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Severe Insulin-Resistance Hyperandrogenic Syndromes

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Androgen Excess Disorders in Women

Part of the book series: Contemporary Endocrinology ((COE))

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Abstract

Insulin resistance is associated with a wide variety of markedly heterogeneous clinical disorders, either inherited or acquired, that may result in acanthosis nigricans, ovarian hyperandrogenism, and ovulatory dysfunction. These include the type A (primarily affecting lean women and resulting from defects of the insulin receptor), type B (resulting from an autoimmune process affecting the insulin receptor), and type C (a variant of type A, the hyperandrogenic, insulin-resistant, and acanthosis nigricans [HAIR-AN] syndrome) insulin resistance syndromes. More rare syndromes include leprechaunism, the Rabson-Mendenhall syndrome, and a heterogeneous group of lipodystrophic syndromes. HAIR-AN is generally present in obese women who do not demonstrate defects of the insulin receptor, although they may exhibit postreceptor defects. Although the type A and B insulin resistance syndromes are rare causes of ovarian hyperandrogenism, type C may affect up to 3% of women with androgen excess. These patients may be treated as other women with ovarian hyperandrogenism, namely using lifestyle modification, oral contraceptives, insulin sensitizers, and antiandrogens, although a few patients may require further suppression of their hypothalamic-pituitary-ovarian axis with a long-acting gona-dotropin-releasing hormone analog. It is likely that HAIR-AN patients are at greater risk for metabolic dysfunction and diabetes mellitus, although long-term studies are lacking. Finally, the distinction between HAIR-AN and the polycystic ovary syndrome (PCOS) is becoming less clear as more patients with PCOS are being recognized as potentially having postreceptor abnormalities in insulin signaling. Better characterization awaits more detailed phenotyping and genotyping studies.

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Author information

Authors and Affiliations

  1. Department of Molecular Endocrinology, Merck Research Laboratories, Rahway, NJ

    David E. Moller

  2. Metabolic Medicine, Addenbrooke’es Hospital, Cambridge, UK

    Antonio Vidal-Puig

  3. Department of Obstetrics and Gynecology, Cedars-Sinai Medical Center; Department of Obstetrics and Gynecology, and Department of Medicine, The David Geffen School of Medicine at University of California, Los Angeles, CA

    Ricardo Azziz

Authors
  1. David E. Moller
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  2. Antonio Vidal-Puig
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  3. Ricardo Azziz
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Editor information

Editors and Affiliations

  1. Center for Androgen Related Disorders, Cedars-Sinai Medical Center; and Department of Obstetrics and Gynecology, and Department of Medicine, The David Geffen School of Medicine at UCLA, Los Angeles, CA

    Ricardo Azziz MD, MPH, MBA

  2. Department of Internal Medicine, Virginia Commonwealth University, Richmond, VA

    John E. Nestler MD

  3. Department of Endocrine Gynaecology and Reproductive Medicine, Lille University Hospital, Lille, France

    Didier Dewailly MD

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© 2006 Humana Press Inc., Totowa, NJ

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Moller, D.E., Vidal-Puig, A., Azziz, R. (2006). Severe Insulin-Resistance Hyperandrogenic Syndromes. In: Azziz, R., Nestler, J.E., Dewailly, D. (eds) Androgen Excess Disorders in Women. Contemporary Endocrinology. Humana Press. https://doi.org/10.1007/978-1-59745-179-6_11

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  • DOI: https://doi.org/10.1007/978-1-59745-179-6_11

  • Publisher Name: Humana Press

  • Print ISBN: 978-1-58829-663-4

  • Online ISBN: 978-1-59745-179-6

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