Normal Growth and Essential Fatty Acid Status in Children With Intestinal Failure on Lipid Limitation

Objectives: Lipid limitation, that is, < 1 g center dot kg - 1 center dot day - 1 of soy oil lipid emulsion ( SOLE), has been suggested as a method to reduce the risk of intestinal failure ( IF)- associated liver disease ( IFALD). There are limited data as to the effects of this strategy on growth and essential fatty acid ( EFA) status. The aim of the study was to assess growth, prevalence of cholestasis, and EFA deficiency in patients with IF who were provided daily SOLE at a dose <= 1 g center dot kg - 1 center dot day-1. Methods: Medical records were retrospectively reviewed from 9 patients age 16 months to 8 years who had IF requiring parenteral nutrition support for > 12 months. Parenteral nutrition supplied a mean of 53% of total energy ( range 24%-86%). Results: Mean SOLE dose was 0.61 g center dot kg center dot 1 center dot day - 1 ( range 0.4- 0.81 g center dot kg - 1 center dot day - 1). After 1 month of lipid limitation between 2011 and 2014, no patient developed IFALD as defined by a direct bilirubin > 2 mg/ dL. The median direct bilirubin was 0.1 mg/ dL ( range 0.075- 0.85 mg/ dL). No patient developed EFA deficiency as defined by a triene- to- tetraene ratio > 0.2 ( median 0.026, range 0.017- 0.076). Height z scores increased from mean of - 2.568 ( range - 10.8 to 0.878) to - 0.484 ( range - 3.546 to 0.822). Weight z scores increased from mean of - 1.412 ( range - 5.871 to 0.906) to 0.595 ( range 2.178 to 0.926). Conclusions: In this case series, lipid limitation allowed normal growth while preventing the development of cholestasis and EFA deficiency.