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Mounier-Kun syndrome

Clinical picture. From an early age cough with sputum, repeated exacerbations of bronchopulmonary disease, increase in respiratory failure during the period of exacerbation and with age. Deformation of the nail phalanxes in the form of "drum sticks".

Radiographic signs: deformation of the pulmonary pattern with areas of consolidation. The expansion of the lumen of the trachea and large bronchi. Bronchiectasis in the lower lobe segments.

In bronchoscopy, the expansion of the lumen of the trachea (bronchi), thickening of the walls with bulging into the lumen of the interchondral spaces, pathological secretion.

Treatment. Treatment is conservative, aimed at fighting
with bronchopulmonary infection.

Monogenic lung diseases:

1) primary ciliary dyskinesia and Cartagene's syndrome;

2) idiopathic diffuse pulmonary fibrosis (Hammen-Rich syndrome, idiopathic fibrosing alveolitis);

3) primary pulmonary hypertension (Aers syndrome);

4) idiopathic hemosiderosis of the lungs (Celen-Gellerstedt syndrome);

5) Goodpasture syndrome;

6) family spontaneous pneumothorax;

7) alveolar microlithiasis;

8) alveolar proteinosis;

9) cystic fibrosis;

10) insufficiency a ^ antitrypsin.

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Mounier-Kun syndrome

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