46,XX male sex reversal syndrome and its treatment recommendation (report of 4 cases)
- VernacularTitle:46,XX男性性别逆转综合征(附四例报告)
- Author:
Yunman TANG
;
Lugang HUANG
;
Zhiying LIU
- Publication Type:Journal Article
- Keywords:
Sex reversal,gonadal;
Sex chromosomes;
Diagnosis;
Children
- From:
Chinese Journal of Urology
2001;0(11):-
- CountryChina
- Language:Chinese
-
Abstract:
Objective To analyze the clinical,endocrinal and genetic features of 46,XX male syndrome. Methods Retrospectively collect and analyze the clinical data of 4 cases of 46,XX male sex reversal syndrome. Results Four patients were all sociopsychologically males.Among them,2 cases had cryptorchidism,3 hypospadia and 1 severe chordee with short urethra.Neither ovary nor uterus was detected through B ultrasonography or surgical exploration.Assessment of serum sex hormone suggested hypergonadotropic hypogonadism.Genetic detection indicated 46,XX karyotype as normal female's type. Conclusions 46,XX male has nearly normal male phenotype otherwise with testes dysgenesis and most are infertile.The target of treatment is to correct the urinogenital malformation and to compensate hormone defect with androgen.
