Continuous venovenous hemofiltration in the management of acute decompensation in inborn errors of metabolism.
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.
Thompson
G N
GN
Department of Paediatrics, University of Melbourne, Murcoch Institute, Australia.
Butt
W W
WW
Shann
F A
FA
Kirby
D M
DM
Henning
R D
RD
Howells
D W
DW
Osborne
A
A
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*
D000596:Amino Acids; D000597:Amino Acids, Branched-Chain; D005227:Fatty Acids