title: [Anesthetic management of an infant with Menkes disease].
abstract: Menkes disease is a rare, sex-linked recessive disorder characterized by kinky hair, convulsion, mental retardation, bone and connective tissue lesions, and hypothermia. These symptoms have been attributed to suppression of copper-dependent enzymes resulting from copper deficiency. We report a case of a 7-month-old infant with Menkes disease who underwent repair of inguinal hernia. Anesthesia was maintained with sevoflurane-N2O-O2, and the operation was carried out uneventfully. Although the patient had been medicated with anticonvulsants preoperatively, transient seizure occurred in the recovery room. We also discuss pathophysiology and anesthetic management of a patient with Menkes disease.
authors.affiliation: Department of Anesthesia, Kyoto University Hospital.
authors.forename: M
authors.initials: M
authors.lastname: Sugimoto
authors.forename: K
authors.initials: K
authors.lastname: Shindo
authors.forename: K
authors.initials: K
authors.lastname: Shingu
authors.forename: K
authors.initials: K
authors.lastname: Mori
mesh_terms: D000769:Anesthesia, Inhalation; D000775:Anesthesia, Spinal; D000927:Anticonvulsants / Q000008:administration & dosage; D002648:Child; D003300:Copper / Q000378:metabolism; D006552:Hernia, Inguinal / Q000150:complications / Q000601:surgery*; D006801:Humans; D008297:Male; D007706:Menkes Kinky Hair Syndrome / Q000150:complications / Q000378:metabolism; D011292:Premedication
chemical_list: D000927:Anticonvulsants; D003300:Copper