ABSTRACT
Children with short bowel syndrome (SBS) are at high risk for intestinal failure associated liver disease (IFALD). The aim of this retrospective follow-up study was to compare weaning off parenteral nutrition (PN) and IFALD between necrotizing enterocolitis (NEC) and non-NEC induced SBS. Altogether, 77 patients with neonatal SBS treated by our multidisciplinary intestinal failure unit (NEC n = 38, non-NEC SBS n = 39) were included and followed-up at least for 2 years until median age of 10 years (interquartile range, 6.0-16). Occurrence and characteristics of IFALD was assessed with liver biopsies obtained at median age of 3.2 (1.0-6.7) years (n = 62) and serum liver biochemistry. Overall, NEC patients had less end-jejunostomies and autologous intestinal reconstruction surgeries performed compared to non-NEC patients (< 0.05), while remaining small bowel anatomy was comparable between groups. Cumulative weaning off PN was more frequent and duration of PN shorter among NEC patients (P < 0.05). Overall cumulative probability of histological IFALD was lower among NEC patients during whole follow-up period (P = 0.052) and at 10 years (P = 0.024). NEC patients had lower ALT and GGT levels at last follow-up (P < 0.05 for all). In univariate Cox regression analysis, absence of end-jejunostomy, NEC diagnosis, longer remaining small bowel length, multidisciplinary treatment and prematurity were predictive for weaning off PN, while NEC diagnosis and lower birth weight in addition to multidisciplinary care protected from histological IFALD. Neonates with NEC induced SBS reached enteral autonomy earlier than those with non-NEC SBS, which associated with more efficient resolution of histological IFALD among long-term survivors.
