Successful Continuous Venovenous Hemofiltration in a
Neonate with Hyperammonemia from Ornithine
Transcabamylase Deficiency
Kwanchai Pirojsakul MD*, Kanchana Tangnararatchakit MD*,
Jarin Vaewpanich MD**, Kanyapat Niyomvit RN****, Duangjai Chanhom RN****,
Rungnapa Udomchaisakul RN****, Wiwat Tapaneya-Olarn MD*, Duangrudee Wattanasirichaigoon MD***
Affiliation :
* Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital,
Mahidol University, Bangkok, Thailand
** Division of Pediatric Critical Care, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital,
Mahidol University, Bangkok, Thailand
*** Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital,
Mahidol University, Bangkok, Thailand
**** Department of Nursing, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand
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.
Keywords : Hemodialysis, Hemofiltration, Hyperammonemia, Neonate, Urea cycle disorder
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