title: Anaesthetic considerations for liver transplantation in propionic acidemia.
abstract: Propionic acidemia (PA) is an autosomal recessive disorder of metabolism due to deficiency of the enzyme propionyl-CoA carboxylase (PCC) that converts propionyl-CoA to methylmalonyl-CoA with the help of the cofactor biotin inside the mitochondria. The resultant accumulation of propionyl-CoA causes severe hyperammonaemia and life-threatening metabolic acidosis. Based on the positive outcomes, liver transplantation is now recommended for individuals with recurrent episodes of hyperammonaemia or acidosis that is not adequately controlled with appropriate medical therapies. We report anaesthetic management of two children with PA for liver transplantation at our institution. It is essential for the anaesthesiologist, caring for these individuals to be familiar with the manifestations of the disease, the triggers for decompensation and management of an acute episode.
authors.affiliation: Department of Hepatobiliary and Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India.
authors.forename: Akila
authors.initials: A
authors.lastname: Rajakumar
authors.affiliation: Department of Hepatobiliary and Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India.
authors.forename: Ilankumaran
authors.initials: I
authors.lastname: Kaliamoorthy
authors.affiliation: Department of Hepatobiliary and Liver Transplant Surgery, Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India.
authors.forename: Mettu Srinivas
authors.initials: MS
authors.lastname: Reddy
authors.affiliation: Department of Hepatobiliary and Liver Transplant Surgery, Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India; Department of Hepatobiliary and Liver Transplant Surgery, Institute of Liver Studies, King's College, London.
authors.forename: Mohamed
authors.initials: M
authors.lastname: Rela
keywords: Hyperammonaemic crisis; inborn metabolic errors; liver transplantation; organic acidemia; propionic acidemia and anaesthesia