Anesthetic Management for Pediatric Liver Transplantation in a Patient With Propionic Acidemia: A Case Report
نویسندگان
چکیده
منابع مشابه
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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BACKGROUND Propionic acidemia (PA) is a rare but severe recessive autosomal disease, presenting with non specific signs in the first years of life. Prenatal diagnosis is invasive (amniocentesis) and limited to suspect cases. No screening test has been described, in particular no correlations between prenatal sonography and PA have been documented so far. CASE PRESENTATION We report the case o...
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ژورنال
عنوان ژورنال: Journal of Investigative Medicine High Impact Case Reports
سال: 2021
ISSN: 2324-7096,2324-7096
DOI: 10.1177/23247096211015025