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Acute adrenal crisis complicating hypertensive congenital adrenal hyperplasia due to 11β‐hydroxylase deficiency
Author(s) -
Liel Yair
Publication year - 1993
Publication title -
clinical genetics
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.543
H-Index - 102
eISSN - 1399-0004
pISSN - 0009-9163
DOI - 10.1111/j.1399-0004.1993.tb04456.x
Subject(s) - congenital adrenal hyperplasia , medicine , 21 hydroxylase , adrenal crisis , adrenal insufficiency , endocrinology , glucocorticoid , androgen excess , hydrocortisone , hyperplasia , blood pressure , obesity , insulin resistance , polycystic ovary
In its classical form, congenital adrenal hyperplasia due to 11 β‐hydroxylase deficiency is characterized by hypertension and abnormal sexual development. Suppression of ACTH secretion by means of administering glucocorticoids fulfills the therapeutic goal of reducing blood pressure and decreasing androgen production. The present report describes the case of a patient suffering from congenital adrenal hyperplasia due to 11β‐hydroxylase deficiency who developed an acute adrenal crisis, unprovoked by stress, following interruption of glucocorticoid replacement therapy. It is suggested that patients on a suppressive dose of glucocorticoids for adrenal hyperplasia are at increased risk for acute adrenal insufficiency if therapy is interrupted, and that deoxycorticosterone (DOC) in the absence of a glucocorticoid is insufficient to prevent manifestations of adrenal crisis.