8193 Mauriac Syndrome Uncovered, The Insulin Access Impact in Type 1 Diabetes Mellitus

Abstract

Abstract Disclosure: D.H. Sacoto: None. R. Belokovskaya: None. K. Gebran: None. K. Madani: None. A. De Rosairo: None. D. Patel: None. A.A. Franco-Akel: None. Introduction: Mauriac syndrome is a rare complication of poorly controlled type 1 diabetes mellitus (T1DM). It is reversible with appropriate glycemic control; however, factors affecting healthcare access to insulin are leading to its re-emergence. We present a case of Mauriac syndrome as a complication of uncontrolled T1DM due to insulin access. Case: A 22-year-old Hispanic female with a history of T1DM presented to the emergency department with a two-day history of abdominal pain. She was diagnosed with diabetic ketoacidosis (DKA) (blood glucose: 802 mg/dL; pH 7.14; bicarbonate: 7 mmol/L; anion gap: 34; positive serum ketones). Incidental transaminitis was noted. R factor showed a cholestatic pattern of 0.6 (AST: 136 U/L, ALT: 49 U/L, alkaline phosphatase:286 U/L). US liver revealed an enlarged liver (craniocaudal: 16.5 cm) without biliary obstruction. Her phenotype consisted of short stature (4.8 feet) with a moon face and a protuberant abdomen. Her records revealed a normal karyotype 46 XX, with a bone age of 4 years, pubertal delay, low insulin-like growth factor-1 levels (58 ng/mL), and long-standing hyperlipidemia. Over the years, she experienced multiple DKA episodes in the context of uncontrolled T1DM (hemoglobin A1c:11-13 %). The main problem identified included difficulty with insulin access, specifically related to insurance coverage as well as managing her disease. Glycemic control has significantly improved with continuous glucose monitoring and updated insurance status. Discussion: Mauriac syndrome is characterized by hepatomegaly, transaminitis, delayed pubertal maturation, growth failure, and cushingoid features. Hyperglycemia leads to intrahepatic glycogen deposition and the lack of insulin to lipolysis and ketosis, leading to high cortisol levels and growth delay. Factors leading to uncontrolled T1DM include lack of insulin access. The high cost is considered a major barrier, with an average price per millimeter of insulin tripled from 231 to 736 US dollars. Even patients with insurance tend to have high co-pays and deductibles, with a 61% chance of delay or denial due to pre-approval requirements. Lower socioeconomic status, lack of access to devices such as insulin pumps, migratory status, social support, and ethnic background are other factors related to glycemic control. Our patient belongs to a family of immigrants with low socioeconomic status, which might have contributed to the development of Mauriac syndrome. Since clinical and laboratory abnormalities are reversible with appropriate glycemic control, advocating for policies that facilitate better insulin access is paramount. Presentation: 6/2/2024