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Hospital pediatrics. Classification of malformations of the bronchopulmonary system (the most important)

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48. Classification of malformations of the broncho-pulmonary system. Chronic and acquired diseases in children

A malformation is an anomaly in most cases of intrauterine development, resulting in gross changes in the structure and function of an organ or tissue.

Classification of malformations of the bronchopulmonary system.

1. Malformations associated with the underdevelopment of the organ as a whole or its anatomical, structural, tissue elements:

1) lung agenesis;

2) lung aplasia;

3) lung hypoplasia;

4) cystic hypoplasia (polycystic);

5) tracheobronchomegaly (Mounier-Kun's syndrome);

6) Williams-Campbell syndrome;

7) congenital lobar emphysema.

2. Defects associated with the presence of excess dysembryogenetic formations:

1) accessory lung (lobe) with normal blood supply or with abnormal blood supply;

2) lung cyst with normal blood supply or with abnormal blood supply;

3) hamartoma and other tumor-like formations.

3. Unusual anatomical arrangement of lung structures, sometimes of clinical significance:

1) reverse arrangement of the lungs (Kartegener's syndrome);

2) mirror lung;

3) tracheal bronchus;

4) share of the unpaired vein.

4. Localized violations of the structure of the trachea and bronchi:

1) stenosis;

2) diverticula;

3) tracheoesophageal fistulas. 5. Anomalies of blood and lymphatic vessels:

1) stenosis of the pulmonary artery and its branches;

2) varicose pulmonary veins;

3) multiple arteriovenous fistulas without clear localization.

Hereditary diseases of the respiratory system, according to various authors, range from 5 to 35% of the total number of patients with nonspecific lung diseases.

Chronic lung diseases in children (S. Yu. Kaganov, 2003).

1. Infectious and inflammatory diseases.

2. Congenital malformations of the bronchopulmonary system.

3. Hereditary lung diseases.

4. Lung lesions in other hereditary diseases.

5. Allergic diseases of the lungs. Acquired diseases:

1) a common type of pathological changes that cause obstruction;

a) allergic inflammation, bronchial asthma;

b) infectious inflammation;

2) recurrent and chronic obstructive bronchitis;

3) local type of pathological changes causing obstruction (mechanical factors);

4) foreign body, tumor, infectious granuloma, post-traumatic cicatricial stenosis.

Author: Pavlova N.V.

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