According to an article from news-medical.net, researchers are beginning to delve into the causes of a rare lung disease called primary ciliary dyskinesia. This research is revealing previously unknown information about what causes this disease, and could hopefully lead to a cure or more effective treatments being developed.
In primary ciliary dykinesia (also known as Kartagener syndrome), the cilia which line the respiratory tract are unable to function normally. The disorder also affects the cilia in the fallopian tubes and the flagella of sperm cells. It is an inherited genetic disorder. In a healthy person, the hairlike cilia move together in unison to direct mucus towards the throat. This movement provides a defense against infection, so any irregularity causes the likelihood of infection in the lungs and respiratory tract to increase greatly. In primary ciliary dyskinesia (PCD), the cilia beat out of sync with one another, and fail to clear the mucus, which is a habitat for infectious microbes. Symptoms can include frequent infection, long term damage to the respiratory system, and loss or impaired sense of smell and hearing. While life expectancy is typically normal for people with PCD, a lung transplant may be required in severe cases. To learn more about this disorder, click here.
So far, research has shown that the cilia in a person with PCD appear to be structured normally, but even a single molecular motor protein that is misshapen can keep the cilia from working normally. There is no cure for PCD at this juncture. While doctors can treat the frequent infections that occur as a result, there is currently no way to get the cilia to function normally again.