Cystic fibrosis

A genetic disorder, characterized by a tendency to develop chronic lung infections and an inability to absorb fats and other nutrients from food. The main feature of cystic fibrosis (CF) is secretion of sticky mucus, which is unable to flow freely, in the nose, throat, airways, and intestines. The course and severity of the disease vary. Typically, a child passes unformed, pale, oily, foul-smelling faeces and may fail to thrive. Often, growth is stunted and the child has recurrent respiratory infections. Without prompt treatment, pneumonia, bronchitis, and bronchiectasis may develop, causing lung damage. Most male sufferers and some females are infertile. CF causes excessive loss of salt in sweat, and heatstroke and collapse may occur in hot weather. Prompt treatment with intensive physiotherapy and antibiotics helps to minimize lung damage from chest infections. Pancreatin and a diet rich in proteins and calories are given to bring about weight gain and more normal faeces. However, despite treatment, most people with CF suffer permanent lung damage and have a reduced life expectancy. Lung or heart–lung transplants have produced good results, and specific gene therapy is becoming a possibility. Amniocentesis can determine whether or not a fetus is affected, or, alternatively, newborn babies can be screened for the disease; early diagnosis and treatment improves the long-term prognosis.


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