Jurnal Urologi Universitas Airlangga
ISSN 23026480
Vol. 1 / No. 2 / Published : 2013-01
Order : 13, and page :13 - 13
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Original Article :
Masculinization in 46 xx congenital adrenal hyperplasia (cah) patient
Author :
- Dimas Sindhu Wibisono*1
- Ardy Santosa*2
- Tarmono*3
- no data*4
- no data*5
- no data
- no data
- no data
- no data
- no data
Abstract :
Introduction: Congenital adrenal hyperplasia (CAH) is the most common cause of ambiguous genitalia in the newborn and the most common cause of female pseudohermaphroditism. it is a classic example of an inborn error of metabolism (cortisol synthesis). divided into three categories: 1. salt wasters, 2. simple virilizers, 3. nonclassic patients. Case: We reported a 25 years old phenotype female 946 XX), with male sexual behavior and orientation which raised as a male. from physical examination we found 5cm of clitoris with severe chordee, normal internal genitalia, persistent urogenital sinus, perineal MUE, rugated major labia, and pubic hair. from chromosomal examination, there is mutation on gene CYP21A2. the patient two sisters also have the same abnormality. the patient has underwent chordectomy and planned to do urethroplasty on the next step of the operation. Conclusion: Most female with CAH has been treated medically by corticosteroid and perform feminizing genitoplasty. but in this case, the patient wanted to be a male. for the first step of operation, we have done chordectomy and for the next step we are planning to do urethroplasty. after the first operation, the patient have 8cm of "penis" with no complain of erection.
Keyword :
Congenital adrenal hyperplasia, female pseudohermaphroditism, cortisol synthesis, chordectomy, urethroplasty,
References :
Diamond DA,(2007) Sexual differentiation: normal & abnormal no data : Elsevier Science
Baskin SL,(2008) Abnormalities of sexual determination & differentiation no data : Mc Graw Hill
Moore LK, Persaud TVN,(2006) The urogenital system no data : Saunders
Naughton CK, Andriole GL,(2002) Adrenal tumors; management of urologic malignancies no data : Churcill Livingstone
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Volume : 1 / No. : 2 / Pub. : 2013-01 |
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