JOHN E. JOHNSON JR., M.D.
One of the most remarkable advances in clinical endocrinology has been the elucidation of the pathogenesis, clinical features, and treatment of congenital adrenal hyperplasia.1-3 In the fetal adrenal cortex of affected persons, synthesis of hydrocortisone is defective owing to a deficiency of specific hydroxylating enzymes. A larger amount of adrenocorticotrophin (ACTH) stimulation of the adrenal is necessary to produce that blood level of hydrocortisone which will in turn suppress and regulate pituitary production of ACTH. Thus, an equilibrium is established at a supranormal level of ACTH release, which causes morphologic hyperplasia of the adrenal cortex. The functional concomitant is increased
JOHNSON JE. VIRILIZING CONGENITAL ADRENAL HYPERPLASIA: TWENTY-FIVE YEARS LATER1. Ann Intern Med. ;54:924–937. doi: 10.7326/0003-4819-54-5-924
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Published: Ann Intern Med. 1961;54(5):924-937.
Adrenal Disorders, Endocrine and Metabolism.
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