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

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

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...

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Bibliographic Details
Main Authors: Adrian C. Mattke, Fumiaki Shikata, James McGill, Rob Justo, Prem Venugopal
Format: Article
Language:English
Published: Wiley 2020-09-01
Series:JIMD Reports
Subjects:
cardiac dysfunction
children
ECMO
extracorporeal
OTC deficiency
renal replacement therapy
Online Access:https://doi.org/10.1002/jmd2.12135

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