An unusual case of ovarian resistance syndrome.
BACKGROUND
Women who present with elevated gonadotropins, primary amenorrhea, and sexual infantilism usually have an abnormal karyotype or premature ovarian failure. We describe a rare case of ovarian resistance secondary to a gonadotropin post-receptor defect.


METHODS
An 18-year-old presented with amenorrhea and sexual infantilism. Original workup led physicians to believe the patient had uterine agenesis with premature ovarian failure. Ovarian biopsy proved the presence of follicles. After 48 months on hormone replacement, the patient went through the stages of puberty, including menarche. Re-evaluation proved the presence of a uterus.


CONCLUSIONS
Ovarian resistance is a rare cause of hypergonadotropic hypogonadism. With this type of ovarian dysfunction, women present early in life with amenorrhea, elevated gonadotropins, and normal karyotypes.
Department of Obstetrics and Gynecology, Harbor-UCLA Medical Center, Torrance, California 90509, USA.
Simone
S
Katz
John
J
Marshall
Omid
O
Khorram
D000328:Adult; D000365:Age Determination by Skeleton; D001851:Bone Diseases, Metabolic / Q000150:complications; D004966:Estrogens, Conjugated (USP) / Q000627:therapeutic use; D005260:Female; D006128:Growth; D006801:Humans; D010050:Ovarian Function Tests; D016649:Primary Ovarian Insufficiency / Q000150:complications / Q000175:diagnosis* / Q000188:drug therapy*
D004966:Estrogens, Conjugated (USP)