P-37: Single-Center Experience with Teduglutide in Pediatric Patients with Short Bowel Syndrome Associated Intestinal Failure

Introduction: Intestinal failure (IF) is a chronic illness characterized by long-term dependence on parenteral support (PS) for nutrition and growth. The intestinal hormone, glucagon-like peptide 2 (GLP-2), induces mucosal proliferation, increases absorption, decreases gastric secretion, and slows transit time. Patients with short bowel syndrome (SBS) can lose GLP-2’s intestinotrophic effects. Teduglutide is a recombinant GLP-2 analog approved by the FDA in 2019 for pediatric patients with SBS-associated IF (SBS-IF). A small body of literature describes GLP-2 use in this population. We present our single-center experience with teduglutide in pediatric patients with SBS-IF. Methods: We retrospectively analyzed outcomes in patients with SBS-IF treated with teduglutide since FDA approval. Primary end-point was autonomy from PS, defined as parenteral nutrition (PN) or intravenous fluids (IVF). Secondary end-points included reduction in PS volume, days, and hours. Results: Six patients (50% male) were treated with teduglutide, with median age 5 years (range 1-12). The most common etiology of SBS-IF was necrotizing enterocolitis (83%). All patients had colon in continuity. The median duration of treatment was 10 months (range 3-12). PS requirement decreased by 1 or more days in two patients. One patient fully transitioned from PN to IVF after 12 months. Three patients received lower PS volume with a median reduction of 0.6 liters/week (range 0-2.4 L). One patient received PS for fewer hours per day. No patients achieved enteral independence. The most common side effects included abdominal discomfort and distention, which self-resolved or resolved with lower teduglutide dose. Three patients developed hypokalemia requiring potassium supplementation. Two patients required teduglutide discontinuation, one due to chronic pancreatitis and the other to severe electrolyte abnormalities. Conclusion: Our preliminary experience with teduglutide in children with SBS-IF demonstrates successful partial weaning from PS. Chronic pancreatitis and severe electrolyte abnormalities, including hypokalemia, leading to discontinuation of teduglutide have not been previously reported in pediatric patients. Close monitoring of electrolytes, pancreaticobiliary function and micronutrients is needed while on teduglutide. Further multicenter data will determine long-term efficacy and safety of teduglutide in children with SBS-IF.