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Symptoms of Immotile Cilia Syndrome

How Does Immotile Cilia Lead to Symptoms?

The Effects of Ineffective Ciliary Motion

The basic problem in Immotile Cilia Syndrome is that because the cilia fail to beat (or, less commonly, they fail to beat in an effective fashion), mucous fails to be cleared out of the respiratory passages. This mucous because stuck in the respiratory passages, and can block these passages. In addition, mucous is an excellent breeding ground for bacteria. Mucous that remains in place for extended periods of time eventually becomes infected with bacteria. The bacteria release chemicals which can damage tissues in the area, especially in the lungs. In addition, inflammatory cells, recruited to help eliminate these bacteria, can release chemicals such as enzymes, which cause further local tissue damage.

Types of Abnormal Cilia

Abnormalities of any of the parts of cilia can lead the cilia being unable to beat. Abnormalities of all the components of cilia have been seen in individuals with Immotile Cilia Syndrome, including absence of the inner or outer dynein arms (or both), absence of the central spokes, absence of the nexin links, and various abnormalities of the microtubules. Abnormalities involving the dynein arms are the commonest cause of Immotile Cilia Syndrome. As all of these abnormalities lead to failure of the cilia to move, all of these abnormalities lead to the same symptoms. In rare instances, cilia from individuals with Immotile Cilia Syndrome look normal using the electron microscope, but video-microscope images show that the cilia fail to beat (for unknown reasons), or do not beat in a coordinated, forwards-and-backwards way. The symptoms in these individuals are the same as in other people with Immotile Cilia Syndrome.

Bacterial Infection in People with Immotile Cilia Syndrome

Four types of bacteria are responsible for most of the persistent respiratory tract infections in people with Immotile Cilia Syndrome:

Bacteria Infection in Immotile Cilia Syndrome Infection in Other People
Hemophilus (Hemophilus influenzae, Hemophilus species) Hemophilus is the most common bacteria causing persistent respiratory infection in people with Immotile Cilia Syndrome. Hemophilus is a fairly common cause of pneumonia. It is a frequent cause of episodes of bronchitis in smokers. It is also a common cause of ear infections and sinusitis.
Streptococcus pneumoniae (or "Pneumococcus") Pneumococcus is another common bacteria causing persistent respiratory infection in people with Immotile Cilia Syndrome. Pneumococcus is the most common cause of pneumonia. It is also a common cause of ear infections and sinusitis.
Staphylococcus aureus Staphylococcus is a less-frequent cause of respiratory infection. Staphylococcus is an uncommon cause of pleurisy and pneumonia. It also causes ear infections and impetigo.
Pseudomonas aeruginosa Pseudomonas tends to be a more common cause of respiratory infections in older individuals with Immotile Cilia syndrome. Pseudomonas is an uncommon cause of infection in otherwise-healthy people. It is a common cause of lung infection in people with Cystic Fibrosis, where it causes the sputum to be green-tinged.

What's The Difference Between a Bacteria and a Virus?

Bacteria are microscopic, one-celled animals which can ingest nutrients, grow, and multiply. Bacteria cause many mild infections, like ear infections and bladder infections, as well as serious infections such as pneumonia. However, because they are alive, they can be killed using conventional antibiotics.

Viruses, on the other hand, consist of genetic material (or DNA) surrounded by a coating, or envelope, made of protein. Viruses cannot grow or multiply on their own. Viruses must inject themselves into a living cell, where the virus' DNA can instruct the cell to use the cell's machinery to construct more viruses. Eventually, the cell fills with viruses and bursts, releasing viruses which can infect other cells. Because viruses aren't truly "alive," they cannot be "killed" with conventional antibiotics. However, fortunately, most viral infections are minor, and the viruses can be eliminated by the body's immune system. Common viral infections include colds, influenza, and the stomach flu. A few viruses, such as herpes, can cause persisting infections.

Note: Hemophilus influenza is a bacteria. Influenza virus is a virus which causes influenza.

What are the symptoms of Immotile Cilia Syndrome?

Problems in the Lungs Because the cilia don't move mucous in people with Immotile Cilia Syndrome, the only way people with Immotile Cilia Syndrome can clear mucous out of the lungs is by coughing. Because of this, people with Immotile Cilia Syndrome usually cough more than other people. When mucous becomes stuck in the bronchial passages, a number of problems can develop:
  • As mucous in the bronchial passages become infected and thicker, individuals with Immotile Cilia Syndrome can start coughing up thick mucous, called phlegm or sputum. Because increased mucous is constantly present in the bronchial tubes of people with Immotile Cilia Syndrome, people with this condition always have some bacteria in the bronchial tubes. The permanent presence of bacteria in the bronchial tubes is called chronic bacterial infection. People with Immotile Cilia Syndrome usually have chronic infection with the same bacteria for extended periods of time. Mucous plugging the bronchial tubes at birth can cause temporary difficulty breathing and even collapse of the alveoli the bronchial tube are supplying (this is caused atelectasis). Many babies with Immotile Cilia have temporary difficulty breathing and may need oxygen, for a period after birth.
  • Bronchial tubes can become narrowed because of the mucous, causing difficulty breathing or wheezing .
    • Signs of difficulty breathing include having the little spaces between the ribs (or at the upper part of the stomach, below the rib cage, or at the hollow at the front of the neck) being sucked in with each breath, or breathing very fast.
    • Wheezing is a whistling sound which comes from the chest and which is usually heard while the person is breathing out. 
  • Viral infections, such as colds or "the flu" (influenza) increase production of mucous in the respiratory passages in everyone. Viral infections, and the increased amount of mucous, promote the growth of bacteria in the bronchial tubes. This can cause people with Immotile Cilia Syndrome to have recurring episodes of bronchial infection, or bronchitis. Symptoms of bronchitis include increased frequency of cough, increased amounts of sputum production (or a change in the colour of the sputum, or the sputum becoming thicker), difficulty breathing, wheezing, and/or fever.
  • When mucous remains lodged in, and blocks a bronchial tube for a prolonged period of time, infection can spread throughout the lung tissue beyond the site of obstruction. This is known as lung infection, or pneumonia. Immotile Cilia Syndrome is a well-recognized cause of recurrent pneumonia in children. The lungs have 6 lobes (3 on each side). The bronchus going to the right middle lobe (or the left middle lobe, in people with Kartagener's Syndrome) is particularly long and narrow, making it particularly prone to becoming blocked with mucous. The right middle lobe is the commonest location for pneumonia in people with Immotile Cilia Syndrome.

  • When mucous completely blocks a bronchial tube for a very long time and not removed, the air is gradually absorbed from the air sacs beyond the site of obstruction while the lung tissue can fill with mucous and infected secretions. This can cause this lung tissue to become collapsed, and turn into a hard, airless lung scar. Once again, the right middle lobe is the most frequent location for a lung scar.
  • When the bronchial tubes remain chronically infected, bacteria and inflammatory cells can release chemicals which can damage the walls of the bronchial tubes. As the bronchial tubes become increasingly damaged, their walls soften, and the bronchial tubes expand. The bronchial tubes become baggy and floppy. This leads to more harmful effects:
    • More mucous can accumulate in pouches and crevices in the baggy, soft, bronchial tube walls;
    • During coughs, the bronchial tube wall tends to collapse, trapping mucous inside, rather than acting as a rigid tube through which mucous can be expelled.
    This leads to more mucous accumulating in the bronchial tubes, producing a vicious cycle, where increasing amounts of infected mucous produce further damage and softening of the bronchial tube walls, leading to the accumulation of more mucous. The presence of soft, dilated bronchial tubes is called Bronchiectasis.
Normal airway Bronchial tubes with Bronchiectasis
Normal Airway (bronchial tubes and air sacs) Airway with Bronchiectasis (note that the airways are baggy and enlarged, and the collections of mucous in pockets formed by the baggy airways)

The symptoms of bronchiectasis include frequent cough, persistent production of larger amounts of sputum, and shortness of breath (particularly with exercise). Bronchiectasis is probably the most serious complication of Immotile Cilia Syndrome, as the soft, mucous-filled bronchial tubes interfere with air flow in and out of the lungs, and large amounts of mucous in the bronchial tubes can completely block the tube, promoting the development of pneumonia and lung scarring.

Are Immotile Cilia Syndrome and Cystic Fibrosis Related?

People with Cystic Fibrosis produce mucous which is abnormally thick, so it tends to become stuck in many of the same places which are problems in people with Immotile Cilia Syndrome, including the bronchial tubes and sinuses. In Cystic Fibrosis, the cilia are normal. Cystic Fibrosis, like Immotile Cilia Syndrome, is a genetic condition, but it is considerably more common, with an incidence of 1 in 2,000 people. 85% of people with Cystic Fibrosis also have problems with mucous obstructing the pancreas, leading to severe digestive problems, such as weight loss or poor weight gain, and frequent stools. Because Cystic Fibrosis is a much more common condition, much more research has been, and is being performed to help us understand how the disease can progress, and how it can be treated. Most of the treatments used for Immotile Cilia Syndrome have been "borrowed" from research on what are the best treatments for Cystic Fibrosis. However, in general, lung disease progresses considerably more rapidly in people with Cystic Fibrosis. Immotile Cilia Syndrome tends to cause more severe sinus and ear problems.

Tell Me About The Symptoms of Immotile Cilia Syndrome In Other Parts of the Respiratory Tract 

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