Laetitia-Marie Petit, Cécile Talbotec, Solène Ganousse-Mazeron, Catherine Poisson, Odile Corriol, Sixtine Jarde, Olivier Goulet, Virginie Colomb

Hôpital Necker-University Paris Descartes, Paris, France

Intestinal Failure (IF) requires Parenteral Nutrition (PN) and, when IF is protracted, home-PN. Analysis of patient’s outcome over several years should help to decide properly intestinal  transplantation (ITx) with regards to capacity off weaning off PN and  incidence of associated liver disease (IFALD).

Methods: Retrospective study of the medical data of patients discharged on Home-PN (HPN) between 01.01.2000 and 31.12.2009, including outcome, pathology, infections, liver disease.

Results: A total of 250 patients were discharged on HPN during the period of study. Among them, 217 patients (age 3 months to 15 years) received HPN for primary digestive disease.  Short bowel syndrome (SBS) is the most frequent condition (56%). PN weaning may be achieved according to the length of remnant bowel (p < 0.01). Three other underlying digestive diseases require home-PN: motility disorders (10%) including total intestinal aganglionosis and intestinal pseudo-obstruction syndrome, congenital enteropathy (8%) including microvillous atrophy and tufting enteropathy, inflammatory bowel diseases (8%). The other patients (18%) had lymphatic disease, metabolic disorders, immune deficiency or GvHD.  From the whole cohort, 51% of the patients were weaned off PN during the period of inclusion.  The mean and median duration of HPN increased for the 4 groups, but mostly for congenital enteropathy and motility disorders. The incidence of catheter related sepsis increased mildly (+10%) during the study period. Liver disease ( 43%) was more frequent  in patients with congenital enteropathy and very short bowel syndromes. These patients are those with the longest duration of home-PN. The sensitive criteria chosen for the study can explain the high rate of hepatopathy (43%). The mortality of the patients strongly decreased, with 2.5% death rate on the whole cohort. Fourteen patients received intestinal or liver/intestinal transplantation because of onset of liver disease or end stage liver disease. Among them only 5 (36%) have currently a functional graft with complete PN weaning.

Conclusion: Liver disease associated with long-term HPN requires ITx, so that PN related complications were the most relevant indication for transplantation but also un negative predictor factor for outcome. Comparison of mortality on HPN and rate of success of ITx should lead to a multidisciplinary evaluation in a reference center care, of the child’s clinical, family, and psychosocial situation.