SAT-291 Gonadotropin Releasing Hormone Agonist Therapy Improves Hyperandrogenism in an Adolescent with Type A Insulin Resistance Syndrome

• DOI: 10.1210/js.2019-sat-291
• Publication Date: 2019-05-10T07:30:24Z
• Authors: Emily Paprocki; Romina Barral; Tania Burgert
• Keywords: Hyperandrogenism; hirsutism; Acanthosis Nigricans

Background: Type A insulin resistance (IR) is a form of severe IR due to heterozygous mutations in the receptor gene. It presents with acanthosis nigricans, IR, and hyperandrogenism absence obesity or lipodystrophy. Treatment aims decrease androgens improve sensitivity. We describe first adolescent patient having successful resolution clinical biochemical during GnRH agonist treatment. Clinical Case: An female was referred for secondary amenorrhea hirsutism. She had normal BMI prominent Laboratories revealed LH 11.6 mIU/mL, FSH 4.2 elevated total free testosterone 96 2.21 ng/dL, respectively, glucose HbA1c (5.6 %). diagnosed PCOS OGTT as per standard care our program. Fasting 80 mg/dL fasting level 63.1 mIU/mL. The 2-hour 199 1480 uIU/mL. Pelvic ultrasound showed enlarged ovaries. Due dysglycemia, lipids, entertained. Genetic testing mutation gene missense variant pointing towards diagnosis IR.The been prescribed treatment metformin spironolactone although her adherence taking medications sporadic. then trialed on an oral contraceptive which led monthly menstrual cycles. However, levels remained at 139 3.06 ng/dL respectively. Her hirsutism not improved depression. Twelve months from initial presentation, decision made try leuprolide she subsequently began 11.25 mg/month intramuscular injections. continued well. One month after injection, follow-up labs normalization levels, 7.1 0.16 decreased 0.3 Hirsutism markedly improved. Five into therapy, repeat still impaired tolerance (0-minute 91 mg/dL, 90.9 uIU/mL; 181 1038 uIU/mL) along increased (6.4%). Conclusion: who experienced bidirectional relationship between including LH-independent effects steroidogenesis has debated. Given patient’s reduction despite persistent hyperinsulinism, this case challenges idea that increases independently gonadotropins. therapy should be considered cases IR.