11β-hydroxylase deficiency medical therapy: Difference between revisions
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As with other forms of congenital adrenal hyperplasia, the primary therapy of 11β-hydroxylase deficient congenital adrenal hyperplasia is lifelong [[glucocorticoid]] replacement in sufficient doses to prevent [[adrenal insufficiency]] and suppress excess mineralocorticoid and androgen production.<ref> 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 lifelong [[glucocorticoid]] replacement in sufficient doses to prevent [[adrenal insufficiency]] and suppress excess mineralocorticoid and androgen production.<ref> 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 of DOC. | * 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 of DOC. | ||
* Long term [[glucocorticoid]] replacement issues are similar to those of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency and involve 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. See [[congenital adrenal hyperplasia]] for a more detailed discussion of androgen suppression and fertility potential in adolescent and adult women. | * Long term [[glucocorticoid]] replacement issues are similar to those of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency and involve the 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. See [[congenital adrenal hyperplasia]] for a more detailed discussion of androgen suppression and fertility potential in adolescent and adult women. | ||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Revision as of 21:30, 29 January 2016
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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
The mainstay of therapy for congenital adrenal hyperplasia due to 11β-hydroxylase deficiency is glucocorticoid therapy.
Medical Therapy
As with other forms of congenital adrenal hyperplasia, the primary therapy of 11β-hydroxylase deficient congenital adrenal hyperplasia is lifelong 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 of DOC.
- Long term glucocorticoid replacement issues are similar to those of congenital adrenal hyperplasia due to 11β-hydroxylase deficiency and involve the 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. See congenital adrenal hyperplasia for a more detailed discussion of androgen suppression and fertility potential in adolescent and adult women.
References
- ↑ 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