MELAS sendromlu toksik megakolonlu olguda anestezi yönetimi / Anestheshetic management in a patient with MELAS syndrome receiving the diagnosis of toxic megacolon
Yükleniyor...
Tarih
2018
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
Maltepe Tıp Dergisi
Erişim Hakkı
Attribution-NonCommercial-NoDerivs 3.0 United States
info:eu-repo/semantics/openAccess
info:eu-repo/semantics/openAccess
Özet
MELAS sendromu; mitokondrial düzeyde nokta mutasyon sonucu gelişen, mitokondrial miyopati, laktik asidoz, ensefalopati ve inme benzeri epizotlarla karakterize progresif nörodejeneratif genetik bir hastalıktır. Yüksek morbidite ve mortaliteye sahip olan bu sendrom; özellikle beyin, kardiyovasküler gastrointestinal ve nöromuskuler sistem olmak üzere tüm organları etkiler. Metabolik asidoz, hipotermi, malign hipertermiye yatkınlık, kas gevşeticilere karşı artmış direnç ve uzamış etkiden dolayı bu hastalarda uygulanacak anestezi yöntemi, monitörizasyon, ilaç ve sıvı rejimi anestezistler açısından önem arz eder. Bu yazıda toksik megakolon tanısı ile acil hemikolektomi geçiren MELAS sendromlu bir olguda uyguladığımız anestezi yönetimini sunulmuştur.
MELAS syndrome is a progressive, neurodegenerative genetic disorder characterized by point mutations in the mitochondrial DNA, mitochondrial myopathy, lactic acidosis, encephalopathy, and stroke-like episodes. The syndrome has high morbidity and mortality, affecting all the organs, especially the brain, cardiovascular, gastrointestinal and neuromuscular systems. Due to metabolic acidosis, tendency to hypothermia and to malign hyperthermia, increased resistance to muscle relaxants as well as prolonged effects, the method for anesthetic management, monitorization, medication and fluid regimes are of crucial significance for anesthesiologists in these patients. In this article, we present anesthetic management performed on a patient with MELAS syndrome who had received the diagnosis of toxic megacolon and underwent emergency hemicolectomy.
MELAS syndrome is a progressive, neurodegenerative genetic disorder characterized by point mutations in the mitochondrial DNA, mitochondrial myopathy, lactic acidosis, encephalopathy, and stroke-like episodes. The syndrome has high morbidity and mortality, affecting all the organs, especially the brain, cardiovascular, gastrointestinal and neuromuscular systems. Due to metabolic acidosis, tendency to hypothermia and to malign hyperthermia, increased resistance to muscle relaxants as well as prolonged effects, the method for anesthetic management, monitorization, medication and fluid regimes are of crucial significance for anesthesiologists in these patients. In this article, we present anesthetic management performed on a patient with MELAS syndrome who had received the diagnosis of toxic megacolon and underwent emergency hemicolectomy.
Açıklama
Anahtar Kelimeler
MELAS sendromu, toksik megakolon, anestezi yönetimi, MELAS syndrome, toxic megacolon, anesthetic management
Kaynak
Maltepe Tıp Dergisi
WoS Q Değeri
Scopus Q Değeri
Cilt
10
Sayı
2
Künye
Örki, T., Kurul Özcan, L. ve Avan, D. (2018). MELAS sendromlu toksik megakolonlu olguda anestezi yönetimi / Anestheshetic management in a patient with MELAS syndrome receiving the diagnosis of toxic megacolon. Maltepe Tıp Dergisi. 10(2), s. 58-61.