Successful continuous venovenous hemofiltration in a neonate with hyperammonemia from ornithine transcabamylase deficiency.

Abstract

Ornithine transcabamylase (OTC) deficiency is the most common and severe form of abnormal urea synthesis. It can result in hyperammonemia, severe neurologic manifestation, brain edema, and early death. Rapid removal of ammonia by hemodialysis can decrease mortality and morbidity in the patients with severe increase of ammonia levels. However hemodialysis (HD) in infants and young children are technically difficult to perform. Continuous venovenous hemofiltration (CVVH) is increasingly used as an alternative for HD, but performing CVVH in a neonate can be problematic due to small body size and difficult vascular access. The authors reported a successful CVVH using umbilical vein as a vascular access site for ammonia removal in a neonate with OTC deficiency with progressive elevation of plasma ammonia. Technical problems, pitfalls in performing the CVVH, and how the authors overcame the problems are discussed.