title: Anesthesia management in a young child with aromatic l-amino acid decarboxylase deficiency.
abstract: Aromatic l-amino acid decarboxylase (AADC) deficiency is characterized by an almost complete absence of sympathetic autoregulation, because of very low levels of circulating catecholamines. Here, we report the successful management of four consecutive anesthesia procedures in a young child presenting with AADC deficiency. Our experience suggests that, with appropriate anticipation of the potential autonomic disturbances, anesthesia, at least for minor surgical and diagnostic procedures, can be conducted safely in patients with AADC deficiency.
authors.affiliation: Department of Anesthesiology, Pharmacology and Surgical Intensive Care, University Hospital of Geneva, Geneva, Switzerland. Laszlo.Vutskits@hcuge.ch
authors.forename: Laszlo
authors.initials: L
authors.lastname: Vutskits
authors.forename: Caroline
authors.initials: C
authors.lastname: Menache
authors.forename: Sergio
authors.initials: S
authors.lastname: Manzano
authors.forename: Charles-André
authors.initials: CA
authors.lastname: Haenggeli
authors.forename: Walid
authors.initials: W
authors.lastname: Habre
mesh_terms: D000768:Anesthesia, General; D018685:Anesthetics, Inhalation; D001142:Aromatic-L-Amino-Acid Decarboxylases / Q000172:deficiency*; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008738:Methyl Ethers; D000077149:Sevoflurane
chemical_list: D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane; D001142:Aromatic-L-Amino-Acid Decarboxylases