Genetic Defects of Cortisol Synthesis ΓÇô Hypertension (2)
Replacement therapy with glucocorticoids rapidly suppresses ACTH levels and hence corticosterone and deoxycorticosterone levels. This results in correction of the hypertension and electrolyte abnormalities.
In the presence of 11b-hydroxylase deficiency, defective cortisol secretion leads to a secondary elevation of ACTH and to bilateral adrenal hyperplasia. In addition, there are high circulating levels of 11-deoxycortisol and deoxycorticosterone present. Hypertension ensues, as does virilism as a result of the elevated androgen levels. The process can be reversed by glucocorticoid replacement therapy.
