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Objective and Aim: Liver disease is a potentially life-threatening complication of intravenous/parenteral nutrition (PN). Our aim was to determine the incidence, aetiology and outcome of intestinal failure-associated liver disease (IFALD) in hospitalised children treated with long-term PN (>27 days)., Methods: Over 4 years all long-term intestinal failure (IF) patients were reviewed for the possible predisposing factors of age, diagnosis, PN lipid, sepsis, length of PN treatment and length of hospitalisation. Outcome measures were IFALD incidence, severity and prognosis., Results: Of 60/279 (22%) children aged 0-18 years who developed IFALD, 13 (5%) progressed to type 3/end stage disease. IFALD was associated with younger age (p=0.03), longer treatment (p<0.001), longer hospitalisation (p=0.01), surgical diagnosis (p=0.005) and prematurity (p=0.03). IFALD was not associated with sepsis. Intestinal surgery was associated with IFALD independently of age (p=0.03). Survival was 86%, with three deaths attributed to IFALD (1% of all cases), all of which were surgical., Conclusion: IFALD incidence was lower than previously reported in paediatric patients, with surgical neonates at greatest risk.

Abstract: Purpose: Our aim was to determine incidence, severity, and outcome, as well as predisposing factors and underlying diagnoses, of intestinal failure-associated liver disease (IFALD) in surgical infants requiring long-term parenteral nutrition (PN). Methods: We retrospectively studied surgical infants receiving PN for at least 28 days for congenital or acquired intestinal anomalies over a 5-year period (January 2006 to December 2010). Intestinal failure-associated liver disease was defined as type 1 (early)—persistent elevation of alkaline phosphatase for 6 weeks or longer; type 2 (established)—additional elevated total bilirubin (≥50 μmol/L); and type 3 (late)—additional clinical signs of end-stage liver disease. Results: Eighty-seven infants required PN for at least 28 days. Intestinal failure-associated liver disease occurred in 29 infants (33%). Intestinal failure-associated liver disease was managed medically in all but 2 patients who underwent intestinal elongation. None were referred for intestinal or liver transplant. Intestinal failure-associated liver disease has been reversed in 17 (59%) of cases to date. Sixty-one children receiving long-term PN (70%) have achieved enteral autonomy, whereas 12 (14%) require home PN. Severity of IFALD was significantly associated with duration of PN and female sex. Conclusion: Intestinal failure-associated liver disease remains a fairly common but rarely life-threatening complication of intestinal failure in surgical infants. Intestinal failure-associated liver disease can be reversed in more than half of these children, and enteral autonomy was achieved in more than two thirds, even with minimal use of intestinal elongation. This is the first study to demonstrate an association between the severity of IFALD in surgical infants and female sex. [Copyright &y& Elsevier]