Ryoko Owaki-Nakano1, Midoriko Higashi2, Kohei Iwashita1, Kenji Shigematsu1, Emiko Toyama1, Ken Yamaura3. 1. Department of Anesthesiology, Fukuoka University Hospital, 7-45-1 Nanakuma, Jonan-ku, Fukuoka, 814-0180, Japan. 2. Department of Anesthesiology and Critical Care Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan. higashi.midoriko.976@m.kyushu-u.ac.jp. 3. Department of Anesthesiology and Critical Care Medicine, Graduate School of Medical Sciences, Kyushu University, 3-1-1 Maidashi, Higashi-ku, Fukuoka, 812-8582, Japan.
Abstract
BACKGROUND: Glutaric 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 PRESENTATION: A 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. CONCLUSIONS: To avoid catabolism in patients with glutaric acidemia, appropriate glucose administration is important, depending on the surgical risk.
BACKGROUND:Glutaric 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 PRESENTATION: A 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. CONCLUSIONS: To avoid catabolism in patients with glutaric acidemia, appropriate glucose administration is important, depending on the surgical risk.
Authors: P Vellekoop; E F Diekman; I van Tuijl; M M C de Vries; P M van Hasselt; G Visser Journal: Mol Genet Metab Date: 2011-01-27 Impact factor: 4.797
Authors: Hidde H Huidekoper; Mariëtte T Ackermans; An F C Ruiter; Hans P Sauerwein; Frits A Wijburg Journal: Arch Dis Child Date: 2014-07-04 Impact factor: 3.791
Authors: Georgianne L Arnold; Johan Van Hove; Debra Freedenberg; Arnold Strauss; Nicola Longo; Barbara Burton; Cheryl Garganta; Can Ficicioglu; Stephen Cederbaum; Cary Harding; Richard G Boles; Dietrich Matern; Pranesh Chakraborty; Annette Feigenbaum Journal: Mol Genet Metab Date: 2009-01-20 Impact factor: 4.797
Authors: M M Welsink-Karssies; J A W Polderman; E J Nieveen van Dijkum; B Preckel; W S Schlack; G Visser; C E Hollak; J Hermanides Journal: JIMD Rep Date: 2016-08-13
Authors: A Dernoncourt; J Bouchereau; C Acquaviva-Bourdain; C Wicker; P De Lonlay; C Gourguechon; H Sevestre; P-E Merle; J Maizel; C Brault Journal: Case Rep Crit Care Date: 2019-12-21