Abstract
Background: Insulin Resistance Syndrome (IRS) occurs either due to mutation or auto-antibodies to insulin receptor. Presentation as diabetic ketoacidosis (DKA) although rare has been reported in few case reports. We report a young male presenting with DKA with very high insulin requirement.
Case Presentation: A 17-year-old male, with untreated diabetes mellitus diagnosed at 12 years of age, presented with vomiting and kussmaul breathing. On examination he was emaciated, had short stature; severe acanthosis nigricans and hypertrichosis. At admission, his plasma glucose was 470 mg/dl, HCO3 6 mEq/L with positive ketones. He was diagnosed as severe DKA and treated with insulin with dose requirement of 120 units/day for adequate glycemic control. GAD-65 antibodies were negative and he had dyslipidemia but no organomegaly. Genetic test was sent for a suspicion of insulin resistance or lipodystrophy which showed homozygous mutation in INSR gene previously described in Rabson Mendenhall syndrome. The child was treated with insulin, metformin and pioglitazone.
Conclusion: Type 1 DM is the most common etiology for DKA. But in patients with very high insulin requirement and signs of insulin resistance, possibility of IRS should be kept and genetic testing offered.