Severe tracheal and bronchial collapse in adults with type II mucopolysaccharidosis.
Rutten M, Ciet P, van den Biggelaar R, Oussoren E, Langendonk JG, van der Ploeg AT, Langeveld M
Orphanet journal of rare diseases, 2016 Apr 26
Abstract
Mucopolysaccharidosis type II (MPSII) patients frequently suffer from dyspnoea caused by restrictive airway disease due to skeletal abnormalities as well as glycosaminoglycans (GAG) accumulation at different levels of the airway, including the trachea. In this study we describe the extent of the tracheal and bronchial narrowing, the changes in airway diameter during respiration and the effects of these obstructions on respiratory function in adult MPSII patients.