Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab

Cystic Fibrosis

  • Manfred Schwab
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_1454-2

Definition

Cystic fibrosis (CF) is a hereditary disease caused by mutations within the ABC transporter-encoding gene ABCC7 (CFTR).

Cystic fibrosis symptoms do not follow the same pattern in all patients but affect different people in different ways and to varying degrees. However, the basic problem is the same – an abnormality in the glands, which produces or secretes sweat and mucus. Sweat cools the body; mucus lubricates the respiratory, digestive, and reproductive systems and prevents tissues from drying out, protecting them from infection. People with cystic fibrosis lose excessive amounts of salt when they sweat. This can upset the balance of minerals in the blood, which may cause abnormal heart rhythms. Going into shock is also a risk. Mucus in cystic fibrosis patients is very thick and accumulates in the intestines and lungs. The result is malnutrition, poor growth, frequent respiratory infections, breathing difficulties, and eventually permanent lung damage. Lung disease is the...

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Copyright information

© Springer-Verlag Berlin Heidelberg 2015

Authors and Affiliations

  1. 1.German Cancer Research Center (DKFZ)HeidelbergGermany