Elsevier

Respiratory Medicine

Volume 101, Issue 10, October 2007, Pages 2224-2226
Respiratory Medicine

Case Report
Long-term use of nebulized human recombinant DNase1 in two siblings with primary ciliary dyskinesia

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Summary

Primary ciliary dyskinesia (PCD) is characterized by ultra-structural defects of the cilia. In this report, we describe the long-term use of nebulized dornase alfa in two siblings with PCD. This is the first report of long-term use of dornase alfa in PCD.

KEYWORDS

Primary ciliary dyskinesia
rhDNase (dornase alfa)
Pulmonary function testing

Abbreviations

PCD
Primary ciliary dyskinesia
rhDNase
Recombinant human DNase1 (dornase alfa)
CF
Cystic fibrosis
PFT
Pulmonary function testing
FEV1
Forced expiratory volume in 1 s
BAL
Bronchoalveolar lavage
DORV
Double outlet right ventricle

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