260.6: PEDIATRIC HOME PARENTERAL NUTRITION (HPN) IN FRANCE: a national survey On the behalf of the French Pediatric HPN network

Abstract

Chronic intestinal failure (CIF) requires long-term parenteral nutrition (PN). In France, 7 centers are certified as Pediatric Home-PN (PHPN) and supported by the French Social Security. The aim was to review the activity of the 7 PHPN. Population and methods: Survey: Jan 1, 2014- Dec 31, 2017: Patients characteristics, HPN indications & duration, turn- over, PN weaning, complications: catheter-related blood stream infections (CRBSIs), cholestatic liver disease (CLD) (total bili > 30 micromol/l). Results: 624 patients < 18 years of age (56.9 % boys) attended one of the 7 PHPN program during the period. Turn-over: 18.7% entering and 13.2% leaving. Increasing population from 265 in 2014 to 352 in 2017 (+33%). Mean & median age at inclusion were 33 & 9 months. Primary digestive disease (PDD) involved 95% of the children. Indications for HPN were short bowel syndrome (SBS): 40.8 ± 7.3 % - congenital enteropathies: 21.9 ±8.5 % - chronic intestinal pseudo-obstruction:15.7 ± 8.9 % – Total aganglionosis: 8.3 ±6.8%; IBD: 4.3 ± 4.2%. Broviac type central venous catheter used in 98%. All patients received tailored PN bags made by hospital pharmacy (16%) or by Baxter-Faconnable® (84%). Intravenous lipid emulsions (ILE) were SMOFlipid® (84%), Clinoleic® (8%), MCT/LCT (6%) or Intralipid® (2%). Mean and median duration of HPN were 82 and 54 months. Causes of resuming HPN were: weaning off PN (79%), transition to adult (15%), death (5%) (90 % of death are cancer or immune deficiency) and intestinal Tx (1%). The main complication was CRBSIs caused by Staphylococcus coagulase negative (70 ± 18%) and Staphylococcus aureus (12.0 ± 11.5 %) (5 fungal infection). Taurolidine lock therapy (TLT) made CRBSIs incidence decreasing from 1.02 per 1000 PN days in 2014 to 0.61 in 2017. Fifteen patients (2.6%) had total bilirubin >30 micromol/l, including 5 cirrhosis, listed for Tx. Conclusions: HPN is a safe and efficient therapy. SBS is the main HPN indication, with the highest rate of PN weaning. CRBSIs and CLD are potentially life-threatening complications, but their rates were low and deaths (4%) were mostly due to the underlying disease. CRBSIs incidence per 1000 days PN decreased dramatically (- 40%) with TLT. Need for intestinal Tx is very limited according to the low rate of life-threatening complications. Patients must be referred early to expert centers for optimal management and follow-up. Data will be updated for the Congress including results of the year 2018