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Lung damage in cystic fibrosis
Last reviewed: 04.07.2025
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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. The symptom complex of bronchopulmonary changes in cystic fibrosis determines the prognosis of the disease by 90%.
Pathogenesis. The viscous secretion of the mucous glands clogs the small bronchi and leads to obstruction of the peripheral respiratory tract, the function of the cilia of the ciliated epithelium is disrupted.
Subsequently, a secondary infection occurs, 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 bronchitis type occur with a diffuse auscultatory picture and prolonged febrile temperature.
Pneumonia in cystic fibrosis is characterized by a protracted course, is often localized in the upper parts of the lungs, bilateral lesions often occur, and there is a tendency to atelectasis and abscess formation.
A characteristic complaint of patients is an almost constant, painful, paroxysmal productive cough with difficult-to-separate sputum and shortness of breath of a mixed nature.
Children usually lag behind in physical development, changes in the nail phalanges are revealed in the form of drumsticks, nails in the form of watch glasses. The chest acquires a "barrel-shaped" shape, which, along with an increase in the abdomen, gives patients with cystic fibrosis a characteristic appearance.
Patients with cystic fibrosis often experience persistent sinusitis; due to the disruption of secretion by the salivary glands, non-specific mumps may develop.
Chronic infectious process and disturbance of digestion and absorption of food lead to fatigue, decreased learning ability, and symptoms of hypovitaminosis A and E are characteristic. Sometimes physical data can be very scanty, which creates a contrast with the pronounced changes on radiographs.
The radiographic picture depends on the severity of the th phase of the disease. Increased, stringy, reticular, cellular pulmonary pattern, signs of impaired bronchial patency, alveolar filling syndrome (infiltration, alveolar edema), "honeycomb lung" syndrome (large-cell deformation of the pulmonary pattern with the formation of thin-walled cavities measuring 0.3-1.0 cm) are revealed.
When studying the respiratory function, obstructive disorders are revealed, and as the disease progresses, they become mixed.
The diagnosis is based on the presence of clinical signs of a bronchopulmonary process, typical symptoms from the gastrointestinal tract, identification of cases of cystic fibrosis in the child's relatives, and a sweat test.
Sweat chloride testing is crucial in confirming the diagnosis. A sweat chloride content above 60 mmol/l is considered diagnostic for cystic fibrosis. If the sweat chloride concentration is between 40 and 60 mmol/l and there are clinical signs of cystic fibrosis, then dynamic observation with repeated testing and DNA diagnostics are necessary. Currently, there is a non-invasive method that allows identifying the 12 most common mutations by testing DNA from material taken by brush biopsy (scraping) from the inner surface of the cheek.
Cystic fibrosis can occur in 1-2% of cases with normal sweat chloride levels. However, there are diseases in which the lotion test can also be positive or borderline (adrenal insufficiency, pseudoaldosteronism, hypoparathyroidism, hypothyroidism, etc.).
Treatment consists of liquefying viscous bronchial secretions, improving the drainage function of the bronchi and antimicrobial therapy, and treating complications of cystic fibrosis.
In order to improve the drainage function of the bronchi and combat mucostasis, mucolytic drugs are constantly used. Preference is given to N-acetylcysteine (fluimucil, mucosalvin) in the form of inhalations and/or per os. The advantage of drugs in this group is that they damage the mucous membrane to a lesser extent with prolonged use and have antioxidant properties.
The administration of mucolytics must be combined with ongoing kinesitherapy, postural drainage, vibration massage and the use of PEP masks for breathing with increased resistance during exhalation.
Antimicrobial therapy is carried out in accordance with the sensitivity of the isolated microorganisms. The addition of Pseudomonas aeruginozae is a bad prognostic sign for a patient with cystic fibrosis, and intravenous administration of antibiotics to which the microorganism is sensitive in a particular patient is mandatory (gentamicin, ciprofloxacin, carbenicillin, fortum, imipenem, etc.).
Proper 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.
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