Bir Mukopolisakkaridoz Tip II Olgusunda Zor Hava Yolu Yonetimi/Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II

Citation metadata

Authors: Damla Hanalioglu, Batuhan Yeke, Ahmet Ziya Birbilen, Serap Sivri and Ozlem Teksam
Date: Aug. 2019
From: Journal of Pediatric Emergency and Intensive Care Medicine(Vol. 6, Issue 2)
Publisher: Galenos Yayinevi Tic. Ltd.
Document Type: Article
Length: 1,767 words

Main content

Abstract :

Mukopolisakkaridoz Tip II (MPS II), X'e bagli resesif gecis gosteren nadir bir hastaliktir ve lizozomal iduronat-2-sulfataz enziminin eksikligi ile karakterizedir. Ilerleyici ust solunum yolu obstruksiyonu MPS II hastalari icin tipiktir. Hava yolu obstruksiyonunun giderilmesinde daha az invaziv yontemler yetersiz kaldiginda trakeostomi etkin bir yontemdir. Bu yazida, acil servisteki izlemi sirasinda akut solunum sikintisi gelisen ve endotrakeal entubasyon ve laringeal maske (LMA) ile hava yolu acikligi saglanamadigindan acil trakeostomi acilan bir MPS II olgusu sunulmustur. Anahtar Kelimeler: Mukopolisakkaridoz, Hunter sendromu, hava yolu guvenligi, trakeostomi 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

Source Citation

Source Citation
Hanalioglu, Damla, et al. "Bir Mukopolisakkaridoz Tip II Olgusunda Zor Hava Yolu Yonetimi/Management of Difficult Airway in a Patient with Mucopolysaccharidosis Type II." Journal of Pediatric Emergency and Intensive Care Medicine, vol. 6, no. 2, 2019, p. 113+. Accessed 20 Oct. 2020.
  

Gale Document Number: GALE|A590952122