C-11 Hydroxylase Deficiency Medication

Updated: Jan 10, 2017
  • Author: Gabriel I Uwaifo, MD; Chief Editor: George T Griffing, MD  more...
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Medication

Medication Summary

The medical therapy for all variants of congenital adrenal hyperplasia centers on adequate glucocorticoid replacement. [18] This will reduce the ACTH-driven adrenal hyperplasia and production of the various hormone precursors. The recommended medications are hydrocortisone for children and hydrocortisone, prednisone, or dexamethasone for adults.

Potassium-sparing diuretics may be used. These agents are often not sufficient if hypertension is severe, in which case, calcium-channel blockers (nifedipine and verapamil) typically are used as first-choice antihypertensives.

Oral contraceptive pill preparations may be used in adult women with mild forms of the disease to ameliorate some of the virilizing symptoms associated with the condition.

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Corticosteroids

Class Summary

These agents are used for glucocorticoid hormone replacement and for androgen suppression associated with congenital adrenal hyperplasia

Hydrocortisone (Hydrocort, Hydrocortone, Cortef)

Principal hormone secreted by the adrenal cortex. White, odorless, crystalline powder largely insoluble in water. Readily absorbed from the GI tract.

Prednisone (Deltasone, Orasone, Sterapred)

Recommended for use in older patients, because it is longer acting than hydrocortisone.

Dexamethasone (Decadron, AK-Dex)

Synthetic adrenocortical steroid. Dexamethasone is a white, odorless, crystalline powder that is stable in air and practically insoluble in water. Lacks virtually any mineralocorticoid activity.

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Dihydropyridine calcium channel blockers

Class Summary

This and other calcium-channel blockers (dihydropyridine and nondihydropyridine) have particular utility in the management of hypertension related to mineralocorticoid excess. They are among the most efficacious antihypertensives used in hypertension associated with congenital adrenal hyperplasia, such as that occurring in cases of 11-beta-hydroxylase deficiency.

Nifedipine (Adalat, Procardia)

Calcium ion influx inhibitor (slow-channel blocker or calcium ion antagonist) that selectively inhibits transmembrane influx of calcium ions into cardiac muscle and vascular smooth muscle without changing serum calcium concentrations. The mechanism by which nifedipine reduces arterial blood pressure involves peripheral arterial vasodilatation by direct effects and resulting reduction in peripheral vascular resistance. Completely absorbed after oral administration. Extensively metabolized to highly water-soluble, inactive metabolites, accounting for 60-80% of the dose excreted in the urine. The elimination half-life is approximately 2 h. Only traces (< 0.1% of the dose) of unchanged form can be detected in the urine. The remainder is excreted in the feces in metabolized form, usually as a result of biliary excretion. Pharmacokinetics are not significantly influenced by the degree of renal impairment. Because hepatic biotransformation is the predominant route for the disposition of nifedipine, the

pharmacokinetics may be altered in patients with chronic liver disease. Because of reports suggesting their association with increased myocardial ischemia and cardiac mortality, the use of short-acting forms of nifedipine for acute and long-term blood pressure management is less popular and generally not preferable compared to using long-acting preparations, such as Procardia XL and Adalat CC.

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Potassium-sparing diuretics

Class Summary

These agents are used to treat hypertension associated with 11-beta-hydroxylase deficiency.

Amiloride (Midamor)

Antikaliuretic diuretic agent. A pyrazine-carbonyl-guanidine that is chemically unrelated to other known antikaliuretic or diuretic agents. Potassium-conserving (antikaliuretic) drug that possesses weak (compared with thiazide diuretics) natriuretic, diuretic, and antihypertensive activity. In some clinical studies, its activity increased effects of thiazide diuretics. Amiloride is not an aldosterone antagonist, and its effects are observed even in the absence of aldosterone. Exerts potassium-sparing effect through inhibition of sodium reabsorption at distal convoluted tubule, cortical collecting tubule, and collecting duct. This decreases the net negative potential of the tubular lumen and reduces potassium and hydrogen secretion, as well as their subsequent excretion.

Spironolactone (Aldactone)

Specific pharmacologic antagonist of aldosterone that acts primarily through competitive binding of receptors at the aldosterone-dependent sodium-potassium exchange site in the distal convoluted renal tubule.

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