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    Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II
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    Case Report
    VOLUME: 6 ISSUE: 2
    P: 113-116
    August 2019

    Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II

    J Pediatr Emerg Intensive Care Med 2019;6(2):113-116
    DOI: 10.4274/cayd.galenos.2018.22931
    Damla Hanalioğlu 1
    Batuhan Yeke 2
    Ahmet Ziya Birbilen 2
    Serap Sivri 3
    Özlem Tekşam 2
    1. Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Çocuk Acil Bilim Dalı, Ankara, Türkiye
    2. Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Ankara, Türkiye
    3. Hacettepe Üniversitesi Tıp Fakültesi, Çocuk Sağlığı ve Hastalıkları Anabilim Dalı, Metabolizma ve Beslenme Ünitesi, Ankara, Türkiye
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    Received Date: 22.02.2018
    Accepted Date: 15.05.2018
    Publish Date: 17.06.2019
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    ABSTRACT

    Mucopolysaccharidosis type II (MPS II) is a rare, X-linked recessive disease characterized by deficiency of lysosomal iduronate-2-sulfatase. Progressive upper airway obstruction is common in patients with MPS II. Tracheostomy is an effective way of managing airway obstruction when less invasive interventions are no longer adequate. Here, we report a patient with MPS II who developed sudden respiratory distress in the emergency department and required tracheostomy as endotracheal intubation and laryngeal mask airway (LMA) failed to secure the airway.

    Keywords: Mucopolysaccharidosis, Hunter syndrome, airway, tracheostomy

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