Cystic fibrosis

Definition of Cystic fibrosis

Cystic fibrosis is an inherited disease that causes thick, sticky mucus to build up in the lungs and digestive tract. It is one of the most common chronic lung diseases in children and young adults, and may result in early death.

Causes, incidence, and risk factors

Cystic fibrosis (CF) is caused by a defective gene which causes the body to produce abnormally thick and sticky fluid, called mucus. This mucus builds up in the breathing passages of the lungs and in the pancreas, the organ that helps to break down and absorb food.

Symptoms

Because there are more than 1,000 mutations of the CF gene, symptoms differ from person to person.

Signs and tests

A blood test is available to help detect CF. The test looks for variations in a gene known to cause the disease. Other tests use to diagnose CF include:

Treatment

An early diagnosis of CF and a comprehensive treatment plan can improve both survival and quality of life. Follow-up and monitoring are very important. If possible, patients should be cared for at cycstic fibrosis specialty clinics, which can be found in many communities. When children reach adulthood, they should transfer to a cystic fibrosis specialty center for adults.

Expectations (prognosis)

Most children with cystic fibrosis are fairly healthy until they reach adulthood. They are able to participate in most activities and should be able to attend school. Many young adults with cystic fibrosis finish college or find employment.

Review

David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc., and Neil K. Kaneshiro, MD, MHA, Clinical Assistant Professor of Pediatrics, University of Washington School of Medicine. – 7/14/2010

Clubbing
Postural drainage
Clubbed fingers
Cystic fibrosis

ADAM Medical Encyclopedia

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