Outcome of home parenteral nutrition in 251 children over a 14-y period: report of a single center

Abstract

SBS was the major indication for HPN in our cohort. IFALD and CRBSIs were potentially life-threatening problems. Nevertheless, complication rates were low, and deaths resulted mostly from the underlying disease.

Pediatric Intestinal Failure: The Key Outcomes for the First 100 Patients Treated in a National Tertiary Referral Center During 1984–2017

“…The presence of liver fibrosis was frequent (56%), but only of mild‐to‐moderate degree (METAVIR stage 1 or 2). These results are in line with a recent study from Paris . We have used a PN lipid restriction policy, using lipids with reduced soy oil source combined with fish oil for prevention of IFALD .…”

“…Our overall survival and ITx rates compare well with recent reports from large European and North American centers ranging between 85%–95% and 5%–8%, respectively . Regarding SBS, our 74% weaning‐off PN rate is somewhat higher than the 55%–64% in those studies.…”

“…Overall, CRBSI rates had a significantly declining incidence toward recent years. By 2013 and thereafter, every patient has received taurolidine CVC locks reported to prevent CRBSIs also in other pediatric IF centers, a result confirmed recently in a randomized placebo‐controlled trial in adult patients with IF . We have used commercially manufactured 3‐in‐1 PN bags since 2011, likely also contributing to our currently low CRBSI rates .…”

“…Pediatric intestinal failure (IF) causes significant morbidity and mortality, affecting neonates and young children during their vulnerable period of somatic, neurological, and psychological development. Recent advances in IF care have shown encouraging results with IF‐associated liver disease (IFALD) through parenterally administered lipid modifications, as to accelerated rate of weaning off parenteral nutrition (PN) using pharmacological agents and increased transplant‐free survival in dedicated intestinal rehabilitation centers . Unfortunately, many of these positive trends may only pertain to the major cause of IF, short bowel syndrome (SBS).…”

“…Traditionally, ITx has been mostly performed because of end‐stage IFALD, loss of vascular access sites, and recurrent sepsis or dehydration episodes . With decreasing incidence of progressive IFALD, long‐term maintenance of vascular access is indeed becoming the major challenge in long‐term IF care . Unfortunately, even in the modern era, post‐ITx survival is around 50% at 10 years, highlighting the importance of postponing the need for ITx by expert nutrition care and prevention of CVC and liver complications in patients with pediatric IF …”