P-251: Diabetes mellitus in children with Prader-Willi syndrome at Vietnam national children’s hospital
T A T Tran, P T Bui, N K Nguyen, T B N Can, T T M Do, T H Nguyen, T K G Dang, T T H Dang, H L Nguyen, T H Nguyen, T M Do, T X Bui, T H Bui, C D VuAbstract
Introduction
Prader–Willi syndrome (PWS) is a multisystem complex disorder characterized by hypothalamic dysfunction leading to hyperphagia, obesity, endocrine abnormalities and developmental delays. Diabetes mellitus in PWS is typically type 2, due to severe insulin resistance related to obesity. The prevalence of diabetes in children with Prader–Willi syndrome ranges from 2% to 17% across different reports.
Objectives
To evaluate the prevalence of diabetes, the clinical and biochemical characteristics in patients with Prader–Willi syndrome and diabetes.
Methods
This was a cross-sectional case series study, 14 children with PWS were diagnosed with diabetes at the Center for Endocrinology, Metabolism, Genetics and Molecular Therapy, Vietnam National Children’s Hospital from 1/2009-12/2025. Patients were evaluated for clinical features, BMI, blood glucose, HbA1c, total cholesterol, arterial blood gas. Statistical analyses were performed using SPSS, with BMI calculations facilitated by the “anthro” package.
Results
Fourteen out of 278 children with Prader–Willi syndrome (5.39%) were diagnosed with diabetes. The median age at diagnosis was 8.8 years (IQR: 8.3–13.5). All patients were obese, with a median BMI of 33.6 kg/m2 (IQR: 29.7–38.1). Acanthosis nigricans was present in 9/14 patients. The median fasting blood glucose level at the time of diagnosis was 11.2 mmol/L (IQR: 6.4–16.4), the median HbA1c was 9.51% (IQR: 7.31–11.9), and the median total plasma cholesterol was 4.9 mmol/L (IQR: 3.4–6.8), with 7/14 patients having hypercholesterolemia, and no patients had diabetic ketoacidosis. Only one patient was receiving growth hormone therapy at the time of diagnosis and discontinued treatment four years after the diagnosis of diabetes.
Conclusions
Children with PWS are at significant risk of early-onset T2D and associated metabolic complications. These findings highlight the need for early metabolic screening and targeted management in children with PWS.