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Signs and symptoms associated with CF include:
- Frequent coughing, chronic cough and sputum production
- Repeated or persistent chest infections suchas bronchitis or pneumonia
- Recurrent sinus infections and nasal congestion caused by nasal polyps
- Abdominal pain or discomfort
- In newborns, lack of stool in the first 1–2 days after birth (meconium ileus)
- Chronic diarrhoea or bulky, foul-smelling greasystools
- Weight loss or malnutrition
- In children, failure to gain weight or grow at a normal rate (failure to thrive)
- Decreased levels of protein in the blood, leading to accumulation of fluid, usually just under the skin (oedema)
CF interferes with electrolyte and fluid balances in the body. In most individuals affected by CF, sweat is up to five times saltier than normal and severe salt depletion can occur in very hot weather.
Much of the illness associated with CF is due to respiratory infections and lung complications. From early childhood the abnormal composition of the fluid lining the airways makes affected individuals prone to infection. The lubricating mucus in the lungs becomes thick and sticky, leading to chronic infections, severe inflammation and eventual lung damage. However, with early diagnosis through neonatal screening, early recognition of infection and aggressive treatment of the infections using oral, inhaled and intravenous antibiotics, chronic infection can be delayed or even prevented in many of those affected.
The excessively thick and sticky secretion lining the pancreatic ducts leads to obstruction and extensive destruction of pancreatic tissue. The resulting deficiency of pancreatic enzymes and bicarbonate causes severe intestinal malabsorption of fat and protein and fat-soluble with foul-smelling, greasy stools. Nutritional support with oral high energy supplements, pancreatic enzyme replacement therapy and supplements of fat-soluble vitamins A, D, E and K can, in many people, achieve improved absorption, nutritional state and growth. So most patients now survive into adult life, but some patients develop CF-related diabetes due to pancreatic damage, and some patients develop osteoporosis.
Other problems and symptoms associated with CF include:
- Pancreatic insufficiency and pancreatitis
- Delayed growth and delayed sexual development at puberty
- Enlargement or rounding (clubbing) of the fingertips and toes
- Chronic liver disease and biliary cirrhosis
- Rectal prolapse (protrusion of the rectum through the anus)
- Male infertility
Last Review Date: March 27, 2019