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OC54 A retrospective cohort comparison study to assess the rate of intestinal failure associated liver disease in paediatric patients using home parental nutrition in a single tertiary centre over time: 1996-2010 Vs 2010-2025

flgastro · 2026-06-29 · canonical JSON source

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Intestinal failure associated liver disease (IFALD) has a multifactorial aetiology, which is becoming better understood. 1 2 We compared our current rates of IFALD with a previously reviewed cohort of patients born between 1996-2010.3Patients were included if using home parenteral nutrition (PN) for medical or surgical events before 3 months age. The definition of IFALD was matched to our previous review. We used the 2009 British society of paediatric gastroenterology, hepatology and nutrition: nutritional working group definition: type 1 = increased alkaline phosphatase >1.5 times upper limit for over 6 weeks, type 2 = plus total bilirubin > 50umol/l and a 50% conjugated fraction for 6 weeks, type 3 = plus clinical signs of end stage liver disease.4As shown in table 1, the rate of IFALD, was lower in the newer cohort with fewer patients developing more severe liver disease with an associated decrease in transplantation. There was an increase in prematurity, motility and medical comorbidities increased. There was a lower rate of enteral autonomy, which could be associated with the increase in motility and medical indications for home PN.Of the 5 patients weaned off PN in the newer cohort, 1 weaned off post-transplant, 3 had neonatal surgery, and 1 had anatomical abnormalities. The majority of those unable to achieve enteral autonomy have medical/motility problems likely needing home PN long term. The increased mortality was linked to comorbidities outside of intestinal failure.IFALD decreased despite the increase in time on PN. The older cohort predominantly used lipofundin lipid whereas the new group used SMOF which is more liver protective. Hepatoprotective measures such as early feeding, lipid free nights and an awareness of the causes of IFALD have had a liver guarding effect, enabling action when the condition may still be reversible.Further review of patient records to explore liver protective management plans and which interventions had the biggest impact would yield useful clinical information.References Cuerda C, Pironi L, Arends J, et al. ESPEN practical guideline: Clinical nutrition in chronic intestinal failure. Clin Nutr. 2021 Sep;40(9):5196–220.Lacaille F, Gupte G, Colomb V, et al. Intestinal failure-associated liver disease: a position paper of the ESPGHAN Working Group of Intestinal Failure and Intestinal Transplantation. J Pediatr Gastroenterol Nutr. 2015 Feb;60(2):272–83.Garrett H, Jones E, Minford J, et al. Successful management of infants with intestinal failure over two decades. Conference poster presentation, ESPGHAN, Athens, 2016.British Society for Paediatric Gastroenterology Hepatology and Nutrition (BSPGHAN) Nutrition Working Group. Review of current management practices in Intestinal Failure Associated Liver Disease; 2009.Abstract OC54 Table 1ResultsDate of birth1996-20102010-2025Numbers21 (67% male)25 (64% male)Gestation (weeks)RangeMeanMedian33-40+363624-403536Mortality3 died, 14%5 died, 20%IFALDTotalStage 1Stage 2Stage 372 (9.5%)1 (4.8%)4 (19%)52 (8%)1 (4%)2 (8%)Weaned off PN13 (54%)5 (20%)Time to wean off PN (months)RangeMeanMedian8-11836285-604148Total duration of PN use for all patients (months)RangeMeanMedian8-119464418-1877760Residual small bowel length (cm)<2525-65>6568756131 – information unavailableLiver transplants:10Small bowel transplants:10Liver & small bowel transplant combined11Aetiology Necrotising enterocolitis Gastroschisis Atresia Volvulus/malrotations Other anatomical Motility Medical365103*3#432326**5##(*1x Hirschprung’s; **5x Hirschprung’s; #1x microvillous inclusion disease, 1x tufting enteropathy, 1x congenital enteropathy; ##3x feed intolerance of undiagnosed aetiology, 1x ischaemic event, 1x TTC7A deficiency)