From childhood to adulthood in chronic intestinal failure: A nationwide study
Year: 2026
Type: article
Study design: Observational Study
Abstract: BACKGROUND AND AIMS: Pediatric chronic intestinal failure (CIF) requires long-term home parenteral nutrition (HPN) to support growth and health. Transitioning to adult care is a critical period, and long-term outcomes remain underexplored. This nationwide study assessed long-term survival, HPN dependence, psychosocial status and characteristics of CIF management in adult patients with pediatric-onset CIF who received HPN during childhood and transition to adulthood. METHODS: We conducted a multicenter retrospective study of patients who started HPN for CIF in childhood and transitioned to adult care still receiving HPN between 1995 and 2020. Clinical characteristics and risk factors for survival and HPN dependence were analysed. RESULTS: Ninety-seven patients (46% female) transitioned at a mean age of 19.2 ± 2.6 years, after HPN began at a median age of 1 year (IQR: 0-8). CIF was caused by short bowel syndrome (60%), motility disorders (27%), and mucosal disease (13%). Management included teduglutide (13%), autologous gastrointestinal reconstruction (6%) and intestinal transplantation (5%). After a median follow-up of 8 years, survival was 91% and HPN dependence 95%. Catheter-related bloodstream infections were the leading cause of death (62%). In multivariate analysis, daily HPN requirement (HR = 5.7, 95%CI: 1.2-26.0, p= 0.03) and intestinal transplantation (HR = 5.9, 95%CI: 1.5-23.0, p= 0.01) were associated with mortality. Teduglutide was the only factor associated with reduced HPN dependence (HR = 10.0, 95%CI: 1.1-88.9, p= 0.04). At last follow-up, 58% of patients were employed or studying, 51% had left the family home, 28% were in a relationship, 20% had psychiatric comorbidities and 14% opioid dependence. CONCLUSION: Pediatric-onset CIF patients show high survival into adulthood despite persistent HPN dependence. GLP-2 analogues may reduce HPN dependence and improve long-term outcomes.
Source: Clinical Nutrition
Institutions: Université Paris Cité, Hôpital Beaujon, Hospices Civils de Lyon, Hôpital Lyon Sud, Assistance Publique Hôpitaux de Marseille, Hôpital de la Timone, Centre Hospitalier Universitaire de Bordeaux, Université de Lille, Centre Hospitalier Universitaire de Lille, Université de Montpellier, Institut Regional du Cancer de Montpellier, Inserm, Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement, Centre Hospitalier Universitaire de Rennes, Nutrition, métabolismes et cancer, Université de Rennes, Université Côte d'Azur, Hôpital l'Archet, Centre Hospitalier Universitaire de Clermont-Ferrand, Hôpital Necker-Enfants Malades
Language: English
Subfield: Nutrition and Dietetics
Field: Nursing
Domain: Health Sciences
Open Access status: hybrid
Estimated APC paid: $3,320
OpenAlex ID: W7155644955