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Mounier-Kuhn syndrome



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

X-ray signs: deformation of the pulmonary pattern with foci of compaction. Expansion of the lumen of the trachea and large bronchi. Bronchiectasis in the lower lobar segments.

With bronchoscopy, the enlarged lumen of the trachea (bronchi), thickening of the walls with bulging in the lumen of the interchillage gaps, pathological secretion.

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

Monogenic lung diseases:

1) primary ciliary dyskinesia and Kartagener's syndrome;

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

3) primary pulmonary hypertension (Aears syndrome);

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

5) Goodpasture syndrome;

6) family spontaneous pneumothorax;

7) alveolar microlithiasis;

8) alveolar proteinosis;

9) cystic fibrosis;

10) insufficiency of antitrypsin.

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Mounier-Kuhn syndrome

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