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Outcomes of Children with Chronic Intestinal Failure

Experience Over Two Decades at a Tertiary Paediatric Hospital

Jo, Sz-Ying Christine*; McCallum, Zoe*,†,‡; Shalley, Helen; Peterkin, Megan; Rogers, Elizabeth; Paxton, Georgia*,†; Wong, Theodoric†,‡; Bines, Julie E.*,†,‡

Journal of Pediatric Gastroenterology and Nutrition: May 6, 2019 - Volume Publish Ahead of Print - Issue - p
doi: 10.1097/MPG.0000000000002384
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Background & Aims: To aid decisions on prognosis and transplantation, this study describes the outcome of children with intestinal failure managed by the multidisciplinary intestinal rehabilitation program at the Royal Children's Hospital, Melbourne.

Methods: Retrospective review of children requiring parenteral nutrition (PN) for >3 months who were assessed for home PN between 1991 and 2011.

Results: 51 children were included. 42 (82%) had short bowel syndrome (SBS), 5 (10%) had chronic intestinal pseudo-obstruction syndrome (CIPOS) and 4 (8%) had congenital enteropathies. Median small bowel length for SBS patients was 45 cm (IQR 30-80) or 23.9% of the expected length for age (IQR 17.0%-40.6%). Overall survival rate was 84% (43/51). Mortality in children (n = 7) occurred after a median of 13.2 months (range 6.2-29.2) with intestinal-failure associated liver disease (IFALD) being the only predictor (p = 0.001). 21/50 (42%) children had IFALD. Children who were premature (p = 0.013), had SBS (p = 0.038) and/or frequent sepsis (p = 0.014) were more likely to develop IFALD. PN weaning occurred in 27/35 (77%) SBS survivors, after a median of 10.8 months (up to 8.2 years), with longer residual small bowel (p = 0.025), preservation of the ileocaecal valve (p = 0.013) and colon (p = 0.011) being predictors. 0/5 (0%) patients with CIPOS and 2/4 (50%) patients with congenital enteropathies weaned off PN. Overall sepsis rate was 7.3 episodes/1000 line days. Frequency of sepsis and longevity of central lines improved with time as patients grew older (both p < 0.001).

Conclusions: Long-term PN with intestinal rehabilitation was effective in treating most children with intestinal failure. Children with severe refractory IFALD may have benefited from intestinal transplantation.

*Department of Paediatrics, The University of Melbourne

Department of Gastroenterology and Clinical Nutrition, Royal Children's Hospital

Intestinal Failure and Clinical Nutrition Research Group, Murdoch Childrens Research Institute, Parkville, Victoria, Australia, 50 Flemington Road, Parkville, Victoria, 3052, Australia.

Address correspondence and reprint requests to Julie E. Bines, Department of Paediatrics, The University of Melbourne, 50 Flemington Road, Parkville, Victoria, 3052, Australia. (e-mail: Julie.bines@rch.org.au)

Received 28 April, 2018

Accepted 7 April, 2019

Conflict of Interest Statement: The authors have no conflicts of interest to report

Funding Sources: This study was funded by internal Department resources.

Theodoric Wong Department of Gastroenterology and Nutrition, Birmingham Children's Hospital, United Kingdom

Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org).

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,