Renal replacement therapy in neonates with an inborn error of metabolism
Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma...
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| Format: | Article |
| Language: | English |
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Korean Pediatric Society
2019-02-01
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| Series: | Korean Journal of Pediatrics |
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| Online Access: | http://www.kjp.or.kr/upload/pdf/kjp-2018-07143.pdf |
| _version_ | 1819094230703800320 |
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| author | Heeyeon Cho |
| author_facet | Heeyeon Cho |
| author_sort | Heeyeon Cho |
| collection | DOAJ |
| description | Hyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma, severe disability, and even death. Thus, emergency treatment for hyperammonemia must be initiated before a precise diagnosis is established. In neonates with hyperammonemia caused by an inborn error of metabolism, a few studies have suggested that peritoneal dialysis, intermittent hemodialysis, and continuous renal replacement therapy (RRT) are effective modalities for decreasing the plasma level of ammonia. In this review, we discuss the current literature related to the use of RRT for treating neonates with hyperammonemia caused by an inborn error of metabolism, including optimal prescriptions, prognosis, and outcomes. We also review the literature on new technologies and instrumentation for RRT in neonates |
| first_indexed | 2024-12-21T23:24:06Z |
| format | Article |
| id | doaj.art-a66489de4e0e4c52834ce58ea3aa7951 |
| institution | Directory Open Access Journal |
| issn | 1738-1061 2092-7258 |
| language | English |
| last_indexed | 2024-12-21T23:24:06Z |
| publishDate | 2019-02-01 |
| publisher | Korean Pediatric Society |
| record_format | Article |
| series | Korean Journal of Pediatrics |
| spelling | doaj.art-a66489de4e0e4c52834ce58ea3aa79512022-12-21T18:46:43ZengKorean Pediatric SocietyKorean Journal of Pediatrics1738-10612092-72582019-02-01622434710.3345/kjp.2018.0714320125553498Renal replacement therapy in neonates with an inborn error of metabolismHeeyeon ChoHyperammonemia can be caused by several genetic inborn errors of metabolism including urea cycle defects, organic acidemias, fatty acid oxidation defects, and certain disorders of amino acid metabolism. High levels of ammonia are extremely neurotoxic, leading to astrocyte swelling, brain edema, coma, severe disability, and even death. Thus, emergency treatment for hyperammonemia must be initiated before a precise diagnosis is established. In neonates with hyperammonemia caused by an inborn error of metabolism, a few studies have suggested that peritoneal dialysis, intermittent hemodialysis, and continuous renal replacement therapy (RRT) are effective modalities for decreasing the plasma level of ammonia. In this review, we discuss the current literature related to the use of RRT for treating neonates with hyperammonemia caused by an inborn error of metabolism, including optimal prescriptions, prognosis, and outcomes. We also review the literature on new technologies and instrumentation for RRT in neonateshttp://www.kjp.or.kr/upload/pdf/kjp-2018-07143.pdfContinuous renal replacement therapyNeonateInborn error of metabolism |
| spellingShingle | Heeyeon Cho Renal replacement therapy in neonates with an inborn error of metabolism Korean Journal of Pediatrics Continuous renal replacement therapy Neonate Inborn error of metabolism |
| title | Renal replacement therapy in neonates with an inborn error of metabolism |
| title_full | Renal replacement therapy in neonates with an inborn error of metabolism |
| title_fullStr | Renal replacement therapy in neonates with an inborn error of metabolism |
| title_full_unstemmed | Renal replacement therapy in neonates with an inborn error of metabolism |
| title_short | Renal replacement therapy in neonates with an inborn error of metabolism |
| title_sort | renal replacement therapy in neonates with an inborn error of metabolism |
| topic | Continuous renal replacement therapy Neonate Inborn error of metabolism |
| url | http://www.kjp.or.kr/upload/pdf/kjp-2018-07143.pdf |
| work_keys_str_mv | AT heeyeoncho renalreplacementtherapyinneonateswithaninbornerrorofmetabolism |