Lung inflammation in cystic fibrosis

In 75-80% of cases of cystic fibrosis, a mixed pulmonary-intestinal form is diagnosed, in 15-20% - a predominantly pulmonary form of the disease. Symptomocomplex bronchopulmonary changes in cystic fibrosis by 90% determines the prognosis of the disease.

Pathogenesis. The viscous secret of the mucous glands clogs the small bronchi and leads to the obstruction of the peripheral respiratory tract, the function of the cilia of the ciliated epithelium is disrupted.

Later, a secondary infection is attached, inflammatory infiltration of the mucous membrane of the bronchial tree appears with the development of obliterating bronchiolitis, bronchiectasis, pneumofibrosis, and obstructive emphysema.

Symptoms. Exacerbations of the bronchial type occur with a diffuse auscultative pattern, a prolonged febrile temperature.

Pneumonia in cystic fibrosis is characterized by a protracted course, more often localized in the upper parts of the lungs, often there is a bilateral lesion, there is a tendency to atelectasis and abscessing.

A typical complaint of patients is almost constant painful paroxysmal productive cough with hard-to-recover sputum, dyspnea of mixed character.

Children, as a rule, lag behind in physical development, changes in nail phalanges are identified by the type of tympanic sticks, nails in the form of hourglasses. The thorax acquires a "barrel-shaped" shape, which, along with the increase in the abdomen, gives patients with cystic fibrosis a characteristic appearance.

Patients with cystic fibrosis often develop sinusitis with persistent flow; due to the violation of secretion of the secretion of salivary glands, the occurrence of nonspecific mumps is possible.

Chronic infectious process and violation of digestion and absorption of food lead to fatigue, decreased ability to learn, symptoms of hypovitaminosis A and E are characteristic. Sometimes physical data can be very scarce, which creates contrast with pronounced changes in radiographs.

The radiological picture depends on the severity of the phase of the disease. Strength, severity, meshiness, cellularness of the pulmonary pattern, signs of bronchial obstruction, alveolar filling syndrome (infiltration, alveolar edema), "cellular lung" syndrome (large-cell deformity of the lung pattern with the formation of thin-walled cavities 0.3-1.0 cm in size) are revealed. .

In the investigation of HPD, obstructive disorders are revealed, and as the disease progresses, they become mixed.

The diagnosis is based on the presence of a bronchopulmonary clinic, typical symptoms on the part of the gastrointestinal tract, the detection of cases of cystic fibrosis in the relatives of the child and the conduct of a sweat test.

The study of sweat chloride is crucial in confirming the diagnosis. The content of chlorides in the fluids above 60 mmol / l is considered diagnostic for cystic fibrosis. If the concentration of sweat chloride is from 40 to 60 mmol / l and there are clinical signs of cystic fibrosis, then a dynamic observation with a repeat of the study and carrying out the DNA diagnostics are necessary. Currently, there is a non-invasive method that allows identifying the 12 most common mutations by examining DNA from a material taken by brush-biopsy (scraping) from the inner surface of the cheek.

Cystic fibrosis can occur in 1-2% of cases with normal parameters of sweat chloride. However, there are diseases in which the lot test can also be positive or borderline (adrenal insufficiency, pseudoaldosteronism, hypoparathyroidism, hypothyroidism, etc.).

Treatment is reduced to liquefaction of viscous bronchial secretion, improvement of drainage function of bronchi and antimicrobial therapy, treatment of complications of cystic fibrosis.

To improve the drainage function of the bronchi and to combat mucostasis, mucolytic drugs are constantly used. Preference is given to N-acetylcysteine (flumucil, mucosalvin) in the form of inhalations and / or per os. The advantage of the preparations of this group is that they damage the mucous membrane to a lesser extent during prolonged use and have antioxidant properties.

The appointment of mucolytics must be combined with the constant kinesitherapy, postural drainage, vibromassage and the use of respiratory masks with increased exhalation resistance.

Antimicrobial therapy is carried out in accordance with the sensitivity of the isolated microorganisms. Accession of Pseudomonas aeruginozae is a poor prognostic sign for a patient with cystic fibrosis and, at the same time, intravenous administration of antibiotics to which the microorganism in a particular patient is sensitive (gentamicin, ciprofloxacin, carbenicillin, fortum, imipenem, etc.).

The correct organization of dispensary observation plays an important role in the successful treatment of patients with cystic fibrosis. Cystic fibrosis is not only a medical, but also a social problem.

[1], [2], [3], [4], [5], [6], [7]