HEMODIALYSIS COMBINED WITH CONTINUOUS PERITONEAL-DIALYSIS IN THE MANAGEMENT OF INBORN-ERRORS OF METABOLISM

In children with inborn errors of metabolism the accumulation of toxic metabolites is greater at initial presentation than that at any time in their lives, and often results in irreversible tissue damage, parti cularly to the central nervous system. Reduction in the levels of circ ulating toxins is a matter of urgency. Conventional methods of managem ent include exchange transfusion, forced diuresis with or without insu lin, continuous peritoneal dialysis and, in some conditions, specific therapies such as enzyme cofactors. These therapies generally achieve correction in 24 hours or more. More recently extracorporal methods li ke hemodialysis or hemofiltration have shown to be more rapidly effect ive. In a newborn with severe hyperammonemia (1039 mug/dl) due to an a rginine-succinase deficiency, we combined hemodialysis to remove quick and effective low molecular weight substances from the organism (seru m-ammonium 447 mug/dl after the first, 338 mug/dl after the second ses sion, respectively) with continuous peritoneal dialysis to keep these substances in non-neurotoxic ranges (<300 mug/dl) until conservative t reatment was successful.