Continuous Renal Replacement Therapy (CRRT) in the Treatment of
Hyperammonemia Associated with Inborn Errors of Metabolism.
D. Eding, H. Marine, N. Hautala, L. Border, J. Harley,
R. Hackbarth, T. Bunchman
Children presenting with severe hyperammonemia require rapid correction of their ammonia levels to minimize neurologic injury. Two newborns presenting with hyperammonemic encephalopathy were treated with hemodialysis (HD) then transitioned to continuous veno-venous hemodiafiltration. CRRT was started using the Gambro Prismaflex, a blood flow of 25mL/kg/minute and citrate anticoagulation. A M60 filter was utilized on an infant with methylmalonic acidemia, with initial flow rates of filter replacement fluid (FRF) at 1.5L/hour and dialysate at 2L/hour. Flow rates were weaned over a 16 hour period and CRRT was discontinued. A
HF 1000 filter was utilized on an infant with a urea cycle defect with both
FRF and dialysate at 4L/hour. Flow rates were decreased over a 52 hour period and CRRT was discontinued. Both infants had ammonia levels of over 1000 mcmol/L prior to therapy, which rapidly decreased on HD and continued to decline on CRRT to levels less than 100 mcmol/L for the duration of the therapy. Sequential HD/CRRT is effective in the acute management of hyperammonemia associated with metabolic disease.
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