|
KMID : 0358419940370112297
|
|
Korean Journal of Obstetrics and Gynecology
1994 Volume.37 No. 11 p.2297 ~ p.2301
|
|
|
A Case of 46,XY Male Pseudohermaphroditism with True Agonadism
|
|
주수만
노성희/김장흡/김진홍/이진우/나종구
|
|
|
Abstract
|
|
|
A 20-year-old phenotypic female with 46,XY genotype presented with primary amenorrhea, no breast development, no pubic hair, no axillary hairr, and no clitoromegaly. The vaginal ended in a 5 cm blind pouch. LH and FSH in serum were raisedin to
the
castrate range. Circulating estradiol levelswere verry low.
Testosterone concentration in serum was lowerlimit of thenormal range for females. Human chorionic gonadotropin(hCG) stimulation did not increase the circulating testosterone concntration. Laparoscopy showed that the patient had neither uterusand
fallopiantube nor remnant of Mullerian products. Gonadal tissue was not observed.
The patient described has clinical, genotypical, andendocrinological characteristics of patients with the syndrome of true agonadism. We report a case of 46,XY true agnoadism with review of literature.
|
|
|
KEYWORD
|
|
|
|
|
|
FullTexts / Linksout information
|
|
 
|
|
|
Listed journal information
|
|
  
|
|