Volume 2015 Supplement 1
The pattern of disorders of sex development in Vietnamese children
© Nga et al; licensee BioMed Central Ltd. 2015
Published: 28 April 2015
KeywordsDisorders of sex development DSD 46,XX DSD 46,XY DSD CAH.
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.
Define the pattern of disorders of sex development according to Chicago’s classification 2006 at National Hospital of Pediatrics in Hanoi, Vietnam (NHP).
overt genital ambiguity (eg, cloacal exstrophy)
apparent female genitalia with an enlarged clitoris, posterior labial fusion, or an inguinal/labial mass
apparent male genitalia with bilateral undescended testes, micropenis, isolated perineal hypospadias, or mild hypospadias with undescended testis
a family history of DSD such as CAIS, and
a discordance between genital appearance and a prenatal karyotype. Method of the study was descriptive observational.
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.
CAH is the most common cause of DSD.
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