Diseases of the respiratory system

Hereditary diseases of the respiratory system

Hereditary diseases of the respiratory system are, according to different authors, from 51 to 35% of the total number of patients with lung diseases.

Distinguish the hereditary diseases of the lungs and lesions of the bronchopulmonary system that accompany various types of hereditary diseases. Monogenic lung diseases can be inherited, as with any hereditary pathology. In this case, inheritance can be carried out both in autosomal dominant and autosomal recessive type. Monogenic lung diseases include tracheobronchomegaly( Mounier-Kuhn syndrome), Williams-Campbell syndrome, primary ciliary dyskinesia( Kartagener syndrome), primary pulmonary hypertension, hereditary spontaneous pneumothorax, idiopathic diffuse pulmonary fibrosis, isolated pulmonary hemosiderosis, Goodpasture's syndrome, alveolar microlithiasis. Among the hereditary diseases in which pulmonary lesions are one of the main clinical manifestations, one should mention cystic fibrosis, as well as various variants of primary immunodeficient conditions.

Lung disorders can occur in other hereditary diseases( Marfan syndrome, Ehlers-Danlos syndrome, Rowley-Rezenberg syndrome, Osler-Randu-Weber syndrome, hemophilia, Klinefelter syndrome), which are inherited by autosomal dominant, autosomal recessive type or areresult of chromosomal pathology. However, most often in the pulmonology clinic there are patients with multifactorial diseases. Among such diseases are bronchial asthma, the prevalence of which is constantly increasing all over the world, as well as exogenous allergic alveolitis. Smoking, respiratory infection and many other factors lead to the formation of emphysema( increased airiness) of the lungs.

The vast majority of hereditary diseases manifest themselves in childhood, are no exception and lung disease.

The severity of congenital and hereditary conditioned lung diseases, their recurrent( recurrent exacerbations of the disease) and chronic nature, decreased performance and disability of both adults and children make the problem not only medical but also social. At the same time, pessimism regarding the effectiveness of therapeutic and health effects in hereditary diseases is not justified. Complex therapy in many patients leads to stabilization of the pathological process and improvement of their condition.