Progressive unilateral lung collapse in cystic fibrosis-a therapeutic challenge

Atelectasis with Torpid Evolution in Patients with Cystic Fibrosis

“…Prados-Sánchez C 1* , Martínez-Redondo M 1 , Máiz-Carro L 2 , Girón-Moreno RM 3 , Quintana-Gallego E 4 , Martínez-Martínez MT 5 , Blanco-Aparicio M 6 , García-Clemente MM 7 , Iturbe-Fernández D 8 , García-Río F 1** , Carpio-Segura CJ 1** , Salcedo-Posadas A 3** , Martín de Vicente C 9** , Costa-Colomer J 10** and Maintz Jochen G 11…”

“…Respiratory complications, usually, afect the quality of life, morbidity and prognosis of the disease [1][2][3][4]. Within respiratory complications, atelectasis is which the least medical literature has generated, without consensus on its etiology, prognosis and treatment [5]. In a previous study of our group, the incidence was 2.66%, 2.33% were lobar and 1.33% segmental [6].…”

Is bronchoscopy an obsolete tool in cystic fibrosis? The role of bronchoscopy in cystic fibrosis and its clinical use

“…This is due various factors such as progression of disease, allergic bronchopulmonary aspergillosis, factors that would further alter the viscosity of sputum such as hyperglycemia and hemoptysis. Bronchoscopy with instillation of recombinant human DNase as single or sequential therapies have been reported after failure of standard of treatment including intravenous antibiotics, intermittent positive pressure breathing, and airway clearance therapy (42)(43)(44)(45)(46). Figure 1 shows a computed tomography image of patient with CF demonstrating progressive right lung atelectasis.…”

Muddy clinical waters: a missed betel nut in the bronchus

“…Progressive collapse and destruction of an entire lung is an uncommon but recognised manifestation of cystic fibrosis 9. To date, eight patients, diagnosed as having cystic fibrosis since infancy, have been reported to develop unilateral lung collapse and bronchiectasis with compensatory hyperinflation of the contra-lateral lung during their adult life 9.…”

“…To date, eight patients, diagnosed as having cystic fibrosis since infancy, have been reported to develop unilateral lung collapse and bronchiectasis with compensatory hyperinflation of the contra-lateral lung during their adult life 9. Reasons for development and impaction of thick mucus in the main bronchus in these eight adult cases are unclear; but suboptimal adherence with medication and airway clearance leading to chronic infection with Pseudomonas aeruginosa , increased viscosity of bronchial secretions due to hyperglycaemia and associated gastro-oesophageal reflux disease have been postulated to be the causative predisposing factors 9. The present case was a toddler; hence the diagnosis of cystic fibrosis was most unlikely.…”