Anesthetic management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency.
Patients with inborn errors of metabolism require special considerations in perioperative care. In the following case report, we describe the successful management of a patient with 3-methylcrotonyl-CoA carboxylase deficiency, a deficit that causes a secondary carnitine deficiency and impaired beta oxidation. Patients may have significant underlying cardiomyopathy, and are at risk for metabolic decompensation, acidosis, and hypoglycemia during periods of stress.
Robbins
Karen A
KA
Department of Anesthesiology and Perioperative Medicine, University Hospitals Case Medical Center, Cleveland, Ohio, USA. robbinska@gmail.com
León-Ruiz
Elias N
EN
D000328:Adult; D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications / Q000175:diagnosis; D000768:Anesthesia, General; D019735:Carbon-Carbon Ligases / Q000172:deficiency*; D009202:Cardiomyopathies / Q000150:complications*; D002331:Carnitine / Q000172:deficiency; D005260:Female; D006801:Humans; D007930:Leucine / Q000378:metabolism*
D019735:Carbon-Carbon Ligases; C021573:methylcrotonoyl-CoA carboxylase; D007930:Leucine; D002331:Carnitine