Weaning infants with short bowel syndrome associated intestinal failure from parenteral nutrition: An ERNICA multicentre survey

Background To explore and compare practices of European Intestinal Rehabilitation Centres participating in the European Reference Network for rare Inherited and Congenital Anomalies (ERNICA) intestinal failure (IF) working group when weaning short bowel syndrome (SBS)-IF infants from parenteral nutrition (PN).Material and methods Questionnaires were distributed by email to 49 ERNICA centres. Details regarding oral/nasogastric (NG) feeding when weaning PN, use of protocols/guidelines, criteria for PN cycling/reducing infusion hours, dietary recommendations, use of synthetic glucagon-like peptide-2 (teduglutide), antibiotics for intestinal bacterial overgrowth and long-term follow-up practices were requested in children with neonatal onset SBS-IF. Descriptive statistical analysis was conducted.Results Thirty-five/49centers from 15 countries participated. In non-thriving infants aged  6months if needed. Local/national guidelines were used by 63% when weaning PN. Criteria for cycling PN included age (range: term-3 months), weight (3-4.5 kg), and enteral feed tolerance with 95% centres reducing PN infusion rate before disconnecting. In 88.6% centres patients were monitored until transitioned to adult care (even if weaned from PN). Dietary recommendations and antibiotic usage varied widely. Teduglutide was used in 23/25(92%) centres from 11 countries where it was approved and funded.Conclusion A variety of nutritional strategies were used to wean PN in SBS-IF infants even in specialist centres using guidelines. NG-tube feeding was used alongside oral intake in most centres, but other practices differed widely. Multicentre studies investigating which practices are associated with the best outcome for infants with SBS-IF are needed to develop evidence-based guidelines.