Background: Long-term parenteral nutrition (PN) is the mainstay of the therapeutic strategy in intestinal failure (IF) due to neonatal short bowel syndrome (SBS). Our aim was to identify the prognostic factors of PN weaning and to assess if measuring plasma citrulline concentrations over time could help to assess the intestinal adaptation process. Methods: This retrospective study included children with neonatal SBS with surgical measurement of the residual bowel length and repeated plasma citrulline assessments during a 4 years follow-up. The degree of IF was assessed by the PN dependency index (PNDI). The analysis was carried out according to SBS anatomic groups: end-jejunostomy (type 1), jejuno-colic (type 2) and jejuno-ileal anastomosis (type 3). Results: Fifty-five patients (8 with type 1, 27 type 2, 20 type 3 SBS) were included. None of the patients with SBS type 1, 11 (41%) with type 2 and 11 (55%) with type 3 were weaned off PN during the study period. Citrulline increased with time in patients weaned off, but not in patients remaining on PN, as shown by a negative relationship between citrulline and PNDI. The increasing citrulline levels was positively correlated to the probability of PN weaning: 2.7 times higher for each point increase in citrulline. No significant effect of age at baseline and residual bowel length was found. Conclusion:The increasing plasma citrulline level over time, according to the SBS type, is a reliable marker of the probability of PN weaning. The prediction of PN weaning assessed from the residual bowel length is not sufficient and should be completed with longitudinal measurements of plasma citrulline levels and analyzed in relation to the anatomical type of SBS.
Introduction: Acute on Chronic Liver Failure (ACLF) is defined as an acute deterioration of pre-existing chronic liver disease related to a precipitating event with increased mortality at 3 months due to multisystem organ failure. Children with Intestinal Failure Associated Liver Disease (IFALD) are at particular risk of ACLF as they often face a long waiting time for appropriate sized liver small bowel transplant donors and are not able to be categorised as super urgent. We aimed to characterise ACLF in children with IFALD. Methods: All BCH patients from 2000-2020 with IFALD determined to be in ACLF utilising European Foundation for Study of Chronic Liver Failure (EASL CLIF) criteria were included. Descriptive statistics was presented as median (range) and CLIF C ACLF score calculated to predict 28-day mortality. Results: 6 children with IFALD were included. 3 patients had Gastroschisis, 2 Necrotising Enterocolitis (NEC) and 1 total colonic Hirschsprung's. Median age at ACLF onset was 1 year (0.8-2). ACLF triggers were sepsis in 5 and GI bleed in 1. Bilirubin at time of ACLF onset was 600 umol/L (506-870), Creatinine 30 umol/L (14-124), Prothrombin time 22 (15-35), Grade 3,4 hepatic encephalopathy (2/6), ventilation (2/6) and CLIF C ACLF score 31 (28-49). There was 100% mortality (n=6) between 2000-2012. Median time from ACLF admission to death was 29 days (6-60). Transplant listing occurred in 4, 3 pre ACLF with a median of 17 days (10-120) and 1 7 days post ACLF. 2 children were thought to be too unwell to list. Discussion: Children with IFALD are vulnerable to develop ACLF, particularly from triggering factors e.g. line sepsis. Children presenting with ACLF had end stage IFALD and median time to death was short, with not enough time for rescue transplantation. The decrease in ACLF episodes in last decade may be related to less incidence of IFALD due to advances in parenteral nutrition. Conclusion:Children with end stage IFALD can be at risk of developing ACLF especially following line sepsis and thus further prioritisation in those with IFALD at risk of ACLF should be considered.
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