A case of neonatal hyperammonemia successfully treated by efficient continuous hemodialysis

Abstract

The prognosis of neonatal hyperammonemia due to an inborn error of metabolism is poor, and as irreversible brain damage occurs within a short period of time, it is necessary to rapidly reduce the blood ammonia level. While exchange transfusion and peritoneal dialysis have been performed to remove blood ammonia, their effectiveness has often been insufficient, and hemodialysis is necessary for rapid and reliable removal of ammonia. To remove ammonia by hemodialysis, it is important to maximize the blood flow rate (Qb) and set the dialysate flow rate (Qd) at a level sufficient for the maximized Qb. We treated a neonate with ornithine transcarbamylase deficiency by continuous hemodialysis. The Qb was set at 25mL/min, and the Qd was increased to 3,000mL/hr (50mL/min), at which the Qd/Qb was 2.0, and then appropriately adjusted. This case is presented because a favorable outcome could be obtained by setting the Qb and Qd at levels higher than were previously reported.