Volume 2015 Supplement 1

Abstracts from the 8th APPES Biennial Scientific Meeting

Open Access

The pattern of disorders of sex development in Vietnamese children

  • Pham Thu Nga1,
  • Vu Chi Dung2,
  • Bui Phuong Thao2,
  • Nguyen Ngoc Khanh2,
  • Can Thi Bich Ngoc2,
  • Hoan Nguyen Thi2 and
  • Dat Nguyen Phu2
International Journal of Pediatric Endocrinology20152015(Suppl 1):P115

https://doi.org/10.1186/1687-9856-2015-S1-P115

Published: 28 April 2015

Keywords

Disorders of sex development DSD 46,XX DSD 46,XY DSD CAH.

Background

Disorders of sex development (DSD) are defined as congenital condition in which development of chromosomal, gonadal, or anatomical sex is atypical. The Chicago DSD classification includes three main diagnostic categories: sex chromosome DSD, 46,XY DSD and 46,XX DSD.

Aims

Define the pattern of disorders of sex development according to Chicago’s classification 2006 at National Hospital of Pediatrics in Hanoi, Vietnam (NHP).

Method

Patients were examined, diagnosed and treated DSD or ambiguous sex at (NHP) from 31/07/2002 to 31/7/2012. Criteria that suggest DSD include
  1. 1.

    overt genital ambiguity (eg, cloacal exstrophy)

     
  2. 2.

    apparent female genitalia with an enlarged clitoris, posterior labial fusion, or an inguinal/labial mass

     
  3. 3.

    apparent male genitalia with bilateral undescended testes, micropenis, isolated perineal hypospadias, or mild hypospadias with undescended testis

     
  4. 4.

    a family history of DSD such as CAIS, and

     
  5. 5.

    a discordance between genital appearance and a prenatal karyotype. Method of the study was descriptive observational.

     

Results

51.7% patients had 46,XX DSD, among them 98.6% had definitive diagnosis. Congenital adrenal hyperplasia (CAH) is the most common cause of 46,XX DSD (91.9%). Rate of 46,XY DSD was 25%, however 83.3% had no definitive diagnosis. 23.3% of patients had chromosome DSD, among them 88.3% chromosome DSD was Turner syndrome.

Conclusion

CAH is the most common cause of DSD.

Authors’ Affiliations

(1)
Hanoi Medical University
(2)
Vietnam National Hospital of Pediatrics

Copyright

© Nga et al; licensee BioMed Central Ltd. 2015

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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