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Intestinal failure-associated liver disease in surgical infants requiring long-term parenteral nutrition.
MedLine Citation:
PMID:  22325390     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
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.
Authors:
Mark Bishay; Judith Pichler; Venetia Horn; Sarah Macdonald; Marlene Ellmer; Simon Eaton; Susan Hill; Agostino Pierro
Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  47     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2012 Feb 
Date Detail:
Created Date:  2012-02-13     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  359-62     Citation Subset:  IM    
Copyright Information:
Copyright © 2012 Elsevier Inc. All rights reserved.
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