A case of XX male syndrome.
- Author:
Hwan Sik CHOI
1
;
Jong Woo HONG
;
Eui Je JO
;
Se Jong SHIN
;
Sung Hyup CHOI
Author Information
1. Department of Urology, Inje University, Paik Hospital, Pusan, Korea.
- Publication Type:Case Report
- Keywords:
46;
XX male
- MeSH:
46, XX Testicular Disorders of Sex Development*;
Adult;
Biopsy;
Child;
Female;
Genitalia;
H-Y Antigen;
Humans;
Hypogonadism;
Hypospadias;
Infant;
Infant, Newborn;
Infertility;
Male
- From:Korean Journal of Urology
1991;32(2):357-360
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
The 46, XX male or sex-reversal syndrome is a rare entity, which may be reported first by de la Chapelle and associates in 1964, an additional 135 cases have been recognized, yet only 20 percent of these patients have been diagnosed during childhood. The 46, XX male may be associated with hypogonadism and infertility in adult, and occasionally, sexual ambiguity in the neonate. At least 10% of patients have had hypospadia or ambiguous external genitalia. The 46, XX male was diagnosed with cytogenic study, H-Y antigen, hormonal study testicular biopsy, radiologic study. Here, we report a case of 19 month-old child XX-male with hypospadia and chordee.