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Upper airways abnormalities and tracheal problems in Morquio's disease
P P Walker1, E Rose, J G Williams
1Department of Respiratory Medicine, University Hospital Aintree, Liverpool, UK. paulpwalker@hotmail.com
Thorax
|May 3, 2003
Summary
Morquio disease, a metabolic disorder, causes respiratory issues. Adult patients often need specialist respiratory care due to airway problems and potential respiratory failure, especially with advanced home ventilation.
Area of Science:
- Metabolic Disorders
- Respiratory Medicine
- Genetics
Background:
- Morquio disease is a rare inherited metabolic disorder.
- It leads to progressive skeletal abnormalities and systemic complications.
- Respiratory compromise is a significant concern in affected individuals.
Observation:
- Adult patients with Morquio disease frequently develop upper airway obstruction.
- Respiratory failure can occur, necessitating advanced respiratory support.
- Referral to specialized respiratory units is increasing due to home ventilation advancements.
Findings:
- The study details the respiratory management of two adult patients with Morquio disease.
- It highlights the challenges in managing complex airway issues.
- Experiences underscore the need for multidisciplinary respiratory care.
Implications:
- Improved understanding of respiratory complications in Morquio disease.
- Guidance for managing adult patients with this rare condition.
- Highlights the role of specialist respiratory units in managing complex metabolic disorders.