Indian Journal of Anaesthesia  
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CASE REPORT
Year : 2016  |  Volume : 60  |  Issue : 1  |  Page : 50-54

Anaesthetic considerations for liver transplantation in propionic acidemia


1 Department of Hepatobiliary and Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India
2 Department of Hepatobiliary and Liver Transplant Surgery, Institute of Liver Disease and Transplantation, Global Health City, Chennai, Tamil Nadu, India
3 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

Correspondence Address:
Akila Rajakumar
Department of Hepatobiliary and Liver Transplant Anaesthesia and Intensive Care, Institute of Liver Disease and Transplantation, Global Health City, 439, Cheran Nagar, Perumbakkam, Chennai - 600 100, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5049.174799

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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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