title: Swyer syndrome in a woman with pure 46,XY gonadal dysgenesis and a hypoplastic uterus: a rare presentation.
abstract: OBJECTIVE
  To report a rare presentation of Swyer syndrome in a woman with pure 46,XY gonadal dysgenesis.
  
  
  METHODS
  Case report.
  
  
  METHODS
  Academic gynecology and obstetrics department.
  
  
  METHODS
  A 28-year-old woman with primary amenorrhea.
  
  
  METHODS
  Laparoscopic gonadectomy.
  
  
  METHODS
  Age at diagnosis, risk of gonadal malignancy, bone mineral density, uterus size, height.
  
  
  RESULTS
  The patient had pure 46,XY gonadal dysgenesis with hypoplastic uterus, which is a rare finding in Swyer syndrome. She had developed no gonadal malignancies and, despite receiving estrogen, had remained amenorrheic.
  
  
  CONCLUSIONS
  Further studies should be done on this rare chromosomal condition to find the exact factors affecting the uterus size and to improve the ways of inducing puberty in such patients by considering the age of diagnosis in order to help them have a normal sex life and also the ability to carry a fetus in their immature uteruses.
authors.affiliation: Jondishapour University of Medical Sciences, Faculty of Medicine, Ahvaz, Iran. negar_k_md@yahoo.com
authors.forename: Negar
authors.initials: N
authors.lastname: Karimian
authors.forename: Saber
authors.initials: S
authors.lastname: Ghadakzadeh
authors.forename: Mahdi
authors.initials: M
authors.lastname: Eshraghi
mesh_terms: D000015:Abnormalities, Multiple; D000328:Adult; D000568:Amenorrhea / Q000209:etiology; D002369:Castration; D005260:Female; D006061:Gonadal Dysgenesis, 46,XY / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D006801:Humans; D010535:Laparoscopy; D013577:Syndrome; D014599:Uterus / Q000002:abnormalities* / Q000601:surgery