Hyperandrogenism-insulin resistance-acanthosis nigricans (HAIR-AN) syndrome is a special and rare subtype of polycystic ovarian syndrome. It can lead to hyperandrogenism (HA), insulin resistance (IR), and acanthosis nigricans (AN) accompanied by acne, hirutism, irregular menstruation, and other androgen excess symptoms. A case of pediatric HAIR-AN syndrome with severe AN was admitted to the Department of Endocrinology, China-Japan Friendship Hospital. The patient's clinical manifestations, laboratory data, imaging features, and gene sequencing were analyzed, and the patient was diagnosed with pediatric HAIR-AN syndrome. Obesity, IR, hyperglycemia, menstrual disorder, and AN were significantly improved after treating with metformin and liraglutide. HAIR-AN syndrome occurs in various forms. When the patient appears unexplained acanthosis nigricans and menstrual disorders, the disease should be considered possible. Early diagnosis and symptomatic supportive treatment can improve the quality of life.
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http://dx.doi.org/10.11817/j.issn.1672-7347.2023.230100 | DOI Listing |
Zhong Nan Da Xue Xue Bao Yi Xue Ban
December 2023
Department of Endocrinology, China-Japan Friendship Hospital, Beijing 100029, China.
Hyperandrogenism-insulin resistance-acanthosis nigricans (HAIR-AN) syndrome is a special and rare subtype of polycystic ovarian syndrome. It can lead to hyperandrogenism (HA), insulin resistance (IR), and acanthosis nigricans (AN) accompanied by acne, hirutism, irregular menstruation, and other androgen excess symptoms. A case of pediatric HAIR-AN syndrome with severe AN was admitted to the Department of Endocrinology, China-Japan Friendship Hospital.
View Article and Find Full Text PDFJCEM Case Rep
January 2023
Department of Medicine, University of Rochester School of Medicine and Dentistry, Rochester, New York 14642, USA.
Hyperandrogenism, insulin resistance, and acanthosis nigricans (HAIR-AN) is a severe subphenotype of polycystic ovary syndrome (PCOS). A 32-year-old woman with HAIR-AN and class 3 obesity presented to an endocrinology clinic after she failed sequential trials of treatment with metformin, estrogen-progestin OCP, spironolactone, leuprolide, and a levonorgestrel intrauterine device. She complained of hirsutism and acanthosis nigricans severely affecting her quality of life and had secondary amenorrhea.
View Article and Find Full Text PDFMol Genet Metab Rep
June 2023
Unit of Biochememistry, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Vasco de Quiroga #15, Belisario Dominguez, Sección XVI, Tlalpan, 14080 Mexico City, Mexico.
Severe insulin resistance can be caused by rare genetic defects in the insulin receptor known as insulin receptoropathies. These genetic defects cause a wide spectrum of clinical manifestations ranging from mild syndromes to lethal disorders. Among those is the HAIR-AN an extreme subtype of polycystic ovary syndrome (PCOS).
View Article and Find Full Text PDFRev Med Liege
December 2021
Service de Gynécologie-Obstétrique, CHU Liège, Belgique.
Natl J Maxillofac Surg
March 2021
Department of Pediatric Dentistry, Rural Dental College, Pravara Institute of Medical Sciences, Loni, Maharashtra, India.
Insulin Resistance syndromes (IR's), are a group of genetic disorders caused due a functional defect in chromosome 19p13. It is an autosomal recessive condition. Donohue Syndrome was initially described by Donohue and Uchida in 1948 and 1954, a case of sisters born to parents with a first-degree consanguineous marriage.
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