11β-hydroxylase deficiency natural history, complications and prognosis

Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency Microchapters
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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

If left untreated, patients with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency may progress to develop malignant hypertension. Common complications of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency include muscle weakness, metabolic alkalosis, and azoospermia. Prognosis is generally good with treatment.

Natural History

If left untreated, patients with congenital adrenal hyperplasia due to 11β-hydroxylase deficiency may progress to develop malignant hypertension.

Complications

Complications of Hypertension

• Vascular hemorrhage
• Renal insufficiency
• Left ventricular hypertrophy
• Hypertensive retinopathy
• Stroke

Complications of Hypokalemia

• Muscle weakness
• Metabolic alkalosis
• Paralytic ileus

Other Complications

• Azoospermia
• Oligospermia
• Infertility

Prognosis

• The prognosis of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is generally good with treatment.
• A small percentage of children and adults with infancy or childhood onset congenital adrenal hyperplasia die of adrenal crisis, even after the diagnosis and initiation of treatment.
• When a person is well, missing a dose or even several doses, may not worsen the immediate symptoms. However, glucocorticoid needs are increased during illness and stress.