Idiopathic premature ovarian failure in 63 young women.
BACKGROUND
Premature ovarian failure (POF) in adolescents is defined as primary or secondary amenorrhea associated with high follicle-stimulating hormone (FSH) levels. In normal 46,XX patients, its etiology is most often unknown. We have evaluated the clinical, hormonal and ovarian phenotypes in patients with a normal karyotype who were diagnosed with POF before the age of 18.


METHODS
Sixty-three patients were included in this retrospective study.


RESULTS
The mean patient age was 20.4 years. The patients presented with three clinical patterns: lack of pubertal development (n = 23), primary amenorrhea with interrupted puberty (n = 18), and secondary amenorrhea with normal puberty (n = 22). Ten patients had a familial history of POF and 6 presented with hypothyroidism. The FSH, estradiol and inhibin B levels were not statistically different in the three clinical groups. Fifty percent of the patients presented small ovaries (length <2 cm) at ultrasonography. The presence of follicles was found at histology in only 7 of the 27 patients who underwent an ovarian biopsy.


CONCLUSIONS
46,XX patients presenting with early POF rarely presented a specific, identifiable disorder. We discuss the clinical management and different diagnosis strategies to improve our current knowledge of this syndrome.
Massin
N
N
Department of Endocrinology and Reproductive Medicine, Necker-Enfants Malades Hospital, Faculty of Medicine, Paris V University, Paris, France.
Czernichow
C
C
Thibaud
E
E
Kuttenn
F
F
Polak
M
M
Touraine
P
P
D000293:Adolescent; D000328:Adult; D005260:Female; D042341:Gonadal Hormones / Q000097:blood; D006801:Humans; D010535:Laparoscopy; D010053:Ovary / Q000000981:diagnostic imaging / Q000503:physiopathology; D010908:Pituitary Hormones, Anterior / Q000097:blood; D016649:Primary Ovarian Insufficiency / Q000097:blood / Q000209:etiology* / Q000503:physiopathology; D014463:Ultrasonography
D042341:Gonadal Hormones; D010908:Pituitary Hormones, Anterior