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Intestinal failure-associated liver disease in hospitalised children
  1. Judith Pichler1,4,
  2. Venetia Horn2,
  3. Sarah Macdonald3,
  4. Susan Hill4
  1. 1Department of Paediatric and Adolescent Medicine, University Clinic, Vienna, Austria
  2. 2Department of Pharmacy, Great Ormond Street Hospital for Children NHS Trust, London, UK
  3. 3Department of Dietetics, Great Ormond Street Hospital for Children NHS Trust, London, UK
  4. 4Department of Paediatric Gastroenterology, Great Ormond Street Hospital for Children NHS Trust, London, UK
  1. 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

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.

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Footnotes

  • Competing interests None.

  • Ethics approval The Local Research Ethics Committee approved this study.

  • Provenance and peer review Not commissioned; externally peer reviewed.

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