1. Tracheobronchial stents in mucopolysaccharidosis.
- Author
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Karl, Reiter, Carola, Schoen, Regina, Ensenauer, Thomas, Nicolai, and Huber, Rudolf M.
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MUCOPOLYSACCHARIDOSIS treatment , *SURGICAL stents , *TRACHEAL surgery , *GENETIC disorders , *GLYCOSAMINOGLYCANS , *STENOSIS , *RESPIRATORY insufficiency - Abstract
Introduction The mucopolysaccharidoses are a group of hereditary disorders pathologically characterized by tissue accumulation of glycosaminoglycans due to deficient lysosomal metabolism which often leads to progressive airway stenosis and respiratory insufficiency. Relentless and treatment-refractory narrowing of the lower airways with ensuing severe limitation of quality of life is a challenging problem in mucopolysaccharidoses. Case reports We report 2 cases of MPS (Hunter's and Maroteaux-Lamy's syndrome resp.) in whom tracheal stents were placed to relieve severe tracheal obstruction. The first patient could be weaned from mechanical ventilation after stent placement but showed significant long-term stent-related morbidity. The second patient suffered a severe procedure-related complication due to positioning problems typical for MPS. Conclusions Very good short-term success can be achieved with airway stent placement in patients with MPS and severe lower airway stenosis but a high risk of severe complications and important long-term morbidity have to be weighed against potential individual benefit. [ABSTRACT FROM AUTHOR]
- Published
- 2016
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