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Presenter: Olivier, Goulet, Paris, France
Authors: Olivier Goulet, Irène Lauras, Emmanuelle Dugelay, Marie-Edith Coste, Béatrice Dubern, Anne Breton, Dominique Guimber
Olivier Goulet1, Irène Lauras3, Emmanuelle Dugelay7, Marie-Edith Coste6, Béatrice Dubern5, Anne Breton4, Dominique Guimber2.
1Pediatric Gastroenterology and Nutrition, University Hospital Necker Enfants Malades, Paris, France; 2Ped GI, Jeanne de Flandre, Lille, France; 3Ped GI, CHU Lyon, Lyon, France; 4Ped GI, CHU-Purpan, Toulouse, France; 5Ped GI&N, Hop Trousseau, Paris, France; 6Ped GI, La Timone, Marseille, France; 7Ped GI, Hop R.Debré, Paris, France
Chronic intestinal failure requires long-term PNand home-PN. In France, only 7 centers are certified as Pediatric Home-PN Expert Center (PHPN-EC) and supported by the Social Security. The aim was to review the HPN activity from a national data base.
Population and methods: from Jan 1 to Dec 31, 2015, data include: patients characteristics, indications and duration of HPN, patients turn- over, incidence and cause of PN weaning, complications: catheter-related blood stream infections (CRBSIs), cholestatic liver disease (CLD) (bilirubin > 30 micromol/l.
Results: 307 patients <18 yrs of age (56.9% boys) followed-up in a HPN program (18 patients per million <18 yrs) with a 14.5% increased prevalence from 2014. New patients (n=60) enrolled in 2015 account for 19.5% of the cohort. Mean number of patients per center was 38 ± 33 (16-136). Age was 80.6 ± 25.8 months (4 mths-18 yrs) and mean HPN duration was 51.2±42.3 mths. Primary digestive disease (PDD) involved 291 children (95%). Indications were SBS: 40.8 ± 7.3% (31-50) - congenital enteropathies: 21.9 ±8.5% (5.5-31) - intestinal pseudo-obstruction:15.7 ± 8.9% (4-31) – Total aganglionosis: 8.3 ±6.8% (0-24) and IBD : 4.3 ± 4.2% (0-12.5). Vascular access was a Broviac catheter in 98%. All received tailored PN bags made by hospital pharmacy (16%) or Fasonut-Baxter® (84%). Intravenous lipid emulsions were SMOFlipid® in 84.3 ± 42.8% (0-100) or MCT/LCT. On Dec 31, 2015, 42 patients had left the HPN program after 3 mths to 18 yrs (med 6.5 yrs): weaning (55%) [SBS representing 90% of weaned patients] adult transfert: 35 % or death: 19% (2.5% of the cohort) (90% of death are non PDD related; eg: cancer or immune deficiency).The major complication was CRBSIs, 0.90 ± 0.22 per 1000 days of PN (0.30-1.90). Staphylococcus coagulase negative 70 ± 18% (range (50-100) and Staphylococcus aureus 12.0 ± 11.5 % (15-25) (only 1 fungal infection). 129 patients received taurolidine locks at January 1, 2015 and 107 new in 2015. Eight patients (2.6%) had total bilirubin >30 mmolmol/l, including 2 cirrhosis, listed for liver-intestine transplantation.
Conclusions: In this setting, 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. Nevertheless, their rates were low and deaths (2.5%) were mostly due to the underlying disease. Patients must be referred early to expert centers (PHPN-EC) for optimal management and follow-up.
Evelyne Marinier. Cécile Lambe. Cécile Talbotec. Noel Peretti.
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