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Hyperammonemia and neonatal cardiac failure.
MedLine Citation:
PMID:  21540812     Owner:  NLM     Status:  In-Data-Review    
Abstract/OtherAbstract:
Severe hyperammonemia (hyperNH3) in neonatal cardiac failure after cardiac surgery is rare. We report a case of a 2470-g female infant born at the week 37 of gestation with complex congenital heart disease (truncus arteriosus type III, interrupted aortic arch and tricuspid valve insufficiency) and hemodynamically non-significant intrahepatic arterio-venous malformation. She developed hyperNH3 (highest NH3 blood level: 467 µmol/L) without severe liver failure (INR of 1.9). The origin of the hyperNH3 was multifactorial including limited capacity of liver detoxification function due to congenital porto-caval shunt, liver ischemia, excessive protein intake and increased protein catabolic rate. HyperNH3 treatment partially succeeded in decreasing ammonia level and included discontinuation of protein intake, administration of phenylacetate and sodium benzoate. This case highlights the fact that NH3 detoxification by the liver has limitations for a neonate with multifactorial causes that decrease liver perfusion.
Authors:
T Adamovic; P Jouvet; S Vobecky; L Garel; A L Rougemont; F Alvarez
Publication Detail:
Type:  Journal Article     Date:  2011-03-02
Journal Detail:
Title:  Minerva anestesiologica     Volume:  77     ISSN:  1827-1596     ISO Abbreviation:  Minerva Anestesiol     Publication Date:  2011 May 
Date Detail:
Created Date:  2011-05-04     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0375272     Medline TA:  Minerva Anestesiol     Country:  Italy    
Other Details:
Languages:  eng     Pagination:  554-7     Citation Subset:  IM    
Affiliation:
Department of Pediatrics, Sainte-Justine Hospital, University of Montréal, QC, Montréal, Canada - philippe.jouvet@umontreal.ca.
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