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Bronchoscopically administered recombinant human DNase for lobar atelectasis in cystic fibrosis
D M Slattery1, D A Waltz, B Denham
1Our Lady's Hospital for Sick Children, Crumlin, Dublin, Ireland.
Insights
Bronchoscopic instillation of human recombinant DNase (rhDNase) can expand collapsed lung lobes in cystic fibrosis patients resistant to standard treatments. This approach offers a new option for persistent lobar atelectasis, improving outcomes.
Area of Science:
- Pulmonology
- Biochemistry
- Pediatrics
Background:
- Lobar atelectasis is a frequent complication in cystic fibrosis (CF).
- Standard treatments include antibiotics and chest physiotherapy.
- Persistent atelectasis in pediatric CF patients indicates a poor prognosis.
Observation:
- A subgroup of CF patients exhibits atelectasis resistant to medical therapy.
- Bronchoscopic instillation of human recombinant DNase (rhDNase) was explored in these resistant cases.
- Previous studies showed rhDNase success in other conditions with viscous secretions.
Findings:
- Bronchoscopic rhDNase successfully expanded atelectatic lobes in three pediatric CF patients.
- These patients had been resistant to at least two weeks of medical therapy.
- CF sputum's high DNA content contributes to its viscosity; rhDNase breaks down DNA, improving fluidity.
Implications:
- Bronchoscopic rhDNase instillation is a viable treatment for refractory lobar atelectasis in CF.
- This method may prevent the need for more invasive procedures.
- Further research should evaluate rhDNase efficacy and safety in larger CF cohorts.
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.