Organização: Sociedade Portuguesa de Doenças Metabólicas-SPDM
Temática: Metabolic Diseases - Anaesthesia in Patients with Mucopolysaccharidosis and their Respiratory function
Resumo: The mucopolysaccharidoses (MPS) are a group of metabolic inherited lysosomal storage disorders resulting in the accumulation of glycosaminoglycans (GAGs) in organs and tissues throughout the body. Clinical manifestations include skeletal deformities, spinal cord compression, reduced growth, coarse facial features, intellectual and neurological impairment, cardiac and respiratory problems, etc. In many patients with MPS, GAGs also accumulate in the laryngeal, tracheal and bronchial tissues and this can result in (upper) airway obstruction and impaired respiratory function, and often have excessive tracheobronchial secretions and suffer from frequent upper (ear and nose) respiratory tract infections and pneumonia. Respiratory failure may also be a cause of (premature) death. Patients with MPS frequently require surgery to manage skeletal deformities, spinal cord compression, ear, nose and throat problems, hernia repair and to replace cardiac valves. However, impaired respiratory function and complications such as laryngeal deformities, large tongue, short neck, or instability can complicate endotracheal intubation and anaesthesia and require special measures and attention. This meeting will bring experts in pulmonology and anaesthesiology together with experts in MPS disease to discuss ideas to improve and better understand the underlying pathophysiology of the upper and lower respiratory tract and the associated anaesthesia considerations in patients with MPS, in order to increase awareness of the potential risks and plan a better care for this group of patients.
Programa: (389Kb)
Comunic. Livres: Sim
Envio de Resumos: 26/2/2014
Secretariado: spdm2014@leading.pt
Site de Internet: www.spdmsymposium2014.com
Outras Informações: Registration FREE for satellite meeting only:
http://www.spdmsymposium2014.com/en/content/satellite-symposium/registration/registration.html
Registration to SPDM meeting 20&21Mar has fees.