Methylacetoacetyl-coenzyme A thiolase (MAT) deficiency is an autosomal recessive disease caused by a defect of mitochondrial acetoacetyl-CoA thiolase (T2). There is an error of isoleucine catabolism and ketone body utilization due to mutations in the acetyl-Coenzyme A acetyltransferase 1 (ACAT1) gene. We report a case of a 14 months old Sabahan boy with beta deficiency who presented with severe sepsis and ketoacidosis who subsequently recovered.
Administering anaesthesia for elderly patients with chronic schizophrenia has always been a great challenge to anaesthetists. These patients will usually be on multiple antipsychotic drugs for many years and may lead to delayed awakening, cardiovascular instability, arrhythmias and sudden cardiac death during general anaesthesia. This case report is about the perioperative anaesthetic management of an elderly schizophrenic patient undergoing removal of femur implant. This article will explore important drug interactions and available options for a successful anaesthesia.