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Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report

Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. In addition, patients mig...

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Detalles Bibliográficos
Autores principales: Faris, Ali S, Tawfic, Qutaiba A, Jeyaraj, Leo
Formato: Online Artículo Texto
Lenguaje:English
Publicado: The Korean Society of Anesthesiologists 2014
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4216793/
https://www.ncbi.nlm.nih.gov/pubmed/25368789
http://dx.doi.org/10.4097/kjae.2014.67.4.283
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author Faris, Ali S
Tawfic, Qutaiba A
Jeyaraj, Leo
author_facet Faris, Ali S
Tawfic, Qutaiba A
Jeyaraj, Leo
author_sort Faris, Ali S
collection PubMed
description Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. In addition, patients might have cerebellar ataxia, a high content of protein in the cerebrospinal fluid, proximal myopathy, multiple endocrinopathies, and renal tubular acidosis. We herein report the successful obstetric analgesic and anesthetic management of a 28-year-old parturient patient with KSS who required labor analgesia and proceeded to deliver by cesarean section. We extrapolate that regional analgesia/anesthesia might be beneficial for reducing the metabolic demands associated with the stress and pain of labor in patients with KSS. Efficient postoperative analgesia should be provided to decrease oxygen requirements.
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spelling pubmed-42167932014-11-03 Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report Faris, Ali S Tawfic, Qutaiba A Jeyaraj, Leo Korean J Anesthesiol Case Report Kearns-Sayre syndrome (KSS) is a rare mitochondrial myopathy that usually develops before 20 years of age. It demonstrates multisystemic involvement with a triad of cardinal features: progressive ophthalmoplegia, pigmentary retinopathy, and cardiac conduction abnormalities. In addition, patients might have cerebellar ataxia, a high content of protein in the cerebrospinal fluid, proximal myopathy, multiple endocrinopathies, and renal tubular acidosis. We herein report the successful obstetric analgesic and anesthetic management of a 28-year-old parturient patient with KSS who required labor analgesia and proceeded to deliver by cesarean section. We extrapolate that regional analgesia/anesthesia might be beneficial for reducing the metabolic demands associated with the stress and pain of labor in patients with KSS. Efficient postoperative analgesia should be provided to decrease oxygen requirements. The Korean Society of Anesthesiologists 2014-10 2014-10-27 /pmc/articles/PMC4216793/ /pubmed/25368789 http://dx.doi.org/10.4097/kjae.2014.67.4.283 Text en Copyright © the Korean Society of Anesthesiologists, 2014 http://creativecommons.org/licenses/by-nc/3.0/ This is an open-access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0/), which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle Case Report
Faris, Ali S
Tawfic, Qutaiba A
Jeyaraj, Leo
Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_full Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_fullStr Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_full_unstemmed Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_short Obstetric anesthesia considerations in Kearns-Sayre syndrome: a case report
title_sort obstetric anesthesia considerations in kearns-sayre syndrome: a case report
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC4216793/
https://www.ncbi.nlm.nih.gov/pubmed/25368789
http://dx.doi.org/10.4097/kjae.2014.67.4.283
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