Ovarian resistance to luteinizing hormone: a novel cause of amenorrhea and infertility.
OBJECTIVE
To report the clinical, hormonal, and histopathological features of a woman with ovarian resistance to LH.


METHODS
Clinical study.


METHODS
University hospital.


METHODS
A woman with amenorrhea, sister of a patient with male pseudohermaphroditism due to Leydig cell hypoplasia.


METHODS
Blood drawing before and after GnRH stimulation and also after dexamethasone and hCG administration, pelvic ultrasound, and ovarian biopsy.


METHODS
Karyotype, gonadotropin and steroid measurements, follicular diameter, ovarian histology, and sequencing of the LH receptor gene.


RESULTS
Patient had normal female external genitalia, normal breast development at puberty, rare episodes of vaginal bleeding, and infertility. The karyotype was 46,XX. She had elevated serum LH levels, whereas E2 and P concentrations were in the range seen in the early follicular phase. Pelvic ultrasound revealed a slightly hypoplastic uterus and enlarged polycystic ovaries. A normal follicular reserve for age, antral follicles, and absence of corpora lutea or albicans were observed on ovarian biopsy. Exon 11 of the LH receptor gene had a normal sequence.


CONCLUSIONS
In our patient with ovarian resistance to LH, FSH stimulated follicular development until the preovulatory stage, but E2 levels remained in the early follicular phase range, still sufficient for normal pubertal feminization. Apparently, LH is necessary for ovulation and corpus luteum formation.
Arnhold
I J
IJ
Hospital das Clinicas, University of São Paulo School of Medicine, Brazil.
Latronico
A C
AC
Batista
M C
MC
Carvalho
F M
FM
Chrousos
G P
GP
Mendonça
B B
BB
D000328:Adult; D000568:Amenorrhea / Q000209:etiology* / Q000235:genetics; D004247:DNA / Q000235:genetics; D012734:Disorders of Sex Development / Q000235:genetics; D004351:Drug Resistance; D005260:Female; D006062:Gonadotropins / Q000097:blood; D006801:Humans; D007247:Infertility, Female / Q000209:etiology* / Q000235:genetics; D007621:Karyotyping; D007986:Luteinizing Hormone / Q000502:physiology*; D008297:Male; D006080:Ovarian Follicle / Q000473:pathology; D010053:Ovary / Q000473:pathology / Q000502:physiology*; D011085:Polycystic Ovary Syndrome / Q000000981:diagnostic imaging; D011974:Receptors, LH / Q000235:genetics; D013256:Steroids / Q000097:blood; D014463:Ultrasonography; D014599:Uterus / Q000000981:diagnostic imaging
D006062:Gonadotropins; D011974:Receptors, LH; D013256:Steroids; D007986:Luteinizing Hormone; D004247:DNA