Journal of Kermanshah University of Medical Sciences
Peer-reviewed Medical Quarterly
e-ISSN 2588-2570
p-ISSN 2588-2562
Image Credit:J Kermanshah Univ Med Sci
Outline
Anesthesia in an 8-year old child with familial dysautonomia (Reilly-day syndrome)
Authors
1Dept. of Anesthesia, School of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran
2Dept. of Surgery, School of Medicine, Shahed University of Medical Sciences, Kermanshah, Iran
*Corresponding Author: Corresponding author: Parisa Golfam, Dept. of Anesthesia, School of Medicine, Kermanshah University of Medical Sciences, Kermanshah, Iran, Tel: +98 9181310864 Email: Pargolfam@yahoo.com
Published online:Sep 29, 2012
Article type:Case Report
Received:Sep 03, 2011
Accepted:Feb 21, 2012
How to Cite:Golfam P, Golfam F, Yari M. Anesthesia in an 8-year old child with familial dysautonomia (Reilly-day syndrome). J Kermanshah Univ Med Sci. 2012;16(3):e78804. doi:
Abstract
Background: Familial dysautonomia is a rare genetic disorder with autosomal recessive inheritance that has been mainly reported in the Ashkenazi Jewish population. Autonomic dysfunction, tendency to severe hyperpyrexia, vomiting and recurrent pulmonary aspiration, and pain conception disorder, make a patient an interesting candidate for anesthesia.
Case Report: The patient was an 8-year old girl underwent surgery due to osteomyelitis and foot ulceration. Minimal drug, including sufentanil and nitrous oxide were used. Vital signs remained stable in the patient during the entire procedure.
Conclusion: Careful planning of the anesthetic management, preoperative hydration, understanding the physiological consequences, and being able to titrate the medications utilized are keys to decreasing complications encountered in these kinds of patients.
Highlights
References
- 1.The references of this article is available on PDF.
Copyright
© 2012, Journal of Kermanshah University of Medical Sciences. This open-access article is available under the Creative Commons Attribution-NonCommercial 4.0 (CC BY-NC 4.0) International License (https://creativecommons.org/licenses/by-nc/4.0/), which allows for the copying and redistribution of the material only for noncommercial purposes, provided that the original work is properly cited.
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