Anesthesia Management in a Patient Diagnosed with Mocupolysaccharidosis Type 1: A Case Report

Öz

Mucopolysaccharidoses (MPS) are inherited lysosomal storage diseases caused by the accumulation of glycosaminoglycans (GAG). Enzyme deficiencies involved in GAG degradation are seen in MPS. If GAGs are not broken down, they accumulate in the cell, eventually leading to cell, tissue and organ damage. In addition, the accumulation of GAG in the airway may create various difficulties for airway management of these patients. In this case report, we presented our anesthesia management of the 10 year old patient who had umbilical hernia and circumcision operation.

Anahtar Kelimeler

• Anesthesia
• intubation
• mucopolysaccharidoses.

Mokupolisakkaridoz Tip1 Tanılı Hastada Anestezi Yönetimi: Olgu Sunumu

Öz

Mukopolisakkaridozlar (MPS), glikoz amino glikanların (GAG) birikmesinden kaynaklanan kalıtsal lizozomal depo hastalıklarıdır. MPS’de GAG yıkımında görev alan enzimlerde eksiklik görülür. GAG'lar parçalanamazsa, hücrede birikerek sonunda hücre, doku ve organ hasarına yol açar. Ayrıca bu hastalarda havayolunda GAG birikimi havayolu yönetimi için çeşitli zorluklar oluşturabilir. Biz bu olgu sunumunda 10 yaşında umblikal herni ve sünnet operasyonu olan hastada anestezi yönetimimizi sunduk.

Anahtar Kelimeler

• Anestezi
• entübasyon
• mukopolisakkaridozlar.

Kaynakça

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