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https://dora.health.qld.gov.au/qldresearchjspui/handle/1/4701| Title: | Successful management of a neonate with OTC deficiency presenting with hyperammonemia and severe cardiac dysfunction with extracorporeal membrane oxygenation support and continuous renal replacement therapy | Authors: | McGill, J. Venugopal, P. Justo, R. Mattke, A. C. Shikata, F. |
Issue Date: | 2020 | Source: | Sep 55, (1), 2020, p. 12-14 | Pages: | 12-14 | Journal: | JIMD Rep | Abstract: | Ornithine transcarbamylase (OTC) deficiency is an X-linked urea cycle disorder which-in severe form-results in rapid accumulation of ammonia and glutamine with subsequent irreversible brain injury. We present a case of severe left ventricular dysfunction with hyperammonemic crisis caused by OTC deficiency which was managed with veno-arterial extracorporeal membrane oxygenation support combined with continuous renal replacement therapy. Aggressive treatment led to normalization of ammonia and full left ventricular recovery.2192-8312Mattke, Adrian C | DOI: | 10.1002/jmd2.12135 | Keywords: | cardiac dysfunction;children;EcmoOTC deficiency;extracorporeal;renal replacement therapy | Type: | Article |
| Appears in Sites: | Children's Health Queensland Publications |
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