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From childhood to adulthood in chronic intestinal failure: A nationwide study
Louis Schubert1, Cécile Chambrier2, Lore Billiauws1
1Gastroentérologie et Assistance nutritive, Centre labellisé de Maladies Rares Digestives, Hôpital Beaujon, Assistance Publique des Hôpitaux de Paris, Université Paris Cité, Clichy, F-92110, France.
Insights
Adults with pediatric-onset chronic intestinal failure (CIF) have high survival rates with home parenteral nutrition (HPN). GLP-2 analogues show promise in reducing HPN dependence and improving long-term outcomes for these patients.
Area of Science:
- Gastroenterology
- Pediatric Surgery
- Clinical Nutrition
Background:
- Pediatric chronic intestinal failure (CIF) necessitates long-term home parenteral nutrition (HPN) for growth and health.
- Transitioning from pediatric to adult care is a critical phase for CIF patients, with limited data on long-term outcomes.
- This study evaluates long-term survival, HPN dependence, and psychosocial status in adult patients with pediatric-onset CIF.
Purpose of the Study:
- To assess long-term survival and HPN dependence in adult patients with pediatric-onset CIF.
- To identify risk factors associated with mortality and HPN dependence.
- To evaluate the psychosocial status and management characteristics of these patients in adulthood.
Main Methods:
- A multicenter retrospective study was conducted on patients who initiated HPN for CIF in childhood and continued HPN into adulthood.
- Data were collected between 1995 and 2020, analyzing clinical characteristics and risk factors.
- Statistical analysis included multivariate analysis to determine predictors of survival and HPN dependence.
Main Results:
- Ninety-seven patients transitioned to adult care, with a median follow-up of 8 years. Overall survival was 91%, and HPN dependence was 95%.
- Catheter-related bloodstream infections were the primary cause of death (62%). Daily HPN requirement and intestinal transplantation were associated with increased mortality.
- Teduglutide was linked to reduced HPN dependence. Psychosocial outcomes included employment/study in 58%, relationship in 28%, and psychiatric comorbidities in 20%.
Conclusions:
- Adults with pediatric-onset CIF demonstrate high long-term survival despite ongoing HPN dependence.
- GLP-2 analogues, such as teduglutide, may play a role in reducing HPN dependence and enhancing long-term outcomes.
- Further research into psychosocial support and optimized management strategies is warranted for this growing patient population.
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.
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