Immotile cilia syndrome is a chronic lung disease that occurs when cilia are unable to move. This condition is a rare genetic birth defect. People with this condition have irregular or deficient ciliary motion.
It is also called as Kartagener’s Syndrome, Afzelius’ syndrome, Kartagener’s triad, Primary Ciliary Dyskinesia, Zivert-Kartagener triad and Zivert’s syndrome.
Genetic defects in the structure and function of sensory and motile cilia result in multiple ciliopathies.
Basically, cilia are tiny hair like structures that are present on the surfaces of various types of cells.
The main function of cilia is to shift mucus beyond the respiratory passages. When you get infected, production of mucus increases.
This abnormal mucus move ineffectively or cannot move so that it becomes immovable and blocks respiratory tract leading various complications.
This disorder is primarily due to a ciliary defect. People who have this syndrome are born with this condition.
Symptoms of Immotile cilia syndrome:
- Recurrent or chronic bronchitis
- Recurrent or chronic sinusitis
- Chronic rhinitis
- Olfactory impairments
- Frequent lung infection such as pneumonia
- Frequent ear infection
The most common symptom of this disorder in adults is persistent upper or lower airway disease. Clubbing of the fingers occurs as the result of chronic hypoxia from bronchiectasis.
Males experience infertility and in females it is not usually a characteristic because egg transportation into the fallopian tube is associated with muscle contraction than with ciliary movement.
Treatment for Immotile cilia syndrome:
Antibiotics are used to treat sinusitis and prevent the recurrent infection. Occasionally, surgery is considered to treat sinusitis that involves the removal of nasal polyps.
Inhaled medications and respiratory therapy can help if chronic lung disease develops.
Small tubes are placed through the eardrums to permit infections and fluid to emerge from the middle ear.
Chest physiotherapy is considered to loose the secretion of mucus and is the most commonly considered therapy.
If this therapy is started early in the life, it helps to prevent or stop the development ofbronchiectasis.
Intracytoplasmic sperm injection or ICSI is considered for men to treat infertility. This procedure allows dysmotile or immotile sperm to fertilize the egg.
This procedure involves, injecting a single sperm into an egg for fertilization to take place. But the chancing of having a child is very rare in people with Immotile cilia syndrome.