21-hydroxylase deficiency surgery: Difference between revisions

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{{21-hydroxylase deficiency}}
{{21-hydroxylase deficiency}}
{{CMG}} {{AE}} {{MAD}}
{{CMG}} ;{{AE}}{{MJ}}
== Overview ==
== Overview ==
Reconstructive surgery and Surgical adrenalectomy.  
Reconstructive [[surgery]] and surgical [[adrenalectomy]] are the surgical procedures used for patients with 21-hydroxylase deficiency. Reconstructive [[Gynaecology|gynecologic]] [[surgery]] may be used in [[Adolescent|adolescents]] or [[Adult|adults]], including clitoroplasty and [[vaginoplasty]]. Surgical adrenalectomy can be done in some cases, if there is no response to medical therapy.  
== Surgery ==
== Surgery ==
=== '''Reconstructive surgery''' ===
=== '''Reconstructive surgery''' ===
reconstructive gynecologic surgery as adolescents or adults, including clitoroplasty and vaginoplasty.<ref name="pmid9156043">{{cite journal| author=Premawardhana LD, Hughes IA, Read GF, Scanlon MF| title=Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience. | journal=Clin Endocrinol (Oxf) | year= 1997 | volume= 46 | issue= 3 | pages= 327-32 | pmid=9156043 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9156043  }}</ref>
* Reconstructive [[Gynaecology|gynecologic]] [[surgery]] may be used in [[Adolescent|adolescents]] or [[Adult|adults]], including clitoroplasty and [[vaginoplasty]]. <ref name="pmid9156043">{{cite journal| author=Premawardhana LD, Hughes IA, Read GF, Scanlon MF| title=Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience. | journal=Clin Endocrinol (Oxf) | year= 1997 | volume= 46 | issue= 3 | pages= 327-32 | pmid=9156043 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=9156043  }}</ref>
==== '''Surgical adrenalectomy''' ====
 
The major benefit is lowering adrenal androgen and progesterone secretion. However, It increases dependency on glucocorticoid and mineralocorticoid replacement therapy.<ref name="pmid22904181">{{cite journal| author=Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R et al.| title=Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia. | journal=J Clin Endocrinol Metab | year= 2012 | volume= 97 | issue= 11 | pages= E2084-9 | pmid=22904181 | doi=10.1210/jc.2012-2298 | pmc=3485588 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22904181  }}</ref>
=== '''Surgical adrenalectomy''' ===
* Surgical adrenalectomy can be done in some cases, if there is no response to medical therapy.
 
* The major benefit is lowering [[Adrenal gland|adrenal]] [[androgen]] and [[progesterone]] secretion. However, it increases dependency on [[glucocorticoid]] and [[mineralocorticoid]] replacement therapy. <ref name="pmid22904181">{{cite journal| author=Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R et al.| title=Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia. | journal=J Clin Endocrinol Metab | year= 2012 | volume= 97 | issue= 11 | pages= E2084-9 | pmid=22904181 | doi=10.1210/jc.2012-2298 | pmc=3485588 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22904181  }}</ref>
 
== References ==
== References ==
{{Reflist|2}}
{{Reflist|2}}
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[[Category:Disease]]
[[Category:Pediatrics]]
[[Category:Endocrinology]]
[[Category:Genetic disorders]]
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Latest revision as of 15:31, 24 July 2020

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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

Reconstructive surgery and surgical adrenalectomy are the surgical procedures used for patients with 21-hydroxylase deficiency. Reconstructive gynecologic surgery may be used in adolescents or adults, including clitoroplasty and vaginoplasty. Surgical adrenalectomy can be done in some cases, if there is no response to medical therapy.

Surgery

Reconstructive surgery

Surgical adrenalectomy

  • Surgical adrenalectomy can be done in some cases, if there is no response to medical therapy.

References

  1. Premawardhana LD, Hughes IA, Read GF, Scanlon MF (1997). "Longer term outcome in females with congenital adrenal hyperplasia (CAH): the Cardiff experience". Clin Endocrinol (Oxf). 46 (3): 327–32. PMID 9156043.
  2. Crocker MK, Barak S, Millo CM, Beall SA, Niyyati M, Chang R; et al. (2012). "Use of PET/CT with cosyntropin stimulation to identify and localize adrenal rest tissue following adrenalectomy in a woman with congenital adrenal hyperplasia". J Clin Endocrinol Metab. 97 (11): E2084–9. doi:10.1210/jc.2012-2298. PMC 3485588. PMID 22904181.

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