11β-hydroxylase deficiency medical therapy: Difference between revisions
No edit summary |
No edit summary |
||
| Line 6: | Line 6: | ||
==Medical Therapy== | ==Medical Therapy== | ||
As with other forms of congenital adrenal hyperplasia, the primary therapy of 11β-hydroxylase deficient congenital adrenal hyperplasia is life long [[glucocorticoid]] replacement in sufficient doses to prevent [[adrenal insufficiency]] and suppress excess mineralocorticoid and androgen production.<ref name="w"> Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency. Wikipedia (2016). https://en.wikipedia.org/wiki/Congenital_adrenal_hyperplasia_due_to_11%CE%B2-hydroxylase_deficiency Accessed on January 29, 2016</ref> | As with other forms of congenital adrenal hyperplasia, the primary therapy of 11β-hydroxylase deficient congenital adrenal hyperplasia is life long [[glucocorticoid]] replacement in sufficient doses to prevent [[adrenal insufficiency]] and suppress excess mineralocorticoid and androgen production.<ref name="w"> Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency. Wikipedia (2016). https://en.wikipedia.org/wiki/Congenital_adrenal_hyperplasia_due_to_11%CE%B2-hydroxylase_deficiency Accessed on January 29, 2016</ref> | ||
* Salt-wasting in infancy responds to intravenous saline, dextrose, and high dose [[hydrocortisone]], but prolonged [[fludrocortisone]] replacement is usually not necessary. The [[hypertension]] is ameliorated by glucocorticoid suppression | * Salt-wasting in infancy responds to intravenous saline, dextrose, and high dose [[hydrocortisone]], but prolonged [[fludrocortisone]] replacement is usually not necessary. The [[hypertension]] is ameliorated by glucocorticoid suppression. | ||
* Long term [[glucocorticoid]] replacement requires careful balance between doses sufficient to suppress [[androgen]]s while avoiding suppression of growth. Because the [[enzyme]] defect does not affect [[sex steroid]] synthesis, [[gonads|gonadal]] function at puberty and long-term [[fertility]] should be normal if adrenal androgen production is controlled. | * Long term [[glucocorticoid]] replacement requires a careful balance between doses sufficient to suppress [[androgen]]s while avoiding suppression of growth. Because the [[enzyme]] defect does not affect [[sex steroid]] synthesis, [[gonads|gonadal]] function at puberty and long-term [[fertility]] should be normal if adrenal androgen production is controlled. | ||
==References== | ==References== | ||
Revision as of 17:08, 9 February 2016
|
Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency Microchapters |
|
Differentiating Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency from other Diseases |
|---|
|
Diagnosis |
|
Treatment |
|
Case Studies |
|
11β-hydroxylase deficiency medical therapy On the Web |
|
American Roentgen Ray Society Images of 11β-hydroxylase deficiency medical therapy |
|
Directions to Hospitals Treating Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency |
|
Risk calculators and risk factors for 11β-hydroxylase deficiency medical therapy |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ammu Susheela, M.D. [2]
Overview
The mainstay of therapy for congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is glucocorticoid therapy.[1]
Medical Therapy
As with other forms of congenital adrenal hyperplasia, the primary therapy of 11β-hydroxylase deficient congenital adrenal hyperplasia is life long glucocorticoid replacement in sufficient doses to prevent adrenal insufficiency and suppress excess mineralocorticoid and androgen production.[1]
- Salt-wasting in infancy responds to intravenous saline, dextrose, and high dose hydrocortisone, but prolonged fludrocortisone replacement is usually not necessary. The hypertension is ameliorated by glucocorticoid suppression.
- Long term glucocorticoid replacement requires a careful balance between doses sufficient to suppress androgens while avoiding suppression of growth. Because the enzyme defect does not affect sex steroid synthesis, gonadal function at puberty and long-term fertility should be normal if adrenal androgen production is controlled.
References
- ↑ 1.0 1.1 Congenital adrenal hyperplasia due to 11β-hydroxylase deficiency. Wikipedia (2016). https://en.wikipedia.org/wiki/Congenital_adrenal_hyperplasia_due_to_11%CE%B2-hydroxylase_deficiency Accessed on January 29, 2016