21-hydroxylase deficiency surgery: Difference between revisions

	Congenital adrenal hyperplasia main page
Overview
Classification 21-hydroxylase deficiency
11β-hydroxylase deficiency
17 alpha-hydroxylase deficiency
3 beta-hydroxysteroid dehydrogenase deficiency
Cytochrome P450-oxidoreductase (POR) deficiency (ORD)
Lipoid congenital adrenal hyperplasia
Differential Diagnosis

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Revision as of 15:26, 21 July 2017

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Mohammed Abdelwahed M.D [2]

Overview

Reconstructive surgery and Surgical adrenalectomy.

Surgery

Reconstructive surgery

reconstructive gynecologic surgery as adolescents or adults, including clitoroplasty and vaginoplasty.^[1]

Surgical adrenalectomy

The major benefit is lowering adrenal androgen and progesterone secretion. However, It increases dependency on glucocorticoid and mineralocorticoid replacement therapy.^[2]

References

↑ 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.
↑ 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.

[pmid9156043-1] 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.

[pmid22904181-2] 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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@@ Line 1: / Line 1: @@
 __NOTOC__
-{{21-hydroxylase deficiency}}
+{{Congenital adrenal hyperplasia}}
+{{CMG}} {{AE}} {{MAD}}
-{{CMG}} {{AE}} {{AAM}}
+== Overview ==
+Reconstructive surgery and Surgical adrenalectomy.
-==Overview==
+== Surgery ==
-[[Surgery]] is not the first-line treatment option for patients with congenital adrenal hyperplasia due to 21-hydroxylase deficiency. Surgical reconstruction of abnormal genitalia is usually reserved for severely [[virilization|virilized]] girls.
+=== '''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>
-==Surgery==
+==== '''Surgical adrenalectomy''' ====
-===Early-onset: Severe 21-hydroxylase deficient congenital adrenal hyperplasia===
+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>
-====Reconstructive surgery====
-*[[intersex surgery|Surgery]] is not recommended for genetically male (XY) infants because the excess androgens do not produce anatomic abnormality. However, surgery for severely virilized XX infants is often performed and has become a subject of [[history of intersex surgery|debate]] in the last decade.<ref name="Wikipeadia">https://en.wikipedia.org/wiki/Congenital_adrenal_hyperplasia_due_to_21-hydroxylase_deficiency URL Accessed on 10/15/2015 </ref>
-*Surgical reconstruction of abnormal genitalia has been offered to parents of severely [[virilization|virilized]] girls with congenital adrenal hyperplasia since the first half of the 20th century.
-*The purpose of surgery is:
-:*To make the external genitalia look more female than male
-:*To make it possible for these girls to participate in normal sexual intercourse when they grow up
-:*To improve chances of fertility
-:*To reduce the frequency of urinary infections
 == References ==
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-[[Category:Disease]]
-[[Category:Pediatrics]]
-[[Category:Endocrinology]]
-[[Category:Genetic disorders]]
-[[Category:Intersexuality]]