title: 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.
authors.affiliation: Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. kwanchai.pio@mahidol.ac.th
authors.forename: Kwanchai
authors.initials: K
authors.lastname: Pirojsakul
authors.affiliation: Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Kanchana
authors.initials: K
authors.lastname: Tangnararatchakit
authors.affiliation: Division of Pediatric Critical Care, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Jarin
authors.initials: J
authors.lastname: Vaewpanich
authors.affiliation: Department of Nursing, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Kanyapat
authors.initials: K
authors.lastname: Niyomvit
authors.affiliation: Department of Nursing, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Duangjai
authors.initials: D
authors.lastname: Chanhom
authors.affiliation: Department of Nursing, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Rungnapa
authors.initials: R
authors.lastname: Udomchaisakul
authors.affiliation: Division of Nephrology, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Wiwat
authors.initials: W
authors.lastname: Tapaneya-Olarn
authors.affiliation: Division of Medical Genetics, Department of Pediatrics, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand.
authors.forename: Duangrudee
authors.initials: D
authors.lastname: Wattanasirichaigoon
mesh_terms: D006440:Hemofiltration / Q000379:methods; D006801:Humans; D022124:Hyperammonemia / Q000209:etiology*; D007231:Infant, Newborn; D008297:Male; D020163:Ornithine Carbamoyltransferase Deficiency Disease / Q000150:complications*; D014471:Umbilical Veins; D062666:Vascular Access Devices