Lobar atelectasis may be a complication of pulmonary exacerbations in cystic fibrosis (CF). There are no established guidelines on the management of this condition in patients with CF. Therapeutic bronchoscopy with recombinant human deoxyribonuclease (rhDNase) instillation has been described to be successful in patients not responding to conservative measures. We describe a case of a young man with CF, with previously mild impaired lung function, presenting with cough, desaturation, and worsening dyspnea, persisting for over 6 weeks, despite conservative therapy. Thoracic imaging showed right lower lobe atelectasis, which was successfully treated with bronchoscopy and instillation of rhDNase. Long-term resolution of the atelectasis was confirmed with chest magnetic resonance imaging follow-up.

A successful treatment of a lobar atelectasis in a patient with cystic fibrosis / V. Daccò, C.S. Sciarrabba, F. Corti, C. Rosazza, A. Malfitano, I. Borzani, C. Colombo. - In: PEDIATRIC PULMONOLOGY. - ISSN 8755-6863. - 57:11(2022 Nov), pp. 2868-2871. [10.1002/ppul.26094]

A successful treatment of a lobar atelectasis in a patient with cystic fibrosis

C.S. Sciarrabba
Secondo
;
F. Corti;C. Rosazza;A. Malfitano;I. Borzani
Penultimo
;
C. Colombo
Ultimo
2022

Abstract

Lobar atelectasis may be a complication of pulmonary exacerbations in cystic fibrosis (CF). There are no established guidelines on the management of this condition in patients with CF. Therapeutic bronchoscopy with recombinant human deoxyribonuclease (rhDNase) instillation has been described to be successful in patients not responding to conservative measures. We describe a case of a young man with CF, with previously mild impaired lung function, presenting with cough, desaturation, and worsening dyspnea, persisting for over 6 weeks, despite conservative therapy. Thoracic imaging showed right lower lobe atelectasis, which was successfully treated with bronchoscopy and instillation of rhDNase. Long-term resolution of the atelectasis was confirmed with chest magnetic resonance imaging follow-up.
atelectasis; chest MRI; cystic fibrosis; lung pathology; rhDNase; therapeutic bronchoscopy
Settore MED/38 - Pediatria Generale e Specialistica
nov-2022
Article (author)
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/2434/952997
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