This was a 42-year-old non-smoker male, born by non-consanguineous parents. He presented to the outpatient clinic (in September 2006) with chief complaints of recurrent episodes of common cold, cough with expectoration for the past 15 years. The patient also revealed that he frequently complained of developed cough, cold, rhinorrhea, and wheezing since his childhood. At the time of the first visit, the vital parameters were within normal limits. On auscultation, bilateral wheezes without crackles were present. Electrocardiogram showed evidence of dextrocardia. At the time of the first visit, spirometric evaluation showed mild obstruction. Therapy with association salmeterol/fluticasone via DPI was established, with stabilization of functional respiratory pattern, in the follow up visits. In 2007 the nasal brushing was performed, showing all examined cilia lacking outer dynein arm (Figure ). He was hospitalized for pneumonia to the lower left lobe as reported in the chest CT scan done on September 18, 2013 (Figure ). The patient is still in follow up, in maintenance therapy and treated for exacerbations.
This was a 62-year-old non-smoker female. Married with one child. She presented to the outpatient clinic (in October 2007) complaining of cough with abundant mucopurulent expectoration, many bronchitic exacerbations (> 3 episodes/yrs), recurrent cold, and progressively increasing breathlessness in the past 5 years. In her past history there were frequent visits for recurrent chest infections and pneumonia. At the time of the first visit, the vital parameters were within regular limits, whereas at thoracic auscultation bilateral crackles in the basal region were present. Basal spirometric evaluation showed moderate obstruction with positive reversibility test. Therapy with association of salmeterol/fluticasone was prescribed, with stabilization of respiratory values at the follow up visits. In 2008 the nasal brushing was performed, with demonstration of 100% of cilia lacking outer dynein arms (Figure ). In the last years she suffered of 2/3 exacerbations per year, without need of hospitalization.