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Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis
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Implementation of Non-invasive Point of Care Transient Elastography for Evaluation of Liver Disease in Pediatric Populations with Cystic Fibrosis

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Intestinal failure-associated liver disease in hospitalised children

Judith Pichler1, Venetia Horn, Sarah Macdonald

  • 1Department of Paediatric and Adolescent Medicine, University Clinic, Vienna, Austria.

Archives of Disease in Childhood
|January 17, 2012
PubMed

Insights

Intestinal failure-associated liver disease (IFALD) affects 22% of children on long-term parenteral nutrition (PN), with surgical neonates at highest risk. IFALD incidence is lower than previously reported, with an 86% survival rate.

Area of Science:

  • Pediatric Gastroenterology
  • Hepatology
  • Clinical Nutrition

Background:

  • Liver disease is a serious complication of long-term parenteral nutrition (PN) in children.
  • Intestinal failure-associated liver disease (IFALD) is a specific form of liver injury.
  • Understanding IFALD's incidence, causes, and outcomes in pediatric populations is crucial.

Purpose of the Study:

  • To determine the incidence of IFALD in hospitalized children receiving long-term PN (>27 days).
  • To identify the etiological factors contributing to IFALD development.
  • To assess the prognosis and outcomes of IFALD in this patient group.

Main Methods:

  • A 4-year retrospective review of pediatric patients with intestinal failure (IF) requiring long-term PN.
  • Analysis of potential predisposing factors: age, diagnosis, PN lipid exposure, sepsis, and treatment duration.
  • Outcome measures included IFALD incidence, severity, and survival rates.

Main Results:

  • IFALD developed in 22% (60/279) of children, with 5% progressing to end-stage disease.
  • Risk factors included younger age, longer PN treatment and hospitalization, surgical diagnosis, and prematurity.
  • Sepsis was not associated with IFALD; intestinal surgery independently increased IFALD risk.

Conclusions:

  • The incidence of IFALD in pediatric patients is lower than previously reported.
  • Surgical neonates are identified as the highest-risk group for developing IFALD.
  • Overall survival was 86%, with IFALD-related deaths primarily occurring in surgical cases.
Abstract

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