Pulmonary cystic fibrosis

Cystic pulmonary fibrosis (CPF or CF) is a congenital disease with a family of autosomal recessive inheritance. It is most common among North American whites, with about one in every 2,500 people affected, one in 25 carriers, and other races are rare. As a disease of the exocrine glands, 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 abnormal transport of Na and Cl-, the ducts of the pancreas and some other exocrine glands are filled with mucus, which leads to obstruction. Due to the increase in mucus in the bronchi, the bronchi can be blocked, and certain bacteria (such as Staphylococcus aureus, Pseudomonas aeruginosa, etc.) are easy to grow and reproduce, further causing repeated infections of the lungs and bronchial tubes, followed by cystic pulmonary fibers And severely impaired lung function, with the increase of lung disease and lung function damage, further leading to right heart hypertrophy and heart failure. The lack of pancreatic enzymes can also cause clinical manifestations such as indigestion and developmental disorders. If the disease can be diagnosed early and reasonable comprehensive treatment, most patients may survive to 20 years or longer. Research on various detailed treatment methods is currently underway.

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