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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Veröffentlicht in:Pediatric anesthesia 2006-01, Vol.16 (1), p.82-84
Hauptverfasser: VUTSKITS, LASZLO, MENACHE, CAROLINE, MANZANO, SERGIO, HAENGGELI, CHARLES‐ANDRÉ, HABRE, WALID
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container_end_page 84
container_issue 1
container_start_page 82
container_title Pediatric anesthesia
container_volume 16
creator VUTSKITS, LASZLO
MENACHE, CAROLINE
MANZANO, SERGIO
HAENGGELI, CHARLES‐ANDRÉ
HABRE, WALID
description 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.
doi_str_mv 10.1111/j.1460-9592.2005.01605.x
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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
title Anesthesia management in a young child with aromatic l‐amino acid decarboxylase deficiency
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