Medical Management and Dialysis Therapy for the Infant With an Inborn Error of Metabolism

Summary 

Optimal care of the neonate with hyperammonemia requires expertise in the evaluation, medical management, and decision to initiate dialytic therapy, and therefore compels expeditious collaboration between neonatal intensive care physicians, medical geneticists, and pediatric nephrologists. Neonatal and dialysis nursing expertise also is paramount for the successful provision of dialysis therapy in this setting. The current article addresses the underlying causes, medical management strategies, and dialytic therapy considerations in caring for the neonate with hyperammonemia.

Keywords: Hyperammonemia, urea cycle defects, organic acidurias, hemodialysis, continuous arteriovenous hemodialysis, continuous venovenous hemodialysis