Cystic fibrosis is the most frequent lethal genetic disease of childhood. Formerly known as cystic fibrosis of the pancreas, this entity has increasingly been labeled simply "cystic fibrosis" (or "mucoviscidosis" in Europe).

Manifestations relate not only to the disruption of exocrine function of the pancreas but also to intestinal glands (meconium ileus), biliary tree (biliary cirrhosis), bronchial glands (chronic bronchopulmonary infection with emphysema), and sweat glands (high sweat electrolyte with depletion in a hot environment). First, CF was considered a childhood disease. The median survival age was 8 years. Today, it is 30 years. (The term "median" means that there is half of the population above that number and half below.)

At first the disease was treated phenomenologically, without understanding of the underlying causes. Recent advances have made the mechanisms responsible for CF much clearer; the lack of CFTR (a protein) causes improper regulation of the chloride channel; chloride (Cl) is prevented from leaving the cell.

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