Primary Ciliary Dyskinesia

Primary Ciliary Dyskinesia

What is Primary Ciliary Dyskinesia?

Primary ciliary dyskinesia is a kind of genetic disorder characterized by infections in chronic respiratory tract, abnormally placed internal organs and infertility.

This condition is mainly caused by abnormal flagella and cilia.

What is Cilia in a Cell?

Cilia are fingers like microscopic projections that stick out of the cell surface which is found in airway linings, reproductive system, and other tissues and organs. Flagella are long structures similar to cilia that propel sperm cells forward.

Cilia function by moving back and forth in the respiratory tract in a coordinated manner to transfer mucus towards the throat. This movement helps in eliminating bacteria, fluid and other foreign particles from the lungs. Newborn babies experience difficulties in breathing due to primary ciliary dyskinesia. In these conditions, bacteria remain in the respiratory tract and causes infection due to improper functioning of cilia which plays a major role in erasing fetal fluid from the lungs.

People with primary ciliary dyskinesia also face a chronic cough and year-round nasal congestion. Respiratory tract infections result in a condition known as bronchiectasis which damages bronchi and leads from the windpipe to the lungs and capable of causing other life-threatening breathing complications.

Some individuals develop abnormally placed organs with primary ciliary dyskinesia in their abdomen and chest. The abnormalities arise because of early embryonic development where the difference between the right and left sides of the body arises. According to research, 50% of people have a mirror image reversal of internal organs with primary ciliary dyskinesia.

Diagnosis of primary ciliary dyskinesia

A neonatologist, who is specialized in treating babies suspect Primary ciliary dyskinesia or other lung diseases when a newborn is facing problem in breathing.

A pediatrician, who is specialized in treating children) suspects PCD when a child has chronic sinus or lung infections.

Symptoms of Primary Ciliary Dyskinesia

The sign and symptoms of this syndrome vary from person to person.

  • Sinuses.
  • Wheezing.
  • Shortness of breath.
  • Recurring chest colds.
  • Ear and nose infections.
  • Wet cough from birth or infancy.
  • Lungs and respiratory tract disorders.

Treatment for Primary Ciliary Dyskinesia

Unfortunately, there is no particular treatment discovered to cure faulty airway cilia. Hence, treatment of this syndrome aims at complications and symptoms. Other treatment includes:

  • Eliminating the trapped mucus from the airways.
  • Treating and controlling the sinus, lungs and ear infections.
  • Few antibiotics, anti-inflammatory, and bronchodilators medicines are prescribed to treat certain lung infections, for clearing mucus blocked in the airways, and reducing swelling of the bronchial tubes.
  • Standard treatments for lung disorders and other breathing problems could be treated by aerobic exercise and other chest physical therapy (PCT).
  • Other treatments and oral medicines are given for the patients to clears mucus from bronchi which are necessary for people with this disorder.

This was a brief introduction to Primary ciliary dyskinesia. To know more about primary ciliary dyskinesia and other respiratory tract diseases, visit BYJU’S.

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