Objectives and Study: Intestinal Failure Associated Liver Disease (IFALD) remains common in children with intestinal failure (IF). Aim of this study was to determine incidence and risk factors of IFALD and to understand if the current definition of IFALD is sufficient.

Methods: Patients on home PN for ˃ 12 months were recruited from a tertiary IF clinic. IFALD was defined according to the 2015 ESPGHAN paper, data analysed retrospectively.
1.Early IFALD (‘red flag’): bilirubin>20 μmol/L
2.Established IFALD: bilirubin>100 μmol/L ˃ 2 to 4 weeks
3.Advanced IFALD: 2 + portal hypertension
Underlying IF aetiology, PN duration, incidence of catheter related blood stream infections (CRBSIs), bilirubin, ALT, ALP, GGT, platelets, liver ultrasound and biopsy results were obtained from the case notes.

Results: 52 children were identified;19/52 had short bowel syndrome (SBS), 17/52 motility disorder and 16/52 enteropathy/epithelial disorder. Median length of PN was 4 years (range 1-18 years.)

IFALD was more common in females (11/29 ; 37% versus 4/23;17%; p = 0.22) and in patients with small intestinal bacterial overgrowth (SIBO) (3/8;37% versus 12/44 ;27%; p= 0.67). 2/9 (22%) patients on hepatotoxic medications developed IFALD Vs 13/43 (30%) (P = 0.0045). Highest incidence of IFALD occurred in SBS 9/18 (50%). 29/52 (56%) had persistently normal LFTs and bilirubin.
6/52 (11.5%) were considered ‘red flag’ of which 5 had biliary stasis confirmed on ultrasound, 4 had SBS; 1 was treated for SIBO during this period. 2/52 children (3.8 %) had established IFALD (bilirubin>100 μmolL for >4 weeks). No biopsy was performed as LFTs improved; 1/52 patient (1.9 %) had a bilirubin>100μmol/L for 2 weeks following 2 episodes of CRBSI.1/52 (1.9%) patient with SBS had advanced IFALD portal hypertension but no CRBSIs during the study period. 2/52 (3.8%) patients had a liver biopsy as red flag but bilirubin < 100μmol/L and abnormal ultrasound which confirmed established IFALD. Both had SBS and no episodes of CRBSI during the study period. 5/52 (9.6%) children had a normal bilirubin and elevated ALP >6 weeks; 2 demonstrated biliary stasis on ultrasound. 6/52 (3.1%) had a rise in ALP < 6 weeks, normal bilirubin and ultrasound.

Conclusion: In our cohort IFALD was rare. Risk factors for developing IFALD were CRBSI and SBS, although not statistically significant likely due to small sample size. A definition based largely on bilirubin may not be sufficient to detect IFALD.