What Is the Effect of Cystic Fibrosis on the Lungs?

Pulmonary cystic fibrosis (CPF or CF) is a congenital disease with a family of autosomal recessive inheritance. It is most common in North American whites, and rare in other races. As an exocrine gland lesion, the gastrointestinal and respiratory tracts are often involved. The diagnosis is based on increased NaCl content in sweat, which reflects the abnormal function of exocrine glands. Due to the increase in mucus in the bronchi, the bronchus can be blocked, making certain bacteria (such as Staphylococcus aureus, Pseudomonas aeruginosa, etc.) easy to grow and reproduce, further causing repeated infections of the lungs and bronchial tubes, followed by pulmonary cystic fibers And severely impaired lung function, with the increase of lung diseases and lung function damage, further leading to right heart hypertrophy and heart failure. Due to the lack of pancreatin, it can also cause clinical manifestations such as indigestion and developmental disorders.

Basic Information

English name: systic fibrosis of lung andpancrease
Visiting department: Respiratory Medicine
Multiple groups: North American white

Common causes: Mutation of the transmembrane regulatory factor gene (CFTR) directly causes Pseudomonas aeruginosa infection
Common symptoms: Large amount of fatty stools, dry cough, sticky sputum and difficult to cough, and later paroxysmal cough, increased sputum volume, etc.

Causes of pulmonary cystic fibrosis

Studies have suggested that the occurrence of CF is directly related to mutations in the transmembrane regulatory factor gene (CFTR), which directly leads to P. aeruginosa infection. The direct consequences of P. aeruginosa infection are airway mucus obstruction and progressive lung tissue necrosis.

Clinical manifestations of pulmonary cystic fibrosis

The typical clinical manifestations are repeated respiratory and pulmonary infections, and insufficient pancreatic secretory glands, such as a large amount of fatty stools. The first symptoms of the respiratory tract are cough, mainly dry cough, thick sputum that is difficult to cough, and subsequent paroxysmal cough with increased sputum volume. Hypoxia such as chest tightness, belching, and dyspnea can occur. These symptoms can last for weeks or even months. If there is repeated hemoptysis during bronchiectasis, cyanosis and clubbing fingers may be present in the later stage, often with severe complications such as pulmonary heart disease and heart failure.

Pulmonary cystic fibrosis

Sweat test

Positive.

2. Pancreas stimulation test

Pancreatin was measured, pancreatin was significantly reduced or nearly normal, but bicarbonate was significantly reduced.

3.X-ray inspection

(1) In the early stage of lung texture changes, bronchiectasis is manifested as increased lung texture.

(2) The lobular pneumonia-like changes are manifested as bronchial obstruction and infection below the segment, forming small patchy, fuzzy shadows.

(3) There is a circular shadow around the hilum of the lung field, which is an important X-ray sign of the cystic cavity at the beginning of the bronchus. This circular shadow is an abnormal bronchiectasis, rather than a real cavity, and the upper leaves are more obvious. There may also be signs of air accumulation in the lower lobe, including loss of peripheral blood vessel distribution and diaphragm flatness. At the same time, local obstructive atelectasis, emphysema, lung abscess, and pulmonary heart disease can occur.

(4) Bronchogram examination shows mild and moderate columnar bronchiectasis, which often occurs in the upper lobe of both lungs.

4. Chest CT examination

(1) The bronchial wall thickening and bronchiectasis can be widely distributed in each lobe of both lungs, especially the upper lobe of both lungs. Bronchodilation is mainly light and moderate columnar bronchiectasis. Bronchial wall thickening can occur both in dilated bronchi and non-dilated bronchi. It is often mildly thickened and the inside and outside of the tube wall are relatively smooth.

(2) Diffuse emphysema of the two lungs is characterized by low and uneven lung field density, and the severity of the disease varies in different cases. It is more common in infants and children.

(3) Bronchial mucus plugs are formed due to the retention of mucus secretions in the trachea, which vary in shape depending on the direction of the bronchial tubes in which the mucus is retained, mostly round, oval, tubular, or "V" or "Y" with the tip pointing to the hilar "Shaped high-density shadow, uniform density, smooth and sharp edges, CT value is generally 15 ± 10HU, but the CT value of long-lasting mucus plugs can be as high as 40 ~ 80HU, enhanced scan without enhancement.

(4) Thin-walled balloon-containing cavities due to bronchiectasis, emphysema, and interstitial balloon cysts form cysts of different sizes, mainly distributed in the upper lungs.

(5) Patchy shadows are manifested as infectious bronchial pneumonia and atelectasis of the subsegment, showing patchy high-density shadows ranging in size from 1 to 3 cm. The upper lung field is common or the lesions in the upper lung field are more distributed.

5. Chest MRI

MRI shows that the pathological changes of cystic fibrosis in the respiratory system are clogging of the bronchi and secondary infections by secretions. MRI can better show bronchial mucus emboli and infectious lesions in the lung. Mucus emboli show slightly shorter and longer T1 abnormal signals. The signal is uniform, the edges are smooth and sharp, and their shapes are different. The tip points to the hilum. The secondary infection is mainly lobular pneumonia-like changes.

Diagnosis of pulmonary cystic fibrosis

Laboratory tests for quantitative pilocarpine electroosmosis sweat tests. Because there is a high concentration of NaCl in sweat, in general, Cl- <60mmol / L, if the measurement result C1-> 70mmol / L is positive, it has diagnostic value. Combined with the abnormal function of exocrine glands such as the pancreatic duct, the stool volume is more, and the fat is more. The children are often prone to respiratory infections, the mucus secretions of the respiratory tract increase, and it is easy to cause airway obstruction. Combined with family history, X-ray, CT, MRI, etc. can diagnose the disease.

Differential diagnosis of pulmonary cystic fibrosis

Pulmonary cystic fibrosis often occurs with cystic bronchiectasis, so it must be distinguished from some diseases that cause cystic bronchiectasis. Cystic bronchiectasis is a complication of recurrent or chronic infection. Its performance may resemble multiple cavities, but it is not true. Cavity, but multiple bronchiectasis accompanied by cystic cavities.

Gamma globulin deficiency

This patient is prone to recurrent bacterial infections, secondary airway obstruction and cystic bronchiectasis, and sometimes difficult to distinguish from pulmonary cystic fibrosis, but the patient's blood gamma globulin was significantly reduced or lacking, and there was no high concentration of NaCl is present so it can be identified.

2. Recurrent bacterial pneumonia

Recurrent attacks can cause bronchiectasis. In the early stage, this bronchodilation can be cylindrical and reversible. However, after multiple episodes of pneumonia, varicose veins or cystic bronchiectasis can develop due to bronchial damage. Its characteristics. High-resolution CT (HRCT) can be used instead of bronchography to obtain a diagnosis.

3. Allergic bronchopulmonary aspergillosis

Bronchial cystic dilatation can occur with asthma, but generally the age of onset of the disease is relatively late, there is no family genetic history, fiber bronchoscopy can find aspergillus, and glucocorticoids can be cured.

4. Tuberculous bronchiectasis

Tuberculosis is another cause of cystic bronchiectasis. After long-term tuberculosis infection, cavities can occur in the apex and the entire lung. In addition to necrotic cavities in these transparent areas, bronchodilation must be considered, especially cystic bronchi Dilatation, but bronchiectasis caused by tuberculosis usually have symptoms of poisoning, such as low fever, night sweats, etc. Tuberculosis bacilli can be found in sputum.

Pulmonary cystic fibrosis complications

If there is repeated hemoptysis during bronchiectasis, cyanosis and clubbing fingers may be present in the later stage, often with severe complications such as pulmonary heart disease and heart failure.

Treatment of pulmonary cystic fibrosis

Pulmonary cystic fibrosis, if you can inquire about the medical history in detail, get early diagnosis and reasonable comprehensive treatment, the prognosis is optimistic, most patients can survive to 20 years or even longer. Otherwise, many sick children will often die before the age of 10 due to repeated respiratory infections leading to severe lung function damage, right heart overload, pulmonary heart disease, and heart failure. In the treatment of children with repeated respiratory infections, antibiotics must be applied to control inflammation of the respiratory tract and lungs and prevent further development of the disease. Other treatments include pancreatin supplementation, physical therapy, a high-calorie diet, and multivitamin supplementation, especially vitamins C and E.