Severe tracheal and bronchial collapse in adults with type II mucopolysaccharidosis.

ABSTRACT:

Background

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.

Methods

Five adult MPSII patients (mean age 40 years) were included. Pulmonary function tests and in- and expiratory chest CT scans were obtained. Cross-sectional areas of trachea and main bronchi were measured at end-inspiration and -expiration and percentage collapse was calculated.

Results

There

SUBMITTER: Rutten M 

PROVIDER: S-EPMC4845328 | biostudies-literature | 2016 Apr

REPOSITORIES: biostudies-literature