Retrospective review of children requiring parenteral nutrition (PN) for >3 months who were assessed for home PN between 1991 and 2011″ Jo et al (2019).
Abstract:
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.
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Reference:
Jo, S.C., McCallum, Z., Shalley, H., Peterkin, M., Rogers, E., Paxton, G., Wong, T. and Bines, J.E. (2019) Outcomes of Children with Chronic Intestinal Failure: Experience Over Two Decades at a Tertiary Paediatric Hospital. Journal of Pediatric Gastroenterology and Nutrition. May 6th. doi: 10.1097/MPG.0000000000002384. [Epub ahead of print].