis of this condition. (