THE ROLE OF UROLOGISTS IN MANAGEMENT OF DISORDERS OF SEX DEVELOPMENT
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Abstract
Objective: To evaluate disorder of sex development (DSD) profile at Cipto Mangunkusumo Hospital (RSCM), the management profile, and the role of urologist on diagnostic and therapeutic management. Material & method: We retrospectively collected data from medical record of all DSD cases managed by pediatric endocrinologist, urologist, obstetric gynaecologist at RSCM from January 2002 up to December 2009. 2006 IICP criteria was used as classification. The management profile and the role of urologist were evaluated. Results: there were 133 DSD cases with the majority of cases was congenital adrenal hyperplasia (CAH) followed by androgen insensitivity syndrome (AIS). Most of the cases were diagnosed before one year old and other on pubertal period. Karyotyping, laboratory examination, ultrasonography, genitography, uretrocystoscopy, kolposcopy, diagnostic laparascopy were performed as diagnostic management. Gender assignment was performed by multidisciplinary team. Masculinizing surgery, feminizing surgery, and gonadectomy was done as therapeutic management. Conclusion: The majority case on RSCM’s DSD profile was CAH. The management was performed by multidisciplinary team. Gender assignment decision should be based upon thorough diagnostic evaluation. The urologist has important role on diagnostic and therapeutic management. Keywords: Disorder of sex development, diagnostic management, gender assignment, therapeutic management, urologist.
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Diamond DA. Sexual differentiation: Normal and abnormal. In: Wein K, Novick, Partin, Peters, editor. Campbell Walsh Urology. 9th ed. China: WB Saunders; 2007. p. 3808-19.
Chi C, Lee HC. Ambiguous genitalia in the newborn. Neo Reviews 2008; 9(2): 78-9.
IA Hughes. Consensus statement on management of intersex disorder. Arch Dis Child. 2006; 91: 554-62.
Caroline EB, Sarah M, Imran M, Polly AC, Angela B, John WH, et al. Holistic management of DSD. Best Pract Res Clin Endocrinol Metab. 2010; 24(1): 335-54.
Chistopher P, Ieuan A. Summary of consensus statement on intersex disoreder and their management. Pediatrics. 2006; 118(2): 753-7.
Consortium on the management of disorder of sex development. Clinical guidelines for the management of disorder of sex development in childhood. In: Dreger AD, editor. 1st ed. California: Intersex Society of North America; 2006. p. 9-24.
Rajendran R. Profile of intersex children in south india. Indian pediatrics. 1995; 32(1): 666-71.
Al-Agha AE, Thomsett MJ. The child of uncertain sex: 17 years of experience. J Paediatr Child Health. 2001; 37(4): 348-51.
Rajesh RJ, Sudha R, Desai M. Etiology and clinical profile of ambiguous genitalia - an overview of 10 years experience. Indian Pediatrics. 2006; 43(2): 974-8.
Garry L. Warne, Raza J. Disorders of sex development (DSDs), their presentation and management in different cultures. Rev Endocr Metab Disord. 2008; 41(2): 201-11.
Rajendran R. Profile of intersex children in South India Indian pediatrics. 1995; 32(1): 666-71.
Jeffrey L, Mark PC, Carlos R. Feminizing genital reconstruction in congenital adrenal hyperplasia. Indian Journal of Urology. 2009; 25(1): 17-26.
Rink R. Surgical management of intersexuality, cloacal malformations and other abnormalities of the genitalia in girls. In: Wein K, Novick, Partin, Peters, editor. Campbell Walsh Urology. 9th ed. China: WB Saunders; 2007. p. 3830-9.
Dénes FT, Mendonça BB. Laparoscopic management of intersexual states. Urol Clin North Am. 2001; 28(1): 31-42.
Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorder. Arch Dis Child. 2006; 91: 554-62.
Chertin B, Koulikov D, Hadas-halpern I, Farkas A. Masculinizing genitoplasty in intersex patient. J Urol. 2005; 174(4): 1683-6.
Francisco TD, Maria S, Frederico S, Elaine M.F, Vinicius NB, Ivo JP, et al. Short and long term surgical outcome of masculinizing genitiplasty in a large cohort of patients with disorder of sex development (DSD). J Urol. 2009; 181(4): 1206-10.
Ahmad AA, Ahmad A, Amr M Masoud, Beni-sweif. Functional and cosmetic outcomes of vaginoplasty in egyptian intersex patients. J Urol. 2009; 181(4): 400.
Martine C, Stenvert LS, Katja P. Wolffenbuttel, J. Wolter O, Looijenga LHJ. Germ cell tumors in the intersex gonad: Old paths, new directions, moving frontiers. Endocrine Reviews. 2006; 27(5): 468-84.
Lamberts SW. Germ cell tumors in patients with disorders of sex development: Risk factors, initial developmental stages and targets for early diagnosis. Rotterdam Erasmus Medical Center; 2006.
Maria HC, Frederico A, Elaine MF, Vinicius B, Ivo JA, Francisco TD, et al. Long-term surgical outcome of masculinizing genitoplasty in a large cohort of patients with disorders of sex development. Journal of Pediatric Urology. 2010; 6(1): S89.
Francisco TD, Maria S, Guiomar M, Tania AB, Berenice BM, Frederico S. Anatomical and fungctional outcome of feminizing genitoplasty for ambigous genitalia in patients with virilizing congenital adrenal hyperplasia. J Urol. 2009; 181(4): 400.