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Assessment and outcome of children with intestinal failure referred for intestinal transplantation

Summary Background & aims Chronic intestinal failure (CIF) requires long term parenteral nutrition (PN) and, in some patients, intestinal transplantation (ITx). Indications and timing for ITx remain poorly defined. In the present study we aimed to analyze causes and outcome of children with CIF....

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Published in:Clinical nutrition (Edinburgh, Scotland) Scotland), 2015-06, Vol.34 (3), p.428-435
Main Authors: Ganousse-Mazeron, S, Lacaille, F, Colomb-Jung, V, Talbotec, C, Ruemmele, F, Sauvat, F, Chardot, C, Canioni, D, Jan, D, Revillon, Y, Goulet, O
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Language:English
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Summary:Summary Background & aims Chronic intestinal failure (CIF) requires long term parenteral nutrition (PN) and, in some patients, intestinal transplantation (ITx). Indications and timing for ITx remain poorly defined. In the present study we aimed to analyze causes and outcome of children with CIF. Methods 118 consecutive patients referred to our institution were assessed by a multidisciplinary team and four different categories were defined retrospectively based on their clinical course: Group 1: patients with reversible intestinal failure; group 2: patients unsuitable for ITx, group 3: patients listed for ITx; group 4: patients stable under PN. Analysis involved comparison between groups for nutritional status, central venous catheter (CVC) related complications, liver disease, and outcome after transplantation by using non parametric tests, Mann–Whitney tests, Kruskal–Wallis, Wilcoxon signed rank tests and chi square distribution for percentage. Results 118 children (72 boys) with a median age of 15 months at referral (2 months–16 years) were assessed. Etiology of IF was short bowel syndrome [ n  = 47], intractable diarrhea of infancy [ n  = 37], total intestinal aganglionosis [ n  = 18], and chronic intestinal pseudoobstruction [ n  = 17]. Most patients (89.8%) were totally PN dependent, with 48 children (40.7%) on home-PN prior to admission. Nutritional status was poor with a median body weight at −1.5 z-score (ranges: −5 to +2.5) and median length at −2.0 z-score (ranges: −5.5 to +2.3). The mean number of CVC inserted per patient was 5.2 (range 1–20) and the mean number of CRS per patient was 5.5 (median: 5; range 0–12) Fifty-five patients (46.6%) had thrombosis of ≥2 main venous axis. At admission 34.7% of patients had elevated bilirubin (≥50 μmol/l), and 19.5% had platelets
ISSN:0261-5614
1532-1983
DOI:10.1016/j.clnu.2014.04.015