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Updated: Mar 24, 2026

Porcine Liver Transplantation Without Veno-Venous Bypass As an Extended Criteria Donor Model
Published on: August 17, 2022
Anaesthetic considerations for liver transplantation in propionic acidemia
Akila Rajakumar1, Ilankumaran Kaliamoorthy1, Mettu Srinivas Reddy2
1Department of Hepatobiliary and Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India.
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.
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