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Intestinal failure-associated liver disease in surgical infants requiring long-term parenteral nutrition
Mark Bishay1, Judith Pichler, Venetia Horn
1University College London, Institute of Child Health and Great Ormond Street Hospital for Children, London WC1N 1EH, UK.
Insights
Intestinal failure-associated liver disease (IFALD) affects one-third of surgical infants on long-term parenteral nutrition (PN). IFALD is often reversible, with most infants achieving enteral autonomy.
Area of Science:
- Pediatric Surgery
- Hepatology
- Gastroenterology
Background:
- Long-term parenteral nutrition (PN) is essential for surgical infants with intestinal anomalies.
- Intestinal failure-associated liver disease (IFALD) is a significant complication in this population.
- Understanding IFALD's incidence, severity, and outcomes is crucial for management.
Purpose of the Study:
- To determine the incidence, severity, and outcomes of IFALD in surgical infants.
- To identify predisposing factors and underlying diagnoses associated with IFALD.
- To evaluate the impact of PN duration and sex on IFALD severity.
Main Methods:
- Retrospective study of surgical infants receiving PN for ≥28 days.
- Classification of IFALD into types 1, 2, and 3 based on liver enzyme and bilirubin levels.
- Analysis of patient data over a 5-year period (2006-2010).
Main Results:
- IFALD occurred in 33% of 87 infants requiring long-term PN.
- IFALD was reversible in 59% of cases; 70% achieved enteral autonomy.
- IFALD severity correlated with PN duration and female sex.
Conclusions:
- IFALD is a common, but rarely fatal, complication in surgical infants.
- Most infants with IFALD can achieve enteral autonomy, with a high reversal rate.
- Female sex is associated with increased IFALD severity in infants.
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.
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