ODP383 Clinical Presentation and Management of Diabetes Mellitus in Youth with Prader-Willi Syndrome

Abstract

Abstract Background Diabetes mellitus (DM) is reported in 7–24% of adults with PWS. It is thought that abnormal glucose metabolism develops as a consequence of severe obesity and/or insulin resistance. Given obesity and insulin resistance often develop in childhood in children with PWS, DM occurs in youth as well. There are limited data detailing the clinical presentation of DM, diabetes complications, and therapies used in pediatric patients with PWS. Methods A retrospective chart review of patients less than 21 years of age with genetically confirmed PWS seen July 2014 through July 2021 was performed. Patients were included if they also had a diagnosis of DM based on a single value meeting American Diabetes Association criteria: fasting blood glucose of ≥ 126 mg/dL, HbA1c ≥ 6.5%, 2-hour blood glucose of ≥ 200 mg/dL on oral glucose tolerance test, or random blood glucose of ≥ 200 mg/dL with symptoms of hyperglycemia. Results Of the 88 patients identified with PWS, nine (10%) were found to have DM. Means and range sat diabetes diagnosis were age 11.4 years (7–16), HbA1c8.2% (6.6– 12.4), BMI 38.7 kg/m2 (23.1–73.6),and BMI z-score2.4 (1.3–3.1). Five patients were female. Six patients were non-Hispanic white, two non-Hispanic black, and one Hispanic. Of these nine patients with DM, six had at least three diabetes autoantibodies assessed. All antibodies were negative in three patients, two patients had a weakly positive islet cell antibody only, and one patient had all four autoantibodies positive. Most patients were identified incidentally on screening laboratory evaluation in clinic, two patients had polyuria, and one patient was admitted with vomiting and mixed diabetic ketoacidosis/hyperosmolar hyperglycemic syndrome (DKA/HHS). Diabetes treatment methods included one patient on metformin only; four on metformin and insulin; three on metformin, insulin, and GLP1 receptor agonist; and one patient on insulin only. Of the six patients on insulin, only two were using short acting insulin with meals. No patients were treated using insulin pumps or continuous glucose monitors. No patients underwent bariatric surgery. Only one patient had hypertension requiring treatment with medication. No patients had nephropathy, dyslipidemia, neuropathy, or retinopathy. Conclusions DM is seen in children with PWS and often diagnosed incidentally. Regular screening is important as development of DKA/HHS is possible if diabetes goes unrecognized. While type 2 diabetes is more common than type 1 diabetes, diabetes antibodies should be measured. Standards of care for treatment are lacking resulting in variability in therapeutic regimens. Fortunately, despite markedly elevated BMI at DM diagnosis, diabetes comorbidities are not commonly seen. Presentation: No date and time listed