Study Objective: The HAIR-AN syndrome, a chronic anovulatory state, is characterized by hyperandrogenism, insulin resistance, and acanthosis nigricans (HAIR-AN). To better characterize the interrelationship between steroidogenesis and carbohydrate metabolism in the initiation of the HAIR-AN syndrome, we report pilot studies of adrenal steroidogenesis and insulin sensitivity in adolescent girls. Design: Adrenocorticotropin (ACTH) stimulation tests were performed to assess the relative adrenal contribution to hyperandrogenism and exclude errors in steroidogenesis. Oral glucose tolerance tests (OGTT) were performed to determine insulin sensitivity. Participants: We studied four obese adolescent girls referred for evaluation of hirsutism and/or menstrual irregularities who were found to have acanthosis nigricans. Results: All four girls had elevated basal testosterone levels; 3 had an elevated 17-hydroxypregnenolone (17Preg) incremental elevation at 30 minutes; all had insulin resistance during OGTT; two also had impaired glucose tolerance. Conclusions: These patients fulfill the diagnostic criteria for the HAIR-AN syndrome. Two of four girls had responses to ACTH stimulation consistent with dysregulation of 17α-hydroxylase/17,20-lyase (P450c17), one of several postulated causes for chronic anovulatory syndromes. One girl had an elevated 17-Preg incremental elevation and an increased stimulated 17-Preg/17hydroxyprogesterone (17-Prog) ratio, responses consistent with decreased 3β-hydroxysteroid dehydrogenase activity. The fourth patient appears to have normal steroidogenesis. This preliminary work is consistent with etiologic heterogeneity of the HAIR-AN syndrome.
All Science Journal Classification (ASJC) codes
- Pediatrics, Perinatology, and Child Health
- Obstetrics and Gynecology