Related Experiment Video
Updated: Dec 24, 2025

Flow Cytometric Isolation of Primary Murine Type II Alveolar Epithelial Cells for Functional and Molecular Studies
Published on: December 26, 2012
Tracheal and lower airway changes in a patient with mucolipidosis type II
Thomas S Poore1, Jeremy Prager2, Jason P Weinman3
1Department of Pediatrics, Section of Pulmonology and Sleep Medicine, The Breathing Institute, Children's Hospital Colorado, University of Colorado School of Medicine, Aurora, Colorado.
Introduction:
Mucolipidosis type II (MLII) is a lysosomal storage disease causing systemic deposition of mucopolysaccharides. We describe imaging and bronchoscopy findings not previously reported in the literature in a child with MLII.
Case:
A 9-year-old with MLII s/p hematopoietic stem-cell transplant (HSCT), bronchiectasis, and aspiration presented with recurrent respiratory illnesses. Bronchoscopy and chest computed tomography were performed, showing a saber-sheath trachea with fixed narrowing and curvature.
Discussion:
This case describes potentially life-threatening airway distortion in MLII despite HSCT that cannot be ameliorated with tracheostomy. Etiology is unknown but likely due to abnormal deposition causing an immobile, stenotic airway and restricted thorax.
Related Concept Videos
Cystic Fibrosis: Pathogenesis
CF is primarily caused by a genetic mutation in a chromosome 7 gene coding for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. The most common gene mutation leading to CF is the ΔF508 mutation,...
Chronic Obstructive Pulmonary Disease-II: Pathophysiology
Chronic Inflammation
Cystic Fibrosis: Management
Sinus disease and chronic...
Drugs Used in Upper Respiratory Disorders: Overview
Antihistamines (e.g., Benadryl) block histamines from binding. Histamines are chemicals released during an allergic reaction in the body. As a...
Breathing
The Bronchial Tree
The trachea, commonly known as the windpipe, is a tube that connects the larynx (voice box) to the bronchi. At a point called the carina, it bifurcates into two primary bronchi. The right primary bronchus is wider, shorter, and more vertical than the left primary...

