11β-hydroxylase deficiency epidemiology and demographics

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]

Overview

The prevalence of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is approximately 1 per 100,000 individuals the United States. Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency affects male and female equally. Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency usually affects individuals of the Jewish race.

Epidemiology and Demographics

Prevalence

• The prevalence of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency in the United States is approximately 1 per 100,000 individuals.

Incidence

• Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency accounts for 5 to 8 percent of all cases of congenital adrenal hyperplasia. It is estimated that CAH due to 11-beta-hydroxylase deficiency occurs in 1 in 100,000 to 200,000 newborns. This condition is more common in Moroccan Jews living in Israel, occurring in approximately 1 in 5,000 to 7,000 newborns

Case Fatality Rate

• The case­fatality rate of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is highest.

Age

• Patients of all age groups may develop congenital adrenal hyperplasia due to 11β-hydroxylase deficiency. The mean age of diagnosis is infancy and childhood.

Gender

• Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency affects male and female equally.

Race

• Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency usually affects individuals of the Jewish race.

References

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