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Paper Information

Journal:   TEHRAN UNIVERSITY MEDICAL JOURNAL (TUMJ)   2003 , Volume 61 , Number 3; Page(s) 206 To 211.
 
Paper: 

AMBIGUOUS GENITALIA CHILDREN MEDICAL CENTER HOSPITAL (1983-1999)

 
 
Author(s):  AHMADI I.*, KALANTARI M., KAHBAT-ZAEH A., SAMADI S.M.H.
 
* 
 
Abstract: 

Background: Ambiguous genitalia means difference between gonads morphology and external genitalia. Gender assignment and surgical correction must be done as early as possible to assure unambiguous bonding between the parents and the child. The purpose of this study was to evaluate Ambiguous genitalia in a referral children surgical center.
Materials and Methods: We retrospectively reviewed records of 200 patients admitted to children medical center hospital in a period of 16 years for Ambiguous genitalia surgery. Data about clinical and laboratory examination collected and analyzed.
Results: The prevalence of female pseudohermaphrodism, male pseudohermaphrodism, ture hermaphrodism and Mixed gonadal digenesis was 70.5%, 20.5%, 5% and 4%, respectively. 76.5% and 23.5% of the patients was converted to female and male, respectively. 29% had congenital Adrenal hyperplasia of whom 86.5%, 10.5% and 3% had 21-hydroxylase deficiency, 11β hydroxylase deficiency and 3β HSD deficiency, respectively. 70.5% had 46xx karyotype and 21.5% had 46xy karyotype and the remainder were musaic. 52.5% had relative parents. The malformation were corrected by vaginoplasty (105 patient) and clitoral resection (51 patients). A variety of anomalies was seen.
Conclusion: Age of presentation ranged from 1 day to 13 years. 151 patients were diagnosed in infancy, but only 43 patient was brought for treatment in infancy to this center by parents.

 
Keyword(s): AMBIGUOUS GENITALIA, CONGENITAL ADRENAL HYPERPLASIA
 
References: 
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