CYSTIC FIBROSIS WHAT IS THIS DISEASE CAUSES SYMPTOMS TREATMENT

 

Cystic fibrosis - what is this disease? Cystic fibrosis is a genetic pathology that is transmitted by autosomal recessive type. The disease is characterized by impaired secretion of endocrine glands and vital organs. Mostly affected respiratory and digestive systems.

Causes of pulmonary cystic fibrosis: due to gene mutations, the structure of the protein is disrupted, the secretion of most endocrine glands thickens, it is difficult to evacuate, and this causes severe secondary changes in the respiratory system. The giandliverconsultants provide the best gastrointestinal consultants in USA. In the bronchi develops a chronic inflammatory process, sputum becomes thick and viscous, stagnant, bronchiectasis is formed. Patients with pulmonary cystic fibrosis are prone to developing chronic inflammation in the bronchi. All changes over time lead to hypoxia and the formation of the pulmonary heart.

Signs of cystic fibrosis in adults and children

The disease is manifested by a variety of clinical symptoms. For example, in newborns mycoviscidosis may be manifested by meconic ileus with the development of intestinal obstruction.

·         cough;

·         cyanosis;

·         allocation of thick sputum;

At defeat of bronchopulmonary system, the clinical picture of obstructive bronchitis, bronchiectasis, pulmonary emphysema, infectious-dependent bronchial asthma is gradually formed. In children aged 5-15 years, the disease is often manifested by intestinal colic. Patients complain of bloating, pain, repeated vomiting.

·         Signs of cystic fibrosis in adults with pulmonary disease:

·         painful cough attacks;

·         allocation of viscous mucopurulent sputum;

·         dyspnea;

·         signs of chronic rhinitis - sneezing, runny nose and so on;

·         weakness, reduced efficiency;

·         pale skin;

Such patients have chronic obstructive bronchitis, frequent pneumonia, bronchiectasis, pulmonary emphysema, pulmonary heart. Extra pulmonary manifestations of cystic fibrosis - damage to the liver and biliary tract, pancreas, dysfunction of the gonads.

Diagnosis of cystic fibrosis

Blood tests for cystic fibrosis (determination of blood glucose, liver function tests) show whether there are complications caused by the disease. Analysis of acidity and gases allows you to determine the level of carbon dioxide and oxygen in the blood.

Diagnosis of cystic fibrosis in newborns is neonatal screening. The amount of immunoreactive trypsinogen (digestive enzyme) is determined in the blood of infants. If its level exceeds the norm, it indicates the presence of cystic fibrosis. To confirm the diagnosis, a genetic test is additionally performed. Perinatal diagnosis is performed from the 20th week of pregnancy by determining alkaline phosphatase is enzymes in amniotic fluid.

Sweating test for cystic fibrosis is performed by stimulating sweating in the forearm (electrophoresis with pilocarpine). Next, the amount of chlorides in the sweat is determined. To obtain reliable results, collect at least 50 mg of sweat. If the concentration of chlorides is more than 100 mmol / l, the diagnosis is considered reliable, more than 60 mmol / l - probable. At the limit value, it is recommended to additionally test with prednisolone.

Treatment of patients with cystic fibrosis

Clinical recommendations for cystic fibrosis depend on the form and stage of the disease. When the bronchopulmonary system is affected, the therapy helps to relieve inflammation and facilitate breathing. The giandliverconsultants provide the best liver consultants in USA. It should be noted that there is no universal treatment protocol - in each case an individual scheme is developed.

Therapy includes:

·         postural drainage and breathing exercises that help expel sputum;

·         antibacterial therapy;

·         inhalation bronchodilators (bronchodilators);

·         mucolytic;

Treatment of cystic fibrosis requires a comprehensive approach and is carried out in specialized centers. Medical examinations should take place at least once every three months. The patient passes general blood and urine tests, sputum analysis (for flora and sensitivity to antibiotics), fecal analysis, after which the doctor adjusts the treatment and rehabilitation regime. Read more about the dispensary observation of patients with cystic fibrosis on our website Dobrobut.com.