PSUN141 Suspected Lipodystrophy in a Thin Young Man With Type 1 Diabetes

INTRODUCTION: Lipodystrophies are a group of exceptionally rare adipose disorders typified by complete or partial lack of adipose tissue. They come in two forms, congenital and acquired. Global prevalence is approximately 3 cases per million people. Despite their rarity, lipodystrophies often have common derangements, including, insulin resistance, decreased leptin, lipid abnormalities, and non-alcoholic fatty liver disease. Identifying and diagnosing these disorders is challenging as so few cases exist and may easily be confused for a variety of other illnesses. CASE: Our patient was a 21 year old African American Male with BMI of 14.0 kg/m(2) (46.72 kg, 28.35 cm), with antibody proven type 1 diabetes mellitus diagnosed and extreme weight loss at age 12. His diabetes has been poorly controlled since diagnosis, with episodes of DKA, and a persistently elevated A1c. He has evidence of insulin resistance, requiring about 2.57 units per kilogram of insulin daily. In spite of this fasting blood glucose routinely exceeds 300 mg/dL. Physical exam significant for a generalized lack of adipose tissue. Most recent laboratory results showed: HbA1c >14% (<5.7%), fasting plasma glucose 419 mg/dL (70-99 mg/dL), c-peptide <0.10 ng/mL (0.80-3.85 ng/dL), and GAD65 Ab 18 IU/mL (<5 IU/mL). Interestingly patient also had a decreased leptin of 0.5 ng/mL (1.8-19.9 ng/mL), a known feature of lipodystrophy. However, triglycerides are only 158 mg/dL (<150 mg/dL), which may point more toward isolated leptin deficiency without lipodystrophy. Modified insulin challenge was administered in office to differentiate between true insulin resistance and poor compliance. Insulin lispro 0.3 units per kilogram subcutaneous (14 units total) was administered at time zero, with pre-test SMBG 575 mg/dL. Over the three-hour test, blood sugars reached a nadir of 297 mg/dL. Patient was observed throughout and did not eat or drink anything but water during the test. He showed no signs or symptoms of hypoglycemia at any time during the challenge. Results indicated significant insulin resistance for his body weight. CONCLUSION: Physical exam features, labs, including leptin of 0.5 ng/mL, and relatively severe insulin resistance suggest possible lipodystrophy or isolated leptin deficiency. However, triglycerides were not extremely elevated as expected in lipodystrophy. We have ordered metreleptin for further elucidation of patient's condition. Disclaimer: This research was supported (in whole or in part) by HCA Healthcare and/or an HCA Healthcare affiliated entity. The views expressed in this publication represent those of the author(s) and do not necessarily represent the official views of HCA Healthcare or any of its affiliated entities. Presentation: Sunday, June 12, 2022 12:30 p.m. - 2:30 p.m.