Anesthesia management in a young child with aromatic l-amino acid decarboxylase deficiency.
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.
Vutskits
Laszlo
L
Department of Anesthesiology, Pharmacology and Surgical Intensive Care, University Hospital of Geneva, Geneva, Switzerland. Laszlo.Vutskits@hcuge.ch
Menache
Caroline
C
Manzano
Sergio
S
Haenggeli
Charles-André
CA
Habre
Walid
W
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
D018685:Anesthetics, Inhalation; D008738:Methyl Ethers; D000077149:Sevoflurane; D001142:Aromatic-L-Amino-Acid Decarboxylases