Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation -a case report-

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Intractable metabolic acidosis in a child with propionic acidemia undergoing liver transplantation -a case report-

Propionic acidemia (PA) is a rare autosomal recessive disorder of metabolism caused by deficient activity of the mitochondrial enzyme propionyl-CoA carboxylase. The clinical manifestations are metabolic acidosis, poor feeding, lethargy, vomiting, osteoporosis, neurological dysfunction, pancytopenia, developmental retardation and cardiomyopathy. Liver transplantation has recently been considered...

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ژورنال

عنوان ژورنال: Korean Journal of Anesthesiology

سال: 2013

ISSN: 2005-6419,2005-7563

DOI: 10.4097/kjae.2013.65.3.257