Sindrome de mounier kuhn pdf

Alcaligenes xylosoxidans is a non fermenting gramnegative pathogen common in extraand intrahospital environment, which may be related to immunosuppression states. Mounierkuhn syndrome in an elderly female with pulmonary. People with this condition develop frequent respiratory tract infections and recurrent cough. It was described in 1932 to refer to the presence of tracheobronchomegaly. This patient had lung transplantation later in their clinical course. The abnormally widened trachea and mainstem bronchi are associated with recurrent lower respiratory tract infection and copious purulent sputum production, eventually leading to bronchiectasis and other respiratory complications. The disease commonly affects males and presents in adult life. Mounierkuhn syndrome mimicking lymphangioleiomyomatosis. It is characterized by distinct dilation of the trachea and bronchi and by recurrent lower respiratory tract infections lrtis.

Mounierkuhn syndrome or congenital tracheobronchomegaly. It should be noted that one rare, often overlooked and underrecognized, cause of respiratory tract infections and bronchial dilation is mounierkuhn syndrome mks. The anatomical and physiological changes present in the airways predispose to stagnation within enlarged portions of the. In 1988, the first visualizations of mks in computed tomography ct were described 2, 3, and although numerous case reports are published every year, there have been.

The patient was evaluated using ongoing protocols and provided written informed consent. The condition can be diagnosed by lung function tests, bronchoscopy, and a chest ct scan. Geovanny hernandez1, franco ruales2, leslie vargas3, faiver. Mounier kuhn syndrome is most frequently seen in middle age men before the age of 50 years 1,6. Mounierkuhn syndrome, or tracheobronchomegaly, is a rare clinical and radiologic condition. Mounierkuhn syndrome mks is a unique condition characterized by dilation of the trachea and major bronchi and recurrent pneumonias. We present the case of a man with mounierkuhn syndrome mks, or tracheobronchomegaly, who was referred to the national institutes of health clinical research center because of a potential diagnosis of lymphangioleiomyomatosis lam, a rare condition in men. Tracheobronchomegaly is a very rare congenital disorder of the lung primarily characterized by an abnormal widening of the upper airways. Mounierkuhn syndrome is a somewhat controversial entity and used synonymously with tracheobronchomegaly by most authors 79. Mks can be diagnosed in adult women when the transverse and sagittal diameters of the. We describe the case of a 75 years old male, exsmoker with. The cause of mounierkuhn syndrome is unknown, although cigarette smoke and air pollutants may act as irritating.