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Acute treatment of hyperammonemia by continuous renal replacement therapy in a newborn patient with ornithine transcarbamylase deficiency

Authors
 Hyo Jeong Kim  ;  Se Jin Park  ;  Jae Il Shin  ;  Ji Hong Kim  ;  Ho Sun Eun  ;  Jin Sung Lee  ;  Kook In Park 
Citation
 Korean Journal of Pediatrics, Vol.54(10) : 425-428, 2011 
Journal Title
 Korean Journal of Pediatrics 
ISSN
 1738-1061 
Issue Date
2011
Abstract
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 µg/dL (range, 0 to 45 µg/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
URI
https://ir.ymlib.yonsei.ac.kr/handle/22282913/94938
DOI
10.3345/kjp.2011.54.10.425
Appears in Collections:
1. Journal Papers (연구논문) > 1. College of Medicine (의과대학) > Dept. of Pediatrics (소아청소년과학교실)
Yonsei Authors
김지홍(Kim, Ji Hong) ; 박국인(Park, Kook In) ; 신재일(Shin, Jae Il) ; 은호선(Eun, Ho Seon) ; 이진성(Lee, Jin Sung)
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