skip to main content

Multidisciplinary Management of Disorders of Sex Development in Indonesia, A Prototype in Developing Country

1Faculty of Medicine Diponegoro University, Indonesia

2dr. Kariadi Semarang Hospital, Indonesia

3Faculty of Medicine Diponegoro University, Indonesia

Received: 15 May 2017; Published: 23 Jun 2017.
Open Access Copyright (c) 2017 Journal of Biomedicine and Translational Research

Citation Format:
Abstract

Background : Disorder of sex development (DSD) patients require comprehensive management to improve quality of life. A standardized management protocol for patients in Indonesia is not yet available resulting in patients infrequently received a proper diagnosis. This study reported a multidisciplinary management DSD in Indonesia based on minimal diagnostic facilities and expertise in developing country.

Objectives : The purpose of the study is to review the management of DSD patients in Indonesia relates to providing appropriate gender assignment and to improving patients quality of life.

Methodology : We analyzed the records of DSD patient admitted to the division of Human Genetics Center for Biomedical Research (CEBIOR) Faculty of Medicine Diponegoro University, Semarang, Indonesia from May 2004 - December 2015. Data were collected and analyzed for physical examination, family pedigree karyotyping, hormonal assays and  psychosocial.  Other examination such as ultrasonography, Xray and Cytoscopy were also recorded for selected cases. Bimonthly, Sexual Adjustment Team (SAT) meeting was recorded.

Results : From the total 617 DSD cases we found 426 cases (69,04 %) with 46, XY DSD, 117 cases (18,96%) with 46,XX DSD and 74 cases (12%) with sex chromosome DSD. Most of the patients in the group of 46, XY DSD are Unknown Male Undervirilization (UMU) with 256 cases (60.09%). As the majority cases of 46, XX DSD was Congenital Adrenal Hyperplasia with 81 cases (69.23%). The remaining cases were Androgen Action Disorder (AAD) with 140 cases (32.86%), 46, XY DSD Gonadal Dysgenesis with 30 cases (7.04%), Androgen Excess Disorders with 3 cases (2.56%), Defect of Mullerian Development with 19 cases (16,24%), 3 cases (2.56%) of Androgen Excess and 3 cases (2.56%) of 46, XX Gonadal Dysgenesis.

Conclusion : Comprehensive management for DSD Patients help patient in diagnosis, gender assignment and support patient to improve quality of life. This multidisciplinary of DSD team is the only team in Indonesia that can be used as a model for other center in Indonesia as well as other developing countries with minimal diagnostic facilities.

Note: This article has supplementary file(s).

Fulltext View|Download |  Research Instrument
Untitled
Subject
Type Research Instrument
  Download (270KB)    Indexing metadata
Keywords: Disorder of Sex Development; Sexual Adjustment Team; Chromosome.

Article Metrics:

  1. Hughes IA, Houk C, Ahmed SF, Lee PA, and LWPES1/ESPE2 Consensus group. Consensus statement on management of intersex. Archdischild. 2006;91:554–63
  2. Warne GL, Raza J. Disorders of sex development (DSDs), their presentation and management in different cultures. Rev Endocr Metab Disord. 2008;9(3):227–36
  3. Warne GL. Long-term outcome of disorders of sex development. Sex Dev. 2008;2(4–5):268–77
  4. Hughes IA, Houk C, Ahmed SF, Lee PA. Consensus statement on management of intersex disorders. J Pediatr Urol. 2006;2(3):148–62
  5. Lathrop B, Cheney TB. Ethical perspectives on the management of disorders of sex development in children. Medicolegal Bioeth. 2015;5:27–34
  6. Zayts O, Sarangi S, Thong MK, Chung BHY, Lo IFM, Kan ASY, et al. Genetic counseling/consultation in South-East Asia: A report from the workshop at the 10th Asia pacific conference on human genetics. J Genet Couns. 2013;22(6):917–24
  7. Piprek RP. Molecular and cellular machinery of gonadal differentiation in mammals. Int J Dev Biol. 2010;54(5):779–86
  8. Kousta E, Papathanasiou A, Skordis N. Sex determination and disorders of sex development according to the revised nomenclature and classification in 46,XX individuals. Hormones. 2010;9(3):218–31
  9. Eggers S, Sinclair A. Mammalian sex determinationinsights from humans and mice. Chromosom Res. 012;20(1):215–38
  10. Faradz SM. Genetic analysis for the diagnosis of disorders of sexual development in Indonesia. J Biomed Transl Res. 2016;2:22–4
  11. Özbey H, Etker S. Disorders of sexual development in a cultural context. Arab J Urol. 2013;11(1):33–9
  12. Ediati A, Maharani N, Utari A. Sociocultural aspects of disorders of sex development. Embryo Today Rev. 2016;108(12):380–3
  13. Lee PA, Nordenström A, Houk CP, Ahmed SF, Auchus R, Baratz A, et al. Global disorders of sex development update since 2006: Perceptions, approach and care. Horm Res Paediatr. 2016;85(3):158–80
  14. Ediati A, Faradz SMH, Juniarto AZ, van der Ende J, Drop SLS, Dessens AB. Emotional and behavioral problems in late-identified Indonesian patients with disorders of sex development. J Psychosom Res.2015;79(1):76–84
  15. Ediati A, Juniarto AZ, Birnie E, Drop SLS, Faradz SMH, Dessens AB. Gender development in Indonesian children, adolescents, and adults with disorders of sex development. Arch Sex Behav. 2015;44(5):1339–61
  16. Ediati A, Juniarto A, Birnie E, Okkerse J, de la Croix A, Wisniewski A, et al. Gender change and stigmatization in late-treated Indonesian children, adolescent, and adult patients with DSD. Int J Pediatr Endocrinol. 2015;2015(Suppl 1):O56
  17. Ediati A, Juniarto AZ, Birnie E, Drop SLS, Faradz SMH, Dessens AB. Body image and sexuality in indonesian adults with a disorder of sex development (DSD). J Sex Res. 2015;52(1):15–29
  18. Juniarto Z, van der Zwan YG, Santosa A, Ariani MD, Eggers S, Hersmus R, et al. Hormonal evaluation in relation to phenotype and genotype in 286 patients with a disorder of sex development fr

Last update: 2021-07-28 00:20:44

  1. The Evaluation of Parental Acceptance Towards Children with Sex Chromosomal Disorders of Sex Development Using A Mixed-Method

    Iit Fitrianingrum, Annastasia Ediati, Tri Indah Winarni, Sultana MH Faradz. Journal of Biomedicine and Translational Research, 7 (1), 2021. doi: 10.14710/jbtr.v7i1.10710
  2. Genetic Analysis Reveals Complete Androgen Insensitivity Syndrome in Female Children Surgically Treated for Inguinal Hernia

    Journal of Investigative Surgery, 34 (2), 2021. doi: 10.1080/08941939.2019.1602690

Last update: 2021-07-28 00:20:44

No citation recorded.