Additional immune dysfunction discovered in people with Cystic Fibrosis

enniob

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A BREAKTHROUGH has allowed scientist to discover that adults with Cystic Fibrosis may lack a very important cell surface molecule that is crucial for proper immune system defense. The scientists found that the immunological cell surface molecule HLA-DQ is reduced or absent in many people with CF. The defect is seen in all relevant white blood cells found in the blood and in the lungs. Hence, all of the relevant antigen-presenting cells of the immune system are affected. The HLA-DQ molecule presents crucial parts of invading microbes to immune cells which can then eliminate the microbes.

In further studies the scientists aim to develop a rapid test system for the immune dysfunction that may be of great importance for diagnosis and treatment of cystic fibrosis.

This follows another discovery published recently on the finding that CF lungs do not produce a very important and almost undetectable thin layer of fluid that lines the lungs, called "airway surface liquid" (ASL for short). This liquid and gelatin substance is thought to trap inhaled particles and bacteria, and kill micro-organisms before little hairs called cilia sweep the mucus out of the lungs.

These 2 discoveries combined offer great hope in advancing treatment methods for common microbes like Pseudomonas in lungs of people with CF.

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- chris
 
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