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Hurler Sendromlu Olguda Magnetik Rezonans Görüntüleme Sırasında Anestezi Yaklaşımımız

Anaesthetic Aproach Towards a Patient with Hurler Syndrom in the Magnetic Resonans Imaging Area

Journal Name:

  • İnönü Üniversitesi Tıp Fakültesi Dergisi

Publication Year:

  • 2004

Key Words:

Keywords (Original Language):

Author NameUniversity of AuthorFaculty of Author
Abstract (2. Language): 
Hurler Syndrome is an inherited and congenital disease, disorder of mucopolysaccharide metabolism a-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).
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Abstract (Original Language): 
Bir mukopolisakkarit metabolizması bozukluğu olan Hurler Sendromu (HS) a-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.
FULL TEXT (PDF): 
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  • 4
259-263
  • Turkish

REFERENCES

References: 

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u Olguda Magnetik Rezonans Görüntüleme Sırasında Anestezi Yaklaşımımız
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