Reproductive dysfunction in women with Albright's hereditary osteodystrophy

Anne B. Namnoum, George R. Merriam, Arnold M. Moses, Michael A. Levine

    Research output: Contribution to journalArticle

    Abstract

    Most individuals with Albright's hereditary osteodystrophy (AHO) have deficient expression or function of G(sα), 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(sα) 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°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 μg 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.

    Original languageEnglish (US)
    Pages (from-to)824-829
    Number of pages6
    JournalJournal of Clinical Endocrinology and Metabolism
    Volume83
    Issue number3
    DOIs
    StatePublished - 1998

    Fingerprint

    Parathyroid Hormone
    Gonadotropins
    Adenylyl Cyclases
    Gs GTP-Binding Protein alpha Subunits
    Guanine Nucleotides
    Gonadotropin-Releasing Hormone
    Reproductive History
    Carrier Proteins
    Hormones
    Sexual Development
    Follicular Phase
    Albright's hereditary osteodystrophy
    Erythrocytes
    Incidence
    Serum

    ASJC Scopus subject areas

    • Biochemistry
    • Endocrinology, Diabetes and Metabolism

    Cite this

    Reproductive dysfunction in women with Albright's hereditary osteodystrophy. / Namnoum, Anne B.; Merriam, George R.; Moses, Arnold M.; Levine, Michael A.

    In: Journal of Clinical Endocrinology and Metabolism, Vol. 83, No. 3, 1998, p. 824-829.

    Research output: Contribution to journalArticle

    Namnoum, Anne B. ; Merriam, George R. ; Moses, Arnold M. ; Levine, Michael A. / Reproductive dysfunction in women with Albright's hereditary osteodystrophy. In: Journal of Clinical Endocrinology and Metabolism. 1998 ; Vol. 83, No. 3. pp. 824-829.
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