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Use of Dexmedetomidine for Magnetic Resonance Imaging under Sedation in a Pediatric Patient with Phenylketonuria
Phenylketonuria (PKU) is an inborn error of metabolism caused by a deficiency of the enzyme phenylalanine hydroxylase which results in accumulation of phenylalanine. Patients of PKU presents with seizures, mental retardation, and organ damage and possess a unique challenge to the anesthesiologists when they need anesthetics for diagnostic or surgical procedures. There is limited literature regarding the safety of various anesthetic drugs in PKU patients. None of them reported the use of dexmedetomidine as safer sedative option for such patients. Therefore, we describe the management of such a case posted for magnetic resonance imaging under dexmedetomidine sedation.
Article published online:
19 November 2020
© 2020. Indian Society of Neuroanaesthesiology and Critical Care. This is an open access article published by Thieme under the terms of the Creative Commons Attribution-NonDerivative-NonCommercial-License, permitting copying and reproduction so long as the original work is given appropriate credit. Contents may not be used for commercial purposes, or adapted, remixed, transformed or built upon. (https://creativecommons.org/licenses/by-nc-nd/4.0/).
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- 1 Das SK. Inborn errors of metabolism: challenges and management. Indian J Clin Biochem 2013; 28 (04) 311-313
- 2 Walter JH, Lachmann RH, Burgard P. Hyperphenylalaninaemia. In: Saudubray JM, Van den Berghe G, Walter JH. eds. Inborn Metabolic Diseases. Berlin: Springer; 2012: 251-64
- 3 Vockley J, Andersson HC, Antshel KM. et al. American College of Medical Genetics and Genomics Therapeutics Committee. Phenylalanine hydroxylase deficiency: diagnosis and management guideline. Genet Med 2014; 16 (02) 188-200
- 4 Kaur M, Das GP, Verma IC. Inborn errors of amino acid metabolism in north India. J Inherit Metab Dis 1994; 17 (02) 230-233
- 5 Verma IC. Genetic disorders in India. In: Verma IC. ed. Medical Genetics in India. 1. Pondicherry: Auroma Enterprises; 1978: 5-16
- 6 Guida M, Pesaresi I, Fabbri S, Sartucci F, Cosottini M, Giorgi FS. Epilepsy and phenylketonuria: a case description and EEG-fMRI findings. Funct Neurol 2014; 29 (01) 75-79
- 7 Stuart G, Ahmad N. Perioperative care of children with inherited metabolic disorders. Contin Educ Anaesth Crit Care Pain 2011; 11 (02) 62-68
- 8 Rayadurg V, Uttarwar A, Surve R. Is propofol safe in patients with phenylketonuria?. J Neurosurg Anesthesiol 2018; 30 (01) 85-86
- 9 Lerman J, Coté CJ, Steward DJ. Anesthesia Implications of Syndromes and Unusual Disorders. In: eds. Manual of Pediatric Anesthesia. Switzerland: Springer; 2016: 523-658
- 10 Mason KP, Zurakowski D, Zgleszewski SE. et al. High dose dexmedetomidine as the sole sedative for pediatric MRI. Paediatr Anaesth 2008; 18 (05) 403-411
- 11 Berkenbosch JW, Wankum PC, Tobias JD. Prospective evaluation of dexmedetomidine for noninvasive procedural sedation in children. Pediatr Crit Care Med 2005; 6 (04) 435-439
- 12 Kurdi MS, Theerth KA, Deva RS. Ketamine: current applications in anesthesia, pain, and critical care. Anesth Essays Res 2014; 8 (03) 283-290
- 13 Bouillon T, Bruhn J, Roepcke H, Hoeft A. Opioid-induced respiratory depression is associated with increased tidal volume variability. Eur J Anaesthesiol 2003; 20 (02) 127-133