17 alpha-hydroxylase deficiency medical therapy: Difference between revisions
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{{CMG}}; {{AE}} {{MJ}} | {{CMG}}; {{AE}} {{MJ}} | ||
==Overview== | ==Overview== | ||
The mainstay of therapy for 17 alpha-hydroxylase deficiency is [[glucocorticoid]] therapy. [[Spironolactone]] and [[Estrogen-replacement therapy|estrogen]] also | The mainstay of therapy for 17 alpha-hydroxylase deficiency is [[glucocorticoid]] therapy. [[Spironolactone]] and [[Estrogen-replacement therapy|estrogen]] may also be used. | ||
==Medical Therapy== | ==Medical Therapy== | ||
The goal of therapy is: | The goal of therapy is: <ref name="pmid28576284">{{cite journal |vauthors=El-Maouche D, Arlt W, Merke DP |title=Congenital adrenal hyperplasia |journal=Lancet |volume= |issue= |pages= |year=2017 |pmid=28576284 |doi=10.1016/S0140-6736(17)31431-9 |url=}}</ref><ref name="pmid24622419">{{cite journal |vauthors=Merke DP, Poppas DP |title=Management of adolescents with congenital adrenal hyperplasia |journal=Lancet Diabetes Endocrinol |volume=1 |issue=4 |pages=341–52 |year=2013 |pmid=24622419 |pmc=4163910 |doi=10.1016/S2213-8587(13)70138-4 |url=}}</ref><ref name="pmid3060026">{{cite journal| author=Hughes IA| title=Management of congenital adrenal hyperplasia. | journal=Arch Dis Child | year= 1988 | volume= 63 | issue= 11 | pages= 1399-404 | pmid=3060026 | doi= | pmc=1779155 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3060026 }}</ref> | ||
* Correct the effects of [[mineralocorticoid excess]] | * Correct the effects of [[mineralocorticoid excess]] | ||
* Prevent [[glucocorticoid]] deficiency | * Prevent [[glucocorticoid]] deficiency | ||
* Restore desired secondary [[sexual characteristics]] | * Restore desired secondary [[sexual characteristics]] | ||
* Treatment for 17 alpha-hydroxylase deficiency is [[glucocorticoids]] as | *Treatment for 17 alpha-hydroxylase deficiency is by the use of [[glucocorticoids]] such as: | ||
** Preferred regimen (1): [[Hydrocortisone]] 10 to 25 mg/m2 body surface area/day PO | ** Preferred regimen (1): [[Hydrocortisone]] 10 to 25 mg/m2 body surface area/day PO. | ||
** Preferred regimen (2): [[Prednisolone]] 0.1 mg/kg/day PO | ** Preferred regimen (2): [[Prednisolone]] 0.1 mg/kg/day PO. | ||
** Preferred regimen (3): [[Dexamethasone]] up to 0.5 mg/day PO | ** Preferred regimen (3): [[Dexamethasone]] up to 0.5 mg/day PO. | ||
*As all the patients are clinically female, [[spironolactone]] is the drug of choice to block the [[mineralocorticoid receptor]]: | |||
* [[ | ** Preferred regimen (1): [[Spironolactone]] 25 to 200 mg/day PO. | ||
* [[ | |||
*Treatment should be monitored by: | |||
**[[Blood pressure]] | |||
**[[Deoxycorticosterone]] levels | |||
**[[Electrolyte|Electrolytes]] | |||
*At the time of expected [[puberty]], [[Estrogen replacement therapy|estrogen replacement]] should be started. | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
Latest revision as of 12:24, 23 October 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mehrian Jafarizade, M.D [2]
Overview
The mainstay of therapy for 17 alpha-hydroxylase deficiency is glucocorticoid therapy. Spironolactone and estrogen may also be used.
Medical Therapy
The goal of therapy is: [1][2][3]
- Correct the effects of mineralocorticoid excess
- Prevent glucocorticoid deficiency
- Restore desired secondary sexual characteristics
- Treatment for 17 alpha-hydroxylase deficiency is by the use of glucocorticoids such as:
- Preferred regimen (1): Hydrocortisone 10 to 25 mg/m2 body surface area/day PO.
- Preferred regimen (2): Prednisolone 0.1 mg/kg/day PO.
- Preferred regimen (3): Dexamethasone up to 0.5 mg/day PO.
- As all the patients are clinically female, spironolactone is the drug of choice to block the mineralocorticoid receptor:
- Preferred regimen (1): Spironolactone 25 to 200 mg/day PO.
- Treatment should be monitored by:
- At the time of expected puberty, estrogen replacement should be started.
References
- ↑ El-Maouche D, Arlt W, Merke DP (2017). "Congenital adrenal hyperplasia". Lancet. doi:10.1016/S0140-6736(17)31431-9. PMID 28576284.
- ↑ Merke DP, Poppas DP (2013). "Management of adolescents with congenital adrenal hyperplasia". Lancet Diabetes Endocrinol. 1 (4): 341–52. doi:10.1016/S2213-8587(13)70138-4. PMC 4163910. PMID 24622419.
- ↑ Hughes IA (1988). "Management of congenital adrenal hyperplasia". Arch Dis Child. 63 (11): 1399–404. PMC 1779155. PMID 3060026.