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Reproductive dysfunction in women with Albright's hereditary osteodystrophy.
Namnoum, A B; Merriam, G R; Moses, A M; Levine, M A.
Afiliación
  • Namnoum AB; Department of Gynecology and Obstetrics, The Johns Hopkins University School of Medicine, Baltimore, Maryland 21287, USA.
J Clin Endocrinol Metab ; 83(3): 824-9, 1998 Mar.
Article en En | MEDLINE | ID: mdl-9506735
Most individuals with Albright's hereditary osteodystrophy (AHO) have deficient expression or function of G(s alpha), the alpha subunit of the guanine nucleotide binding protein that stimulates adenylyl cyclase, and are resistant to parathyroid hormone (PTH) and other hormones that act via stimulation of adenylyl cyclase. To determine the incidence and etiology of ovarian dysfunction in women with AHO, we examined the reproductive history and hypothalamic-pituitary-ovarian axis in 17 affected women aged 17-43 yr. All patients had typical PTH resistance and an approximately 50% reduction in erythrocyte G(s alpha) activity, (0.43 +/- 0.03 vs. 0.92 +/- 0.08 for normal control subjects, P < 0.001). Fourteen of the 17 patients (76%) were oligomenorrheic or amenorrheic, more than half had delayed or incomplete sexual development, and only two had a history of earlier pregnancy. Most women were mildly hypoestrogenic, with normal to slightly elevated serum gonadotropin levels. Computer analysis of 24 hour LH measurement showed that the frequency of LH peaks/24 h in AHO women varied widely, but as a group they were not statistically different from a group of normal women studied in the early follicular phase. Administration of 100 microg synthetic GnRH produced normal FSH and LH responses. We conclude that reproductive dysfunction is common in women with AHO and probably represents partial resistance to gonadotropins.
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Reproducción / Displasia Fibrosa Poliostótica Tipo de estudio: Etiology_studies Límite: Adolescent / Adult / Female / Humans Idioma: En Revista: J Clin Endocrinol Metab Año: 1998 Tipo del documento: Article País de afiliación: Estados Unidos
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Colección: 01-internacional Base de datos: MEDLINE Asunto principal: Reproducción / Displasia Fibrosa Poliostótica Tipo de estudio: Etiology_studies Límite: Adolescent / Adult / Female / Humans Idioma: En Revista: J Clin Endocrinol Metab Año: 1998 Tipo del documento: Article País de afiliación: Estados Unidos
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