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Horm Metab Res 1993; 25(12): 646-648
DOI: 10.1055/s-2007-1002198
Originals Clinical

© Georg Thieme Verlag, Stuttgart · New York

Carbohydrate-Deficient Glycoprotein Syndrome in a Girl with Hypogonadism Due to Inactive Follicle Stimulating Hormone

T. Ohzeki, Hiroko Motozumi, K. Hanaki, Hiroko Ohtahara, H. Urashima, T. Tsukuda, S. Kobayashi, K. Shiraki, K. Ohno
  • Department of Pediatrics and Division of Pediatric Neurology, Faculty of Medicine, Tottori University, Yonago, Japan
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Publikationsverlauf

1993

1993

Publikationsdatum:
14. März 2008 (online)

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Summary

We report a very rare case of a girl with hypogonadism probably due to inactive FSH. She had been diagnosed as carbohydrate-deficient glycoprotein syndrome. The 14-year-old patient had no secondary sexual characteristics with the 46,XX karyotype and estradiol (E2) levels were undetectable. Follicle stimulating hormone (FSH) levels were extremely high and responded exaggeratedly to luteinizing hormone-releasing hormone (LH-RH). The E2 levels, on the other hand, were elevated after stimulation with human menopausal gonadotropin (75 units/day) for 5 days, suggesting biologically inactive FSH in the circulation. Abnormal structure in the glycoprotein may be possibly related with hypogonadism.

Key words

Follicle Stimulating Hormone - Hypogonadism - Glycoprotein - Human Menopausal Gonadotropin