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.
This was a 32-year-old non-smoker female. She presented to the outpatient clinic (in October 2007) with chief complaints of recurrent cold, cough with copious expectoration, and progressively increasing shortness of breath for the last 3 years. She had been married for the last 6 years, without children. Her past history was significant in that she had had frequent visits for recurrent chest infections. Her family history revealed no parental consanguinity. Main purpose of the visit in our Unit was a planned pregnancy. At the time of the first visit, the vital parameters were within regular limits, and thoracic auscultation was normal. The patients was fostered to pregnancy, and she has had three children so far. In 2008 the nasal brushing was performed, showing the absence of outer dynein arms in all the ciliary sections (Figure ). In the last year, she suffered of some exacerbations and pneumonia, with 2 hospitalizations in our Unit. She had more chest CT scans, and the last one was done in 2013 (Figure ). Spirometric evaluation at the follow up visits showed mild to moderate obstruction, and therapy with the MDI fixed combination formoterol/beclometasone was established, with stabilization of functional respiratory pattern between the crisis. Exacerbations were treated with antibiotics, and she is still in maintenance therapy with LABA/ICS inhalant therapy. In 2014 diagnosis of autoimmune thyroiditis was done.