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Congenital adrenal hyperplasia - Overview

Alternative Names

Adrenogenital syndrome; 21-hydroxylase deficiency

Definition of Congenital adrenal hyperplasia:

Congenital adrenal hyperplasia refers to a group of inherited disorders of the adrenal gland.

Causes, incidence, and risk factors:

Congenital adrenal hyperplasia can affect both boys and girls. People with congenital adrenal hyperplasia lack an enzyme needed by the adrenal gland to make the hormones cortisol and aldosterone.

Without these hormones, the body produces more androgen, a type of male sex hormone. This causes male characteristics to appear early (or inappropriately).

About 1 in 10,000 to 18,000 children are born with congenital adrenal hyperplasia.

Aldosterone level test

Reviewed last on: 10/9/2008
A.D.A.M. Editorial Team: David Zieve, MD, MHA, Greg Juhn, MTPW, David R. Eltz. Previously reviewed by Chad Haldeman-Englert, MD, Division of Human Genetics, Children's Hospital of Philadelphia, Philadelphia, PA. Review provided by VeriMed Healthcare Network (5/15/2008).

References

Congenital Adrenal Hyperplasia and Related Disorders. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF. Kliegman: Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 577.

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