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  • Hyperandrogenism, insulin resistance and acanthosis nigricans (HAIR-AN syndrome): an extreme subphenotype of polycystic ovary syndrome

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Rare disease
Case report
Hyperandrogenism, insulin resistance and acanthosis nigricans (HAIR-AN syndrome): an extreme subphenotype of polycystic ovary syndrome
  1. Brooke O'Brien1,2,
  2. Rachana Dahiya2,3 and
  3. Rebecca Kimble1,4
  1. 1Paediatric and Adolescent Gynaecology, Department of Paediatric Surgery, Queensland Children's Hospital, South Brisbane, Queensland, Australia
  2. 2School of Medicine, The University of Queensland, St Lucia, Queensland, Australia
  3. 3Department of Endocrinology, Queensland Children's Hospital, South Brisbane, Queensland, Australia
  4. 4Women's and Newborn Services, Royal Brisbane and Woman's Hospital Health Service District, Herston, Queensland, Australia
  1. Correspondence to Dr Brooke O'Brien; drbrookeob{at}gmail.com

Abstract

HAIR-AN—a syndrome of hyperandrogenism (HA), insulin resistance (IR) and acanthosis nigricans (AN)—is a specific subphenotype of polycystic ovary syndrome (PCOS), and it is seen in almost 5% of all women with hyperandrogenism. An adolescent girl aged 11 years old was referred with adrenarche, hyperandrogenism and obesity commencing at age 8. Clinical and biochemical investigations confirmed significant hyperandrogenism and insulin resistance, and a diagnosis of HAIR-AN syndrome was made after exclusion of other differential diagnoses. HAIR-AN syndrome is an important diagnosis for the adolescent gynaecologist to be aware of, and it requires a multidisciplinary approach, including endocrinology input, for optimal management. Weight loss, lifestyle modification and combined hormonal pill and metformin are considered first-line treatment.

  • obstetrics and gynaecology
  • metabolic disorders

https://doi.org/10.1136/bcr-2019-231749

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    Footnotes

    • Contributors BO: patient care, first author, collection data and writing clinical case, literature review, drafting and revising clinical work, revision manuscript. RD: patient care, contribution and revision of manuscript. RK: patient care, critical review of paper, final approval for paper to be published.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Parental/guardian consent obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.