Hurler Sendromlu Olguda Magnetik Rezonans Görüntüleme Sırasında Anestezi Yaklaşımımız
Yükleniyor...
Dosyalar
Tarih
2004
Yazarlar
Dergi Başlığı
Dergi ISSN
Cilt Başlığı
Yayıncı
İnönü Üniversitesi Tıp Fakültesi Dergisi
Erişim Hakkı
Attribution 3.0 United States
Özet
Bir mukopolisakkarit metabolizması bozukluğu olan Hurler Sendromu (HS) α-L iduronidase enzim defektinden
kaynaklanan, birden fazla doku ve organı etkileyen kalıtsal, konjenital bir hastalıktır.
Sendromun klinik seyrinde görülebilecek sorunların saptanması ya da tedavisi amacıyla uygulanacak işlemler
sırasında gereken anestezi uygulamalarında mevcut anomaliler nedeni ile ciddi komplikasyonlar yaşanmaktadır. Bu
sendromda havayolu güçlükleri en sık karşılaşılan sorundur. Kalp damar ve solunum sistemine ait diğer patolojiler
perioperatif mortaliteye neden olabilir.
Yazımızda Hurler Sendromu (Tip I Mukopolisakkaridoz) ön tanısı konulmuş olan 3 yaşındaki olguda manyetik
rezonans görüntüleme sırasında uyguladığımız anestezi yaklaşımımızı sunarak literatür bilgileri ışığında tartışmayı
amaçladık.
Hurler Syndrome is an inherited and congenital disease, disorder of mucopolysaccharide metabolism α-L iduronidase enzyme deficiencyis the causeof the disease which effects many organs and tissues. Present abnormalities accompany to the syndrome frequently cause serious complications during anesthesia which is performed for surgical or diagnostic approaches. One of the major problem for anesthesia is airway difficulty. Multiple factors are present in the mucopolysaccharidoses which make the airway management and trachael intubation potentially hazardous. Cardiovasculary and respiratory pathologies may be the cause of death. In this report we would like to share our experiences, discuss the anesthetic risks and the management of HS patients in the light of literature by presenting our anesthetic approaches towards a 3-year-old patient who is prediagnosed with Hurler Syndrome (Type I Mucopolysaccharidosis).
Hurler Syndrome is an inherited and congenital disease, disorder of mucopolysaccharide metabolism α-L iduronidase enzyme deficiencyis the causeof the disease which effects many organs and tissues. Present abnormalities accompany to the syndrome frequently cause serious complications during anesthesia which is performed for surgical or diagnostic approaches. One of the major problem for anesthesia is airway difficulty. Multiple factors are present in the mucopolysaccharidoses which make the airway management and trachael intubation potentially hazardous. Cardiovasculary and respiratory pathologies may be the cause of death. In this report we would like to share our experiences, discuss the anesthetic risks and the management of HS patients in the light of literature by presenting our anesthetic approaches towards a 3-year-old patient who is prediagnosed with Hurler Syndrome (Type I Mucopolysaccharidosis).
Açıklama
İnönü Üniversitesi Tıp Fakültesi Dergisi
11(4)259-263 (2004)
Anahtar Kelimeler
Hurler Sendromu, Anestezi, Manyetik rezonans görüntüleme, Entübasyon güçlüğü, Hurler Syndrome, Anaesthesia, Magnetic Rezonans Imaging (MRI), Intubation Difficulties
Kaynak
WoS Q Değeri
Scopus Q Değeri
Cilt
Sayı
Künye
Işık, Berrin ; Tekgül, Zeki Tuncel ;İnönü Üniversitesi Tıp Fakültesi Dergisi 11(4)259-263 (2004)
