Anesthesia management in a young child with aromatic l‐amino acid decarboxylase deficiency

Summary 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 p...

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Bibliographic Details
Published in:Pediatric anesthesia 2006-01, Vol.16 (1), p.82-84
Main Authors: VUTSKITS, LASZLO, MENACHE, CAROLINE, MANZANO, SERGIO, HAENGGELI, CHARLES‐ANDRÉ, HABRE, WALID
Format: Article
Language:English
Subjects:
Anesthesia
Anesthesia, General
Anesthesia. Intensive care medicine. Transfusions. Cell therapy and gene therapy
Anesthetics, Inhalation
aromatic l‐amino acid decarboxylase
Aromatic-L-Amino-Acid Decarboxylases - deficiency
autonomic dysregulation
Biological and medical sciences
catecholamine
Child, Preschool
Female
Humans
Infant
Medical sciences
Methyl Ethers
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Summary:Summary 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.
ISSN:1155-5645
1460-9592
DOI:10.1111/j.1460-9592.2005.01605.x