Mucoviscidosis

Cystic fibrosis, also known as fibrocystic degeneration, is an inherited disease that mainly affects the lungs, pancreas and intestines. This disease occurs due to a mutation in the CFTR gene, which encodes a protein responsible for transporting chlorine ions in cells.

In people with cystic fibrosis, this protein does not work properly, causing thick, sticky mucus to form in the lungs, pancreas and other organs. This mucus makes breathing, digestion, and organ function difficult.

The main symptoms of cystic fibrosis include chronic cough, breathing problems, increased salt loss through sweat and digestive problems. Patients with cystic fibrosis also often experience increased fatigue and slow growth.

Treatment of cystic fibrosis is aimed at improving the quality of life of patients and preventing progression of the disease. This may include taking medications such as antibiotics and drugs that thin out mucus, as well as exercise and breathing exercises to help improve lung function. In some cases, a lung or pancreas transplant may be required.

Overall, cystic fibrosis is a severe and chronic disease that can significantly reduce the quality of life of patients. However, modern treatments can help improve the prognosis and prolong the lives of people suffering from this disease.

Cystic fibrosis (CF) is a genetic disease that causes severe impairment of respiratory function. Cystic fibrosis is caused by the development of pathology at the cellular level. In a healthy person, in the lungs, bronchi, pancreas and liver there are cells that produce mucus - ciliated epithelium. It lines the inner walls of hollow organs along with luminal fluid, helping to clear bacteria and other agents. The appearance of cystic or fibrous tissue in the area of ​​the gland leads to the loss of the ability of the ciliated epithelium to fully function: unable to remove pathogens, it promotes the spread of viruses, bacteria, fungi, facilitating their capture in the body. The disease is called cystic fibrosis because as it develops, the mucus becomes thick and viscous, making breathing difficult. More details below.

Cystic fibrosis affects mainly children: up to 20% of patients have a chronic form, the rest of adults suffer from decompensated pathology - it has been diagnosed since 1980 in up to 90%. The number of deaths in the first year of life is up to 70%. Life expectancy with cystic fibrosis varies from 16 years to 60 years or more. The occurrence of the diagnosis is associated with persistent deficiency of enzymes responsible for the transport of chlorides, acini of the lacrimal apparatus and sweat glands. Cystic changes in tissues get worse without therapy - rough cavities grow, making diagnosis difficult. The survival prognosis is considered unfavorable. With cystic fibrosis, the health status of adults deteriorates several times faster than that of children. With the development of congenital CF, the average life expectancy is extremely low - less than a year. More favorable outcomes occur in children with acquired CF