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Anesthesia management in a young child with aromatic l ‐amino acid decarboxylase deficiency
Author(s) -
VUTSKITS LASZLO,
MENACHE CAROLINE,
MANZANO SERGIO,
HAENGGELI CHARLESANDRÉ,
HABRE WALID
Publication year - 2006
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/j.1460-9592.2005.01605.x
Subject(s) - medicine , aromatic l amino acid decarboxylase , anticipation (artificial intelligence) , anesthesia , glutamate decarboxylase , endocrinology , dopamine , biochemistry , enzyme , chemistry , artificial intelligence , computer science
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.