IBAT inhibitors block intestinal absorption of bile acids/disrupt enterohepatic circulation this leads to augmented bile acid excretion in stools.Lipid management has been crucial in reducing the number of children needing intestinal transplantation.Reduction of lipid doses can have negative effects on brain growth Does not prevent hepatic fibrosis progression 2. SMOF lipid allows full dosing of lipids (3 gm/kg).Treatments: Advance enteral feeds, lipid modulation, prevent CLABSI, treat bacterial overgrowth, GLP-2, and STEP procedure/tapering.Lipid reduction also is associated with cholestasis resolution.(Ann Surg 2009 250: 395) showed that fish oil (at lower doses) was associated with improvement/resolution of parenteral nutrition associated cholestasis (PNAC) Intestinal microbiome is altered in patients with IFALD.Even after weaning off PN, studies have shown long-lasting fibrosis and steatosis in more than 40% of patients (>8 yrs off PN).Conjugated bilirubin >2.5 had RR 22.5 for mortality (prior to availability of intestinal transplantation).IFALD presentations: Steatosis, biliary tract disease and cholestasis Biliary cirrhosis related to parenteral nutrition has been the major indication for small bowel transplantation/multi-visceral transplantation.Though not intentional, some important material is likely to have been omitted in addition, transcription errors are possible as well. This blog entry has abbreviated/summarized several presentations.
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