Clearance of amino acids by hemodialysis in argininosuccinate synthetase deficiency.
We determined the dialytic clearance of amino acids involved in ammoniagenesis and nitrogen excretion in a neonate with argininosuccinate synthetase deficiency who underwent acute hemodialysis. Plasma ammonia and plasma and dialysate amino acid concentrations were obtained at baseline, 30-minute intervals during hemodialysis, and 30 minutes after the completion of hemodialysis. Plasma ammonia concentrations declined by 56% during the 90-minute hemodialysis treatment, whereas arginine, citrulline, glutamine, and glycine concentrations decreased by 65%, 55%, 40%, and 34%, respectively. Mean dialytic clearances for arginine, citrulline, glutamine, and glycine were 24, 282, 263, and 189 mL/min per 1.73 m(2), respectively. The high dialytic clearance of citrulline suggests a novel mechanism of hemodialysis removal of nitrogen. Dialytic clearances of glutamine and glycine may prevent further ammoniagenesis in hyperammonemic patients. However, our data suggest that hemodialysis affects the precursors of alternative pathway removal of ammonia. Further study is needed to optimize the intradialytic and interdialytic dosing of substrates.
McBryde
Kevin D
KD
Department of Nephrology, Children's National Medical Center, George Washington University, 111 Michigan Avenue NW, Washington, DC 20010, USA. KMCBRYDE@cnmc.org
Kudelka
Timothy L
TL
Kershaw
David B
DB
Brophy
Patrick D
PD
Gardner
John J
JJ
Smoyer
William E
WE
D000596:Amino Acids / Q000378:metabolism*; D000641:Ammonia / Q000097:blood; D020159:Citrullinemia / Q000097:blood / Q000628:therapy*; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D006435:Renal Dialysis
D000596:Amino Acids; D000641:Ammonia