title: The child with glutaric aciduria type I: anesthetic and perioperative management.
abstract: Glutaric aciduria type I (GA-1) is an inborn error of metabolism caused by a deficiency of glutaryl-CoA dehydrogenase. It presents early in life, usually after an episode of fever, dehydration, infection or fasting, and results in metabolic decompensation and neurologic damage. We report the perioperative management of a 5-year-old boy admitted to the hospital for surgery because of neurogenic hip dislocation. Here we present the preoperative preparation, which focused on appropriate fluid administration and therapy intensification, as well as the safe anesthetic management with inhalation anesthesia and remifentanil, taking into consideration the mitochondrial basis of the disease. Furthermore, the role of postoperative care is emphasized in relation to stress response prophylaxis and the avoidance of complications related to the disorder.
authors.affiliation: Anesthesiology Department, Children's Hospital P. & A. Kyriakou, Arkadias 19-21, 115 26 Athens, Greece. adeltsiotou@yahoo.gr
authors.forename: Adelais G
authors.initials: AG
authors.lastname: Tsiotou
authors.forename: Anna
authors.initials: A
authors.lastname: Malisiova
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Bouzelos
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Velegrakis
mesh_terms: D000592:Amino Acid Metabolism, Inborn Errors / Q000150:complications; D000758:Anesthesia / Q000379:methods*; D001928:Brain Diseases, Metabolic / Q000150:complications; D002675:Child, Preschool; D050770:Glutaryl-CoA Dehydrogenase / Q000172:deficiency; D006617:Hip Dislocation / Q000601:surgery; D006801:Humans; D008297:Male; D011182:Postoperative Care; D011300:Preoperative Care
chemical_list: D050770:Glutaryl-CoA Dehydrogenase