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Published on: June 11, 2017
Anesthesia management in a young child with aromatic l-amino acid decarboxylase deficiency
Laszlo Vutskits1, Caroline Menache, Sergio Manzano
1Department of Anesthesiology, Pharmacology and Surgical Intensive Care, University Hospital of Geneva, Geneva, Switzerland. Laszlo.Vutskits@hcuge.ch
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.
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