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DNase treatment in primary ciliary dyskinesia—Assessment by nocturnal pulse oximetry
Author(s) -
Berge Maartje ten,
Brinkhorst Govert,
Kroon André A.,
de Jongste Johan C.
Publication year - 1999
Publication title -
pediatric pulmonology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.866
H-Index - 106
eISSN - 1099-0496
pISSN - 8755-6863
DOI - 10.1002/(sici)1099-0496(199901)27:1<59::aid-ppul11>3.0.co;2-d
Subject(s) - primary ciliary dyskinesia , medicine , pulse oximetry , nocturnal , anesthesia , bronchiectasis , lung
DNA from disintegrating neutrophils is thought to be an important determinant of the high viscosity of cystic fibrosis sputum. Recombinant human deoxyribonuclease (DNase), an enzyme which cleaves DNA, has proven to be effective in improving sputum clearance from the airways of patients with cystic fibrosis (CF). Inhaled DNase improves pulmonary function, reduces the frequency of respiratory tract infections and improves general well-being in CF. Primary ciliary dyskinesia (PCD), like CF, is a chronic lung disease in which sputum retention leads to lung damage. Impairment of mucociliary transport results in chronic sinusitis, chronic bronchitis, and subsequently bronchiectasis, and in about 50% to situs inversus. Currently, PCD patients are treated with chest physiotherapy, antibiotics, and bronchodilators. We present here an infant with PCD. His severe respiratory symptoms and hypoxemia did not improve, despite nebulized isotonic NaCl solution, physiotherapy, and aggressive antibiotic treatment. Immediately after starting nebulized DNase therapy the respiratory symptoms, lung function, and overnight oximetry improved. During two readmissions because of acute worsening of dyspnea, the baby again showed overnight improvements on treatment with DNase, antibiotics, and bronchodilators. Withholding DNase during an asymptomatic period caused no worsening. The therapeutic potential of nebulized DNase in PCD should be established by controlled trials.