caa a22 4500 475754905 CHVBK 20180406123535.0 cr unu---uuuuu 170329e20000801xx s 000 0 eng 10.1007/s002470000274 doi (NATIONALLICENCE)springer-10.1007/s002470000274 CT-guided radiolabelled aerosol studies for assessing pulmonary impairment in children with bronchiectasis [Elektronische Daten] [M. Pifferi, D. Caramella, C. Bartolozzi, M. Baldini, M. Di Mauro, A. M. Cangiotti] Objective. To determine whether CT-guided mucociliary clearance studies allow differentiation between bronchiectasis associated with primary ciliary dyskinesia (PCD) and those unrelated to congenital or genetically transmitted defects.¶Materials and methods. Fifteen children aged 4-18 years with a CT diagnosis of bronchiectasis were included in the study. Six had PCD, while in nine cases no congenital disorder was demonstrated.¶Results. CT showed bronchiectasis in 26 (29 %) of 90 lung regions. Radiolabelled aerosol studies were conducted globally for each lung and on the regions affected by bronchiectasis. Global half-time of activity (t1/2) values of patients with PCD were significantly higher (P < 0.001) than those with bronchiectasis unrelated to congenital disorders. Among the 26 lung regions in which CT demonstrated bronchiectasis, regional clearance was abnormal in 24 cases. Patients with PCD showed no statistically significant difference between regional and global t1/2 values. Patients with bronchiectasis unrelated to congenital disorders showed significantly higher regional t1/2 values in the affected regions with respect to the corresponding global pulmonary t1/2 (P < 0.06).¶Conclusions. The combination of morphological CT information with functional data concerning the clearance of radiolabelled aerosol adds to our understanding of pulmonary impairment in children with bronchiectasis. In particular, regional studies allow the recognition of different mucociliary clearance patterns in bronchiectasis associated with PCD and those unrelated to congenital or genetically transmitted defects. Springer-Verlag Berlin Heidelberg, 2000 Pifferi M. Department of Paediatrics, University of Pisa, Pisa, Italy, IT aut Caramella D. Diagnostic and Interventional Radiology, University of Pisa, Via Roma 67, 56100 Pisa, Italy, IT aut Bartolozzi C. Diagnostic and Interventional Radiology, University of Pisa, Via Roma 67, 56100 Pisa, Italy, IT aut Baldini M. Department of Paediatrics, University of Pisa, Pisa, Italy, IT aut Di Mauro M. CNR Institute of Clinical Physiology, Pisa aut Cangiotti A. M. Electron microscopy Unit, Institute of Human Morphology, Hospital Umberto I, University of Ancona, Ancona, Italy, IT aut https://doi.org/10.1007/s002470000274 text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s002470000274 text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Pifferi M. Department of Paediatrics, University of Pisa, Pisa, Italy, IT aut NATIONALLICENCE 700 1- Caramella D. Diagnostic and Interventional Radiology, University of Pisa, Via Roma 67, 56100 Pisa, Italy, IT aut NATIONALLICENCE 700 1- Bartolozzi C. Diagnostic and Interventional Radiology, University of Pisa, Via Roma 67, 56100 Pisa, Italy, IT aut NATIONALLICENCE 700 1- Baldini M. Department of Paediatrics, University of Pisa, Pisa, Italy, IT aut NATIONALLICENCE 700 1- Di Mauro M. CNR Institute of Clinical Physiology, Pisa aut NATIONALLICENCE 700 1- Cangiotti A. M. Electron microscopy Unit, Institute of Human Morphology, Hospital Umberto I, University of Ancona, Ancona, Italy, IT aut Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer