Incidence, Prevention, and Treatment of Parenteral Nutrition-Associated Cholestasis and Intestinal Failure-Associated Liver Disease in Infants and Children: A Systematic Review

Department of Surgery, UCL Institute of Child Health, London, UK.
Journal of Parenteral and Enteral Nutrition (Impact Factor: 3.15). 07/2013; 38(1). DOI: 10.1177/0148607113496280
Source: PubMed


Cholestasis is a significant life-threatening complication in children on parenteral nutrition (PN). Strategies to prevent/treat PN-associated cholestasis (PNAC) and intestinal failure-associated liver disease (IFALD) have reached moderate success with little supporting evidence. Aims of this systematic review were (1) to determine the incidence of PNAC/IFALD in children receiving PN for ≥ 14 days and (2) to review the efficacy of measures to prevent/treat PNAC/IFALD.

Of 4696 abstracts screened, 406 relevant articles were reviewed, and studies on children with PN ≥ 14 days and cholestasis (conjugated bilirubin ≥ 2 mg/dL) were included. Analyzed parameters were (1) PNAC/IFALD incidence by decade and by PN length and (2) PNAC/IFALD prevention and treatment (prospective studies).

Twenty-three articles (3280 patients) showed an incidence of 28.2% and 49.8% of PNAC and IFALD, respectively, with no evident alteration over the last decades. The incidence of PNAC was directly proportional to the length of PN (from 15.7% for PN ≤ 1 month up to 60.9% for PN ≥ 2 months; P < .0001). Ten studies on PNAC met inclusion criteria. High or intermediate-dose of oral erythromycin and aminoacid-free PN with enteral whey protein gained significant benefits in preterm neonates (P < .05, P = .003, and P < .001, respectively). None of the studies reviewed met inclusion criteria for treatment.

The incidence of PNAC/IFALD in children has no obvious decrease over time. PNAC is directly correlated to the length of PN. Erythromycin and aminoacid-free PN with enteral whey protein have shown to prevent PNAC in preterm neonates. There is a lack of high-quality prospective studies, especially on IFALD.

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Available from: Giuseppe Lauriti, Feb 14, 2015
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