title: Continuous venovenous hemofiltration in the management of acute decompensation in inborn errors of metabolism.
abstract: Continuous venovenous hemofiltration was used to treat two neonates, one with maple syrup urine disease and the other with an inborn error of long-chain fatty acid oxidation. The latter infant had hypoglycemia, hyperammonemia and lactic acidosis. In both cases, acceptable biochemical control was achieved within 8 hours. This therapy offers the potential to overcome acute crises rapidly in a wide range of inborn errors of intermediary metabolism.
authors.affiliation: Department of Paediatrics, University of Melbourne, Murcoch Institute, Australia.
authors.forename: G N
authors.initials: GN
authors.lastname: Thompson
authors.forename: W W
authors.initials: WW
authors.lastname: Butt
authors.forename: F A
authors.initials: FA
authors.lastname: Shann
authors.forename: D M
authors.initials: DM
authors.lastname: Kirby
authors.forename: R D
authors.initials: RD
authors.lastname: Henning
authors.forename: D W
authors.initials: DW
authors.lastname: Howells
authors.forename: A
authors.initials: A
authors.lastname: Osborne
mesh_terms: D000596:Amino Acids / Q000097:blood; D000597:Amino Acids, Branched-Chain / Q000097:blood; D005227:Fatty Acids / Q000378:metabolism*; D005260:Female; D006440:Hemofiltration / Q000379:methods*; D006801:Humans; D007231:Infant, Newborn; D008052:Lipid Metabolism, Inborn Errors / Q000097:blood / Q000628:therapy*; D008297:Male; D008375:Maple Syrup Urine Disease / Q000097:blood / Q000628:therapy*
chemical_list: D000596:Amino Acids; D000597:Amino Acids, Branched-Chain; D005227:Fatty Acids