[Hemofiltration in acute neonatal metabolic crisis]

Area of Science:

• Metabolic disorders in neonates
• Renal replacement therapy
• Toxic metabolite removal

Context:

• Maple syrup urine disease and ornithine transcarbamylase deficiency are severe metabolic disorders in neonates.
• Encephalopathy is a critical complication of these conditions.
• Traditional treatments may be less effective in metabolite clearance.

Purpose:

• To evaluate the efficacy of continuous arteriovenous hemofiltration (CAVH) and continuous venovenous hemofiltration (CVVH) in treating neonates with specific metabolic disorders.
• To compare the effectiveness of hemofiltration with peritoneal dialysis in removing toxic metabolites.
• To assess the safety and tolerability of continuous hemofiltration in critically ill infants.

Summary:

• Continuous arteriovenous hemofiltration (CAVH) and continuous venovenous hemofiltration (CVVH) were successfully applied to two neonates suffering from maple syrup urine disease and ornithine transcarbamylase deficiency.
• Both methods led to a rapid decrease in plasma concentrations of branched-chain amino acids and ammonium to non-toxic levels.
• Striking clinical improvement in encephalopathy was observed, highlighting the effectiveness of hemofiltration.

Impact:

• Hemofiltration demonstrates superior efficacy over peritoneal dialysis in clearing toxic metabolites associated with metabolic disorders.
• While intermittent hemodialysis offers the highest clearance rates, continuous hemofiltration presents a simple, safe, and effective alternative.
• Continuous hemofiltration is well-tolerated by critically ill infants, making it a valuable therapeutic option.