Baylor College of Medicine – Cholestasis

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While fish-oil lipid emulsions have shown a benefit to the treatment of parenteral nutrition
(PN)-associated cholestasis, the dose is limited to 1 g/kg/day. Similarly, in early
PN-associated cholestasis the dose of soy-based lipid is limited to 1 g/kg/day. Often the
calories that are lost from this relative decreased dose of lipids can be provided by
adjusting the dextrose content of the PN solution and providing a higher glucose infusion
rate. In some cases, this is not tolerated or even with maximizing this strategy, growth is
inadequate. Inadequate growth is a direct cause of poor outcomes including poorer
neurological outcome, failure to be able to stop mechanical ventilation and poorer growth of
their often already damaged intestine. These outcomes can lead to severe disability and
death. Therefore, infants receiving only 1 g/kg/day of lipids who are not adequately growing
must have a greater intake of lipids to meet their needs for weight, length, and head
circumference growth.

SMOFlipid (Fresenius Kabi, Bad Homburg, Germany) contains a mixture of 4 different lipid
sources: soybean oil providing essential fatty acids, olive oil rich in monounsaturated
fatty acids which are less susceptible to lipid peroxidation than polyunsaturated fatty
acids, medium-chain triglycerides showing a faster metabolic clearance than long-chain
triglycerides, and fish oil for the supply of omega-3 fatty acids. It is safe to give in
what is the usual dose for lipid therapy in neonates of 3 g/kg/day, rather than being
limited to 1 g/kg/day as we do with cholestatic infants receiving Omegaven or soy lipids.
Because this product includes both omega-6 and omega-3 lipids, it provides the benefits of
the omega-3s for the liver and provides more than enough omega-6s to meet essential fatty
acid requirements. Its use in situations in which growth is inadequate in babies who must be
restricted to 1 g/kg/day can be expected to improve their growth and likely markedly
increase their chances of both a good neurological outcome and survival.

Purpose: We want to find out if this new intravenous fat mixture (SMOFlipid) will help
promote good growth while reducing the severity (or seriousness) of liver disease or help
put an end to liver disease in infants.

Rare Disease: