From the "Just Breathe Foundation" website;
Although the cause of Mounier-Kuhn is unknown, it is believed the disease is due to an absence of smooth muscle and elastic connective tissue in the trachea and main bronchi. This condition allows the trachea and main bronchi to become flaccid and collapse on expiration or cough.
The dynamics of this irregular airway predisposes a person to recurrent respiratory tract infections, pneumonia, and a combination in most cases of three lung diseases; bronchiectasis, emphysema and pulmonary fibrosis.
Mounier-Kuhn can cause progressive pulmonary disease leading to respiratory failure and death. It is normally pronounced in males and diagnosed during their late 30's or early 40's, though there have been cases of children as young as one year.
There are few who are brave enough to tell their patients.... Oh yes, this is a rare one and this disease carries with it - no cause and no cure.
