FEW DISEASES in the respiratory system are as poorly understood and as frustrating to treat as the syndrome first described clinically by Mounier-Kuhn in 1932, characterized by a diffuse dilation of the trachea and major bronchi. Although some patients with this condition are asymptomatic, the following case demonstrates the usual course of the disease, leading ultimately to death from uncontrolled infection and respiratory insufficiency.
Report of a Case
A nonsmoking 43-year-old woman was first seen in March 1974 with a clinical and roentgenographic picture of right lower lobe pneumonia. For three years she had had a chronic cough, productive of several mouthfuls of purulent sputum each day. The preceding year she had noticed progressive exertional dyspnea. She denied any previous respiratory illnesses and specifically could not recall any neonatal or childhood pneumonia or foreign body aspiration.Repeated sputum cultures yielded a Proteus organism. Smears and cultures were negative for acid-fast