Intestinal failure-associated liver disease in hospitalised children

Pichler, Judith; Horn, Venetia; Macdonald, Sarah; Hill, Susan

doi:10.1136/archdischild-2011-300274

Intestinal failure-associated liver disease in hospitalised children

¹Department of Paediatric and Adolescent Medicine, University Clinic, Vienna, Austria
²Department of Pharmacy, Great Ormond Street Hospital for Children NHS Trust, London, UK
³Department of Dietetics, Great Ormond Street Hospital for Children NHS Trust, London, UK
⁴Department of Paediatric Gastroenterology, Great Ormond Street Hospital for Children NHS Trust, London, UK

Correspondence to Dr Susan Hill, Department of Paediatric Gastroenterology, Great Ormond Street Hospital for Children NHS Trust, London WC1N 3JH, UK; susan.hill{at}gosh.nhs.uk

Contributors The study was designed by SH. The subjects were recruited by JP, VH and SH. Formulation of the parenteral nutrition was performed by VH. SM introduced enteral feeding. Data were collected and analysed by JP, VH and SH. The manuscript was written by JP with significant advice from and consultation with VH, SM and SH. All authors critically appraised the manuscript.

Received 2 May 2011
Accepted 27 November 2011
Published Online First 13 January 2012

Abstract

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.