Acute adrenal crisis complicating hypertensive congenital adrenal hyperplasia due to 11β‐hydroxylase deficiency

Yair Liel

Research output: Contribution to journalArticlepeer-review

10 Scopus citations

Abstract

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. 1993 Blackwell Munksgaard

Original languageEnglish
Pages (from-to)92-93
Number of pages2
JournalClinical Genetics
Volume43
Issue number2
DOIs
StatePublished - 1 Jan 1993
Externally publishedYes

Keywords

  • 11 β‐hydroxylase deficiency
  • adrenal insufficiency
  • congenital adrenal hyperplasia

ASJC Scopus subject areas

  • Genetics
  • Genetics(clinical)

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