Related Experiment Video

Updated: Jun 26, 2026

Rapid Viscoelastic Characterization of Airway Mucus Using a Benchtop Rheometer
08:47

Rapid Viscoelastic Characterization of Airway Mucus Using a Benchtop Rheometer

Published on: April 21, 2022

Airway management in children with mucopolysaccharidoses

Andrea H Yeung1, Morton J Cowan, Biljana Horn

  • 1Department of Otolaryngology-Head and Neck Surgery, University of California, San Francisco, 400 Parnassus Ave, A730, San Francisco, CA 94143-0342, USA. ayeung@ohns.ucsf.edu

Archives of Otolaryngology--Head & Neck Surgery
|January 21, 2009
PubMed

Insights

Children with mucopolysaccharidoses (MPSs) often experience airway obstruction. Bone marrow transplantation can significantly improve airway disease symptoms in these patients, highlighting its potential to alter the natural history of MPS-related complications.

Area of Science:

  • Pediatric Otolaryngology
  • Genetics and Rare Diseases
  • Respiratory Medicine

Background:

  • Mucopolysaccharidoses (MPSs) are rare, inherited metabolic disorders.
  • Excessive glycosaminoglycan accumulation in MPSs leads to progressive multi-systemic disease.
  • Airway compromise is a significant and potentially life-threatening complication in pediatric MPS patients.

Purpose of the Study:

  • To investigate the natural history of airway disease in children diagnosed with MPS.
  • To evaluate the impact of interventions, specifically bone marrow transplantation (BMT), on airway disease progression.

Main Methods:

  • Retrospective medical chart review of 27 pediatric patients with MPS.
  • Analysis of airway disease progression and outcomes over a 20-year period (1984-2004).
  • Assessment of symptoms before and after BMT in eligible patients.

Main Results:

  • Seventy percent (19/27) of patients presented with clinically significant upper airway obstruction.
  • Three patients (11%) required tracheotomy due to severe obstruction.
  • Bone marrow transplantation in 14 patients led to significant improvement in obstructive symptoms in 13 (93%) post-engraftment.

Conclusions:

  • Otolaryngologists must be vigilant for airway complications, including sleep apnea and obstruction, in children with MPS.
  • Successful bone marrow engraftment demonstrates potential to favorably alter the natural course of airway disease in MPS.
  • Early intervention with BMT may offer substantial symptomatic relief and improve long-term outcomes for airway disease in pediatric MPS.
Abstract

More Related Videos

Mucin Agarose Gel Electrophoresis: Western Blotting for High-molecular-weight Glycoproteins09:24

Mucin Agarose Gel Electrophoresis: Western Blotting for High-molecular-weight Glycoproteins

Published on: June 14, 2016

Related Concept Videos

Cystic Fibrosis: Management01:24

Cystic Fibrosis: Management

Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic sinusitis...
Drugs Used in Upper Respiratory Disorders: Overview01:16

Drugs Used in Upper Respiratory Disorders: Overview

Upper respiratory tract disorders, including viral infections and allergic rhinitis, cause significant discomfort and disrupt daily life. Managing these conditions involves a variety of drugs, such as antihistamines, intranasal steroids, decongestants, antitussives, expectorants, and mucolytics. Specific examples of drugs in each category are provided.
Antihistamines (e.g., Benadryl) block histamines from binding. Histamines are chemicals released during an allergic reaction in the body. As a...
Upper Respiratory Drugs: Antitussives, Expectorants, and Mucolytics01:23

Upper Respiratory Drugs: Antitussives, Expectorants, and Mucolytics

Respiratory symptoms, such as congestion and cough, commonly accompany respiratory tract conditions. Various medications, such as antitussives, expectorants, and mucolytics, play crucial roles in providing relief.
Antitussives include codeine, dextromethorphan (Robitussin), and benzonatate (Tessalon). Codeine and dextromethorphan exert their effects centrally by suppressing the cough reflex center in the medulla.  Benzonatate operates peripherally within the respiratory tract by anesthetizing...
Cardiopulmonary Resuscitation II: ACLS Airway Management01:22

Cardiopulmonary Resuscitation II: ACLS Airway Management

Airway management is a key skill in emergency and critical care settings, as maintaining a clear airway is essential for adequate oxygenation and ventilation.Head Tilt-Chin Lift TechniqueThe head tilt-chin lift maneuver is an essential technique primarily used in patients without suspected cervical spine injuries. To perform this maneuver, one hand is placed on the patient’s forehead, and gentle pressure is applied backward to tilt the head. The fingertips of the other hand are positioned under...
Cardiopulmonary Resuscitation V: Advanced Airway Management Techniques01:30

Cardiopulmonary Resuscitation V: Advanced Airway Management Techniques

Airway management is essential in emergency and surgical medicine, ensuring ventilation and oxygenation in patients who cannot maintain their own airway. Clinicians use a range of techniques and devices to secure the airway, depending on the patient’s condition and the clinical context. Key methods include endotracheal intubation, rapid sequence intubation (RSI), supraglottic airway devices, and advanced visualization aids. In cases where these approaches fail, surgical airway interventions are...
Pneumonia V: Nursing management and Prevention01:30

Pneumonia V: Nursing management and Prevention

Nursing management of pneumonia involves promoting airway patency, facilitating rest and conserving energy, encouraging fluid intake, maintaining nutrition, and educating patients.
The nurse must practice strict medical asepsis and adhere to infection control guidelines to minimize healthcare-associated infections.
Enhance airway patency
Position the patient correctly to facilitate drainage of the affected lung segments. Manual or mechanical percussion and vibration can also be employed.