Management of acute episodes of metabolic decompensation are treated by giving glucose containing fluids to restore calorie intake. A dose of IV carnitine can be given if not already being treated with it. Dialysis or hemofiltration can be used if acidosis or hyperammonemia do not resolve with initial therapy or are extreme. A return to oral or G-tube feeds as soon as clinically safe will improve recovery time. Chronic treatment includes restriction of dietary leucine, including leucine restricted medical formula if necessary to give sufficient total protein. Supplementation with carnitine (100 mg/kg/day divided three times daily) and or glycine (150-250 mg/kg/day divided four times daily) will improve metabolite excretion.
IVD