caa a22 4500 445374586 CHVBK 20180317142959.0 cr unu---uuuuu 170323e20110401xx s 000 0 eng 10.1007/s00540-010-1083-2 doi (NATIONALLICENCE)springer-10.1007/s00540-010-1083-2 The child with glutaric aciduria type I: anesthetic and perioperative management [Elektronische Daten] [Adelais Tsiotou, Anna Malisiova, Nikolaos Bouzelos, Dimitrios Velegrakis] Glutaric aciduria type I (GA-1) is an inborn error of metabolism caused by a deficiency of glutaryl-CoA dehydrogenase. It presents early in life, usually after an episode of fever, dehydration, infection or fasting, and results in metabolic decompensation and neurologic damage. We report the perioperative management of a 5-year-old boy admitted to the hospital for surgery because of neurogenic hip dislocation. Here we present the preoperative preparation, which focused on appropriate fluid administration and therapy intensification, as well as the safe anesthetic management with inhalation anesthesia and remifentanil, taking into consideration the mitochondrial basis of the disease. Furthermore, the role of postoperative care is emphasized in relation to stress response prophylaxis and the avoidance of complications related to the disorder. Japanese Society of Anesthesiologists, 2011 Glutaric aciduria type I nationallicence Organic aciduria nationallicence Mitochondrial disorders nationallicence Propofol infusion syndrome nationallicence Inhalation anesthesia nationallicence Tsiotou Adelais Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut Malisiova Anna Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut Bouzelos Nikolaos Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut Velegrakis Dimitrios Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut Journal of Anesthesia Springer Japan 25/2(2011-04-01), 301-304 0913-8668 25:2<301 2011 25 540 https://doi.org/10.1007/s00540-010-1083-2 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s00540-010-1083-2 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Tsiotou Adelais Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut NATIONALLICENCE 700 1- Malisiova Anna Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut NATIONALLICENCE 700 1- Bouzelos Nikolaos Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut NATIONALLICENCE 700 1- Velegrakis Dimitrios Anesthesiology Department, Children's Hospital "P. & A. Kyriakou”, Arkadias 19-21, 115 26, Athens, Greece aut NATIONALLICENCE 773 0- Journal of Anesthesia Springer Japan 25/2(2011-04-01), 301-304 0913-8668 25:2<301 2011 25 540 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer