Dexamethasone suppressible hypergonadotropism in an adolescent patient with Prader-Willi syndrome.
We report an adolescent patient with Prader-Willi syndrome accompanying suppressible hypergonadotropism. The subject is an 18-year-old female. She was obese (body mass index: 35.7) and hypomyotonic with mental retardation. On endocrinological examination, a high serum LH concentration and hyperresponsiveness of luteinizing hormone (LH) to intravenously administered LH-Releasing Hormone (LH-RH) were observed, while the basal follicle stimulating hormone level was within the normal range. In addition, serum dehydroxyepiandrosterone sulfate (DHEA-S) was also increased. Following 2 mg dexamethasone administration for 7 days, serum LH and DHEA-S were almost normalized and hyperresponse of LH to LH-RH completely disappeared. The present study provides evidence that altered responsiveness to adrenal steroid may be involved in the establishment of hypergonadotropinism in an adolescent patient with Prader-Willi syndrome.
Shimizu
H
H
First Department of Internal Medicine, Gunma University School of Medicine, Maebashi, Japan.
Negishi
M
M
Takahashi
M
M
Shimomura
Y
Y
Uehara
Y
Y
Kobayashi
I
I
Kobayashi
S
S
D000293:Adolescent; D003687:Dehydroepiandrosterone / Q000031:analogs & derivatives* / Q000096:biosynthesis; D019314:Dehydroepiandrosterone Sulfate; D003907:Dexamethasone / Q000494:pharmacology*; D005260:Female; D005640:Follicle Stimulating Hormone / Q000097:blood; D007987:Gonadotropin-Releasing Hormone / Q000494:pharmacology*; D006801:Humans; D007986:Luteinizing Hormone / Q000097:blood*; D011218:Prader-Willi Syndrome / Q000150:complications / Q000188:drug therapy* / Q000378:metabolism
D007987:Gonadotropin-Releasing Hormone; D003687:Dehydroepiandrosterone; D019314:Dehydroepiandrosterone Sulfate; D003907:Dexamethasone; D007986:Luteinizing Hormone; D005640:Follicle Stimulating Hormone