Anesthetic and perioperative management in children with non-ketotic hyperglycinemia
Manejo anestésico en paciente pediátrico con hiperglicinemia no cetósica
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Abstract
We report a child with a diagnosis of non-ketotic hyperglycinemia (NKGH), a rare neurometabolic disease caused by a defect in the glycine cleavage system, encoded by the GLDC, GCSH, AMT and GCSL genes resulting in elevated synaptic glycine levels generating a prolonged agonist effect on N-methyl-D-aspartate (NMDA) receptors. It is associated with hypotonia, seizures and swallowing disorders, which will depend on the age at presentation. A literature review was conducted to tailor perioperative approach.
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Chiu C-F,Lin J-L, Lin J-J, Tseng M-H, Lo F-S, Chiang M-C. Nonketotic Hyperglycinemia of Infants in Taiwan. Pediatr Neonatol. 2016;57(5):420–6. doi: 10.1016/j.pedneo.2015.10.008 DOI: https://doi.org/10.1016/j.pedneo.2015.10.008
Ramirez N, Flynn JM, Casalduc F, Rodriguez S, Cornier AS, Carlo S. Musculoskeletal manifestations of neonatal nonketotic hyperglycinemia. J Child Orthop. 2012;6(3):199–203. doi: 10.1007/s11832-012-0407-1 DOI: https://doi.org/10.1007/s11832-012-0407-1
Genç Sel Ç, Kılıç M, Yüksel D, Aksoy A, Kasapkara ÇS, Ceylaner S, et al. Nonketotic hyperglycinemia: Clinical range and outcome of a rare neurometabolic disease in a single-center. Brain Dev. 2017;40(10):865–875. doi: 10.1016/j.braindev.2018.06.007 DOI: https://doi.org/10.1016/j.braindev.2018.06.007
Suzuki Y, Kure S, Oota M, Hino H, Fukuda M. Nonketotic Hyperglycinemia: Proposal of a Diagnostic and Treatment Strategy. Pediatr Neurol. 2010;43(3):221–4. doi: 10.1016/j.pediatrneurol.2010.04.018 DOI: https://doi.org/10.1016/j.pediatrneurol.2010.04.018
August DA, Nguyen HG. Nitrous oxide for glycine encephalopathy: Correspondence. Pediatr Anesth. 2010;20(10):974–6. doi: 10.1111/j.1460-9592.2010.03412.x DOI: https://doi.org/10.1111/j.1460-9592.2010.03412.x
Berrío Valencia MI, Martínez Ochoa CM, Durango Gómez HC. Complicaciones anestésicas en pacientes con errores innatos del metabolismo sometidos a cirugía no cardiaca. Rev Colomb Anestesiol. 2013;41(4):257–60. doi: 10.1016/j.rca.2013.05.010 DOI: https://doi.org/10.1016/j.rca.2013.05.010
Kure S. Two novel laboratory tests facilitating diagnosis of glycine encephalopathy (nonketotic hyperglycinemia). Brain Dev. 2011;33(9):753–7. doi: 10.1016/j.braindev.2011.03.001 DOI: https://doi.org/10.1016/j.braindev.2011.03.001
Liu C-M, Fan S-Z. Glycine encephalopathy and delayed emergence from anesthesia. Anesth Analg. 2006;103(6):1631. doi: 10.1213/01.ane.0000247193.72647.d7 DOI: https://doi.org/10.1213/01.ane.0000247193.72647.d7
Barker C, Jefferson P, Ball DR. Glycine Encephalopathy and Anesthesia: Anesth Analg. 2007;105(2):544. doi: 10.1213/01.ane.0000265701.64299.92 DOI: https://doi.org/10.1213/01.ane.0000265701.64299.92
Tobias J, Allee J. Perioperative care of a child with non-ketotic hyperglycinemia. Saudi J Anaesth. 2010;4(3):197-201. doi: 10.4103/1658-354X.71578 DOI: https://doi.org/10.4103/1658-354X.71578
Eizaga Rebollar R, García Palacios MV, Morales Guerrero J, Torres LM. Magnesium sulfate in pediatric anesthesia: the Super Adjuvant. Pediatr Anesth. 2017;27(5):480–489. doi: 10.1111/pan.13129 DOI: https://doi.org/10.1111/pan.13129