Diagnosis and treatment of cystic pulmonary fibrosis

Cystic pulmonary fibrosis (Cystic Pulmonary Fibrosis, CPF or CF) is a chronic lung disease. It is an autosomal recessive genetic disease caused by gene mutation. Specifically, the transmembrane regulator gene (CFTR) located on the long arm of the seventh chromosome is mutated, resulting in abnormal protein function, which in turn causes the disorder of chloride ion transmembrane transport. This will lead to an increase in the content of acidic glycoproteins in the secretion, resulting in a sticky secretion, which significantly reduces the mucociliary clearance function, thus causing repeated lung infections. In addition, Pseudomonas aeruginosa infection is also an important cause of the disease, which is easy to colonize and multiply when mucociliary clearance function is reduced, leading to airway mucus obstruction and progressive lung tissue necrosis. The diagnosis of cystic pulmonary fibrosis is usually based on the following steps: Medical history inquiry: Ask whether the patient has a history of long-term exposure to harmful substances (such as dust, smoke, chemicals, etc.), and whether there is a genetic history of cystic pulmonary fibrosis or other related diseases in the family. Symptom assessment: Observe whether the patient has typical symptoms such as cough, expectoration, expiratory dyspnoea, recurrent fever, bronchiectasis, etc. In addition, patients may also have gastrointestinal symptoms, such as gastrointestinal obstruction. Auxiliary examination: Chest X-ray: It may be manifested as hyperinflation, and bronchiectasis, pulmonary fibrosis, emphysema and other signs may occur in the later stage. Lung CT: It can more clearly show the structure of the lung, such as patchy and blurred shadows, cystic changes at the beginning of the bronchus, etc. Sweat examination: Patients with cystic pulmonary fibrosis usually have significantly increased sodium chloride in their sweat, which is an important clue to the diagnosis of the disease. Genetic examination: Genetic tests can detect gene mutations and provide direct evidence for the diagnosis of the disease and its differentiation from other diseases. 2. Treatment of cystic pulmonary fibrosis mainly includes drug therapy, physical therapy, surgical treatment and adjuvant therapy: Drug therapy: Antibiotics: for bacterial infections, such as amoxicillin capsules, salbutamol sulfate tablets, levofloxacin hydrochloride capsules, etc., can inhibit bacterial growth and reduce inflammatory response, thereby alleviating symptoms. Anti-inflammatory drugs, such as aminophylline tablets, are used to reduce lung inflammation. Bronchodilators: used to dilate the bronchi and relieve expiratory dyspnoea. Expectorant: Helps discharge sputum and relieves respiratory tract obstruction. Pancreatic enzyme supplementation: Since patients with cystic pulmonary fibrosis may be accompanied by pancreatic enzyme deficiency, pancreatic enzyme supplementation can help improve digestive function. Physical therapy: Postural drainage: by changing the patient’s position or using specific equipment to promote sputum drainage and reduce airway obstruction. Chest physiotherapy: Such as patting the back, vibration, etc., to help loosen and discharge sputum. Surgical treatment: When drug therapy and physical therapy are ineffective and the condition is serious, surgical treatment, such as lung transplantation, can be considered. Adjuvant therapy: Breathing exercises, such as deep breathing exercises and muscle strengthening exercises, are designed to improve lung ventilation and respiratory muscle strength to improve overall respiratory efficiency. Pulmonary rehabilitation: including nutritional support, psychological counseling and other measures to improve the quality of life of patients and alleviate the development of the disease. Lifestyle adjustment: Avoid exposure to harmful substances: Patients should try to avoid long-term exposure to harmful substances, such as tobacco smoke, air pollution and so on. Eat healthy: Maintain a high-calorie, high-vitamin diet, especially vitamins C and E. Smoking cessation and alcohol restriction: Smoking cessation and alcohol restriction can help reduce the burden on the lungs and delay the progression of the disease. To sum up, the diagnosis of cystic pulmonary fibrosis requires comprehensive consideration of medical history, symptoms, auxiliary examinations and other factors. The treatment should be individualized according to the specific conditions of patients, including drug therapy, physical therapy, surgical treatment and adjuvant therapy. At the same time, patients should also pay attention to adjusting their lifestyle in order to alleviate the condition and improve the quality of life.