Intestinal failure-associated liver disease in surgical infants requiring long-term parenteral nutrition.
OBJECTIVE
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.


CONCLUSIONS
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.
University College London, Institute of Child Health and Great Ormond Street Hospital for Children, London WC1N 1EH, UK.
Mark
M
Bishay
Judith
J
Pichler
Venetia
V
Horn
Sarah
S
Macdonald
Marlene
M
Ellmer
Simon
S
Eaton
Susan
S
Hill
Agostino
A
Pierro
D034861:Abdominal Wall / Q000002:abnormalities* / Q000601:surgery; D000469:Alkaline Phosphatase / Q000097:blood; D002779:Cholestasis / Q000097:blood / Q000209:etiology*; D004655:Emulsions / Q000009:adverse effects; D020345:Enterocolitis, Necrotizing / Q000601:surgery*; D005260:Female; D005526:Food, Formulated; D006801:Humans; D006932:Hyperbilirubinemia / Q000209:etiology; D007223:Infant; D007231:Infant, Newborn; D007234:Infant, Premature; D007235:Infant, Premature, Diseases / Q000209:etiology / Q000601:surgery; D007410:Intestinal Diseases / Q000209:etiology*; D007415:Intestinal Obstruction / Q000151:congenital / Q000601:surgery*; D007422:Intestines / Q000601:surgery; D017093:Liver Failure / Q000097:blood / Q000209:etiology*; D008297:Male; D010289:Parenteral Nutrition, Total / Q000009:adverse effects*; D010743:Phospholipids / Q000009:adverse effects*; D011182:Postoperative Care / Q000009:adverse effects*; D012189:Retrospective Studies; D018805:Sepsis / Q000150:complications; D013024:Soybean Oil / Q000009:adverse effects*
D004655:Emulsions; D010743:Phospholipids; C545823:soybean oil, phospholipid emulsion; D013024:Soybean Oil; D000469:Alkaline Phosphatase