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Korean J Pediatr 2011 October;54(10) :425-428.
Acute treatment of hyperammonemia by continuous renal replacement therapy in a newborn patient with ornithine transcarbamylase deficiency
Hyo Jeong Kim (Kim HJ)1, Se Jin Park (Park SJ)2, Kook In Park (Park KI)1, Jin Sung Lee (Lee JS)3, Ho Sun Eun (Eun HS)1, Ji Hong Kim (Kim JH)1, Jae Il Shin (Shin JI)1
1The Institute of Kidney Disease, Department of Pediatrics, Yonsei University College of Medicine, Seoul, Korea
2Department of Pediatrics, Ajou University Hospital, Ajou University School of Medicine, Suwon, Korea
3Department of Clinical Genetics, Yonsei University College of Medicine, Seoul, Korea
Corresponding Author: Jae Il Shin ,Tel: +82-2-2228-2073, Fax: +82-2-393-9118, Email:
Copyright © 2011 by The Korean Pediatric Society
Ornithine transcarbamylase (OTC) deficiency is well known as the most common inherited disorder of the urea cycle, and 1 of the most common causes of hyperammonemia in newborns. We experienced a case of a 3-day-old boy with OTC deficiency who appeared healthy in the first 2 days of life but developed lethargy and seizure soon afterwards. His serum ammonia level was measured as >1700 g/dL (range, 0 to 45 g/dL). Continuous renal replacement therapy (CRRT) in the mode of continuous venovenous hemodiafiltration was immediately applied to correct the raised ammonia level. No seizure occurred after the elevated ammonia level was reduced. Therefore, CRRT should be included as 1 of the treatment modalities for newborns with inborn errors of metabolism, especially hyperammonemia. Here, we report 1 case of successful treatment of hyperammonemia by CRRT in a neonate with OTC deficiency.
Keywords: Ornithine transcarbamylase deficiency | Hyperammonemia | Continuous renal replacement therapy | Infant | Newborn
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