Chronic intestinal failure and home parenteral nutrition in children : outcomes and perspective
Florence Lacaille1, Laetitia-Marie Petit1, Cécile Lambe1, Delphine Girard2, Caroline Elie2, Olivier Goulet1, Virginie Colomb1
1Pediatric Hepatogastroenterology-Nutrition, Hôpital Necker-Enfants malades, Paris, France; 2Biostatistics, Hôpital Necker-Enfants malades, Paris, France
Aim. To analyze a group of children discharged on Home Parenteral Nutrition (HPN) for chronic intestinal failure (IF) over the 2000-2010 decade, and to compare their outcomes and prognostic factors for weaning off PN.
Methods. Retrospective study of 151 patients on HPN between 01.01.00 and 31.12.09 for primary digestive disease, and comparison with the reference group 1980-2000 [1]. Statistical analysis was performed with Cox model and Kaplan Meier curves.
Results. 151 patients received HPN, 2/3 of them for a short bowel syndrome (SBS, including 6 long-segment Hirschsprung disease), motility disorders (MD) (11%), congenital mucosal disorders (CMD) (9%), inflammatory bowel diseases (9%), others (5%). Probability of weaning off PN was 73% for the whole group, and also for the SBS group. The survival, close to 100%, was the most dramatical improvement compared to the 1980-2000 period. No death was due to HPN- associated liver disease. In the SBS group, 9% underwent transplantation, intestinal +/- combined with liver. The residual bowel length (over 40cm), the presence of ileo-caecal valve (ICV), presence of more than 50% of the colon, and the daily caloric PN intakes (over 70% of the needs) were prognostic factors in univariate analysis, whereas in the multivariate, only the bowel length was significant (p<0.001). The median duration until weaning off PN was 21 months. MD and CMD appear as poor prognostic factors for weaning off in both statistical analysis (p= 0.048 and p= 0.002).
Conclusion. HPN is a safe and efficient treatment of IF in children, also on the very long-term. Some subgroups had a higher risk for definitive IF : SBS < 40 cm, MD and CMD. These patients should benefit from a multidisciplinary evaluation in a reference care center.