title: Bronchoscopically administered recombinant human DNase for lobar atelectasis in cystic fibrosis.
abstract: Lobar atelectasis is a common complication of cystic fibrosis. The majority of cases respond to intravenous antibiotics and chest physiotherapy. In a subgroup of patients, atelectasis is resistant to medical therapy, and its persistence in the pediatric population is associated with a poor prognosis. Bronchoscopic instillation of human recombinant DNase expanded atelectatic lobes in three children resistant to at least 2 weeks of medical therapy. This method of administration of DNase has been successful in resistant cases of lobar atelectasis in patients with chronic obstructive pulmonary disease, quadriplegia, and status asthmaticus. Purulent cystic fibrosis sputum has a very high DNA content, and DNA has been shown to become more pourable in vitro when treated with rhDNase. Bronchoscopic instillation of rhDNase should be considered in cases of persistent lobar atelectasis unresponsive to medical therapy.
authors.affiliation: Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
authors.forename: D M
authors.initials: DM
authors.lastname: Slattery
authors.forename: D A
authors.initials: DA
authors.lastname: Waltz
authors.forename: B
authors.initials: B
authors.lastname: Denham
authors.forename: M
authors.initials: M
authors.lastname: O'Mahony
authors.forename: P
authors.initials: P
authors.lastname: Greally
mesh_terms: D001999:Bronchoscopy; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications* / Q000000981:diagnostic imaging; D003850:Deoxyribonuclease I / Q000008:administration & dosage* / Q000627:therapeutic use*; D005100:Expectorants / Q000008:administration & dosage* / Q000627:therapeutic use*; D005260:Female; D006801:Humans; D007322:Instillation, Drug; D001261:Pulmonary Atelectasis / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000209:etiology*; D011859:Radiography; D011994:Recombinant Proteins / Q000008:administration & dosage* / Q000627:therapeutic use*
chemical_list: D005100:Expectorants; D011994:Recombinant Proteins; C085693:DNASE1 protein, human; D003850:Deoxyribonuclease I