AI Summary & Key Takeaways
AI InsightIn this case report, we describe the anesthetic management of a pediatric patient with Hurler syndrome who would be examined under general anaesthesia and we review the anaesthesia literature related to this disease.
📌 3 Key Takeaways
Abstract
The mucopolysaccharidoses are heterogeneous group of clinically progressive inherited disorders characterized by excessive storage of mucopolysaccharides. Hurler syndrome is the prototype of inherited mucopolysaccharidoses. For the anesthesiologists,the establishment and maintainence of airway is the most important problem in the cases with mucopolysaccharidoses. In this case report, we describe the anesthetic management of a pediatric patient with Hurler syndrome who would be examined under general anaesthesia and we review the anaesthesia literature related to this disease.
Özet
Mukopolisakkaridozlar, mukopolisakkaritlerin birikimi ile karakterize progresif seyreden kalıtsal, metabolik, heterojen bir grup hastalıktır. Hurler sendromu, kalıtsal, mukopolisakkaridozların prototipidir. Anestezistler için mukopolisakkaridozlu olgularda hava yolunun sağlanması ve sürdürülmesi en önemli problemdir. Bu vakada, genel anestezi altında muayene edilmesi gereken Hurler sendromlu pediatrik bir olguya sorunsuz anestezik yaklaşım, literatür bilgileri gözden geçirilerek tartışılmıştır.