HAIR-AN syndrome
Classification according to ICD-10 | |
---|---|
E28.2 | Polycystic Ovarian Syndrome |
ICD-10 online (WHO version 2019) |
The HAIR-AN syndrome , acronym for H yper A ndrogenämie , I nsulin R esistenz and A canthosis N igricans , is a very rare congenital disease with the eponymous principal features.
The syndrome can be viewed as a subtype of polycystic ovary syndrome in which there is pronounced insulin resistance.
distribution
The disease affects (almost) only women. About 5–10% of women with hyperandrogenemia have HAIR-AN syndrome, up to 40% of young women with menstrual disorders can have this syndrome.
Sometimes there is an association with autoimmune diseases such as Hashimoto's thyroiditis and Graves' disease .
root cause
The disease can be caused by two different changes:
- Antibodies against the insulin receptor
- lack of or reduced number or function of insulin receptors due to a genetic defect.
The increased insulin level leads to an increase in androgen levels .
Clinical manifestations
Clinical criteria are:
- Hyperandrogenemia with hirsutism , acne , seborrhea , menstrual disorder
- Insulin resistance
- Acanthosis nigricans
diagnosis
The diagnosis is based on the clinical findings and the changed laboratory values in the blood serum.
Differential diagnosis
Must be distinguished are the PCOS and insulin resistance syndrome type A .
therapy
Treatment can include antiandrogens and weight loss measures .
literature
- M. Peigné, A. Villers-Capelle, G. Robin, D. Dewailly: Hyperandrogénie féminine. In: Presse medicale. Vol. 42, No. 11, November 2013, pp. 1487-1499, doi: 10.1016 / j.lpm.2013.07.016 , PMID 24184282 (review).
- R. Azziz, E. Carmina, D. Dewailly, E. Diamanti-Kandarakis, HF Escobar-Morreale, W. Futterweit, OE Janssen, RS Legro, RJ Norman, AE Taylor, SF Witchel: The Androgen Excess and PCOS Society criteria for the polycystic ovary syndrome: the complete task force report. In: Fertility and sterility. Vol. 91, No. 2, February 2009, pp. 456-488, doi: 10.1016 / j.fertnstert.2008.06.035 , PMID 18950759 (review).
- KK McClanahan, HA Omar: Navigating adolescence with a chronic health condition: a perspective on the psychological effects of HAIR-AN syndrome on adolescent girls. In: TheScientificWorldJournal. Vol. 6, October 2006, pp. 1350-1358, doi: 10.1100 / tsw.2006.242 , PMID 17072487 (review).
- HA Omar, S. Logsdon, J. Richards: Clinical profiles, occurrence, and management of adolescent patients with HAIR-AN syndrome. In: TheScientificWorldJournal. Vol. 4, July 2004, pp. 507-511, doi: 10.1100 / tsw.2004.106 , PMID 15258677 .
Individual evidence
- ↑ Encyclopedia Dermatology
- ↑ a b c d A. H. Dédjan, A. Chadli u. a .: Hyperandrogenism-Insulin Resistance-Acanthosis Nigricans Syndrome. In: Case Reports in Endocrinology. 2015, 2015, p. 1, doi: 10.1155 / 2015/193097 .
- ^ KM Rager, HA Omar: Androgen excess disorders in women: the severe insulin-resistant hyperandrogenic syndrome, HAIR-AN. In: TheScientificWorldJournal. Vol. 6, January 2006, pp. 116-121, doi: 10.1100 / tsw.2006.23 , PMID 16435040 (review).
- ↑ a b K. B. Elmer, RM George: HAIR-AN syndrome: a multisystem challenge. In: American family physician. Vol. 63, No. 12, June 2001, pp. 2385-2390, PMID 11430453 .
- ↑ M. Manco, M. Castagneto, G. Nanni, C. Guidone, V. Tondolo, AV Greco, G. Gasbarrini, G. Mingrone: Biliopancreatic diversion as a novel approach to the HAIR-AN syndrome. In: Obesity surgery. Vol. 15, No. 2, February 2005, pp. 286-289, doi: 10.1381 / 0960892053268435 , PMID 15802076 .