Abstract
BackgroundGlutaric acidemia is a type of multiple acyl-coenzyme A dehydrogenase deficiency, an inborn error in fatty acid metabolism. In patients with glutaric acidemia, during the perioperative period, prolonged fasting, stress, and pain have been identified as risk factors for the induction of metabolic derangement. This report describes the surgical and anesthetic management of a patient with glutaric acidemia.Case presentationA 56-year-old male patient with glutaric acidemia type 2 underwent a series of surgeries. During the initial off-pump coronary artery bypass surgery, the patient developed renal failure due to rhabdomyolysis upon receiving glucose at 2 mg/kg/min. However, in the second laparoscopic cholecystectomy, rhabdomyolysis was avoided by administering glucose at 4 mg/kg/min.ConclusionsTo avoid catabolism in patients with glutaric acidemia, appropriate glucose administration is important, depending on the surgical risk.
Highlights
BackgroundGlutaric acidemia type 2 is a type of multiple acylcoenzyme A dehydrogenase deficiency, which is an autosomal recessive inborn error of fatty acid metabolism
Glutaric acidemia is a type of multiple acyl-coenzyme A dehydrogenase deficiency, an inborn error in fatty acid metabolism
Glutaric acidemia type 2 is a type of multiple acylcoenzyme A dehydrogenase deficiency, which is an autosomal recessive inborn error of fatty acid metabolism
Summary
Glutaric acidemia type 2 is a type of multiple acylcoenzyme A dehydrogenase deficiency, which is an autosomal recessive inborn error of fatty acid metabolism. Case presentation A 56-year-old male patient (weight, 82 kg; height, 168 cm) with glutaric acidemia type 2 was scheduled for laparoscopic cholecystectomy for cholecystolithiasis He had a history of rhabdomyolysis with lumbago and brown urine as initial symptoms at the age of 44 years. Administration of 2 mg/kg/min glucose using 10% glucose solution was started from midnight of the day before surgery It was continued throughout the operation and postoperatively in the intensive care unit (ICU) using 50% glucose solution. The glucose infusion rate was 2 mg/kg/min using 10% glucose solution, was started from midnight of the day before surgery, and increased to 4 mg/kg/min using 50% glucose solution when introducing anesthesia. There was a slight increase in the postoperative serum CK and myoglobin levels at a maximum value of 534 U/L and 213 ng/mL on POD 1, respectively. The serum creatinine level was normal, and renal function was not impaired (Fig. 1b)
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