hrp0089p2-p351 | Sex Differentiation, Gonads and Gynaecology or Sex Endocrinology P2 | ESPE2018

Two Unrelated Cases of Severe Insulin Resistance Due to Insulin Receptor Mutation Discovered During Adolescence

Maryam Azgal , Natacha Bouhours-Nouet , Odile Camard , Ingrid Allix , Valentine Suteau , Justine Bailleul , Marie-Neige Campas , Regis Coutant

Introduction: Severe insulin resistance due to insulin receptor mutation is usually diagnosed at the neonatal period (Donohue and Rabson-Medenhall syndromes), or later in a type A insulin resistance syndrome. We report here two cases of insulin receptor mutation whose presenting signs were less noticeable.Observation: A 13-year-old girl was referred for short stature (Height −2.5 SDS) with SGA (birth length 44.5 cm, at gestational age 41 weeks). Cl...