Definition

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Normal Cilia  Cilia with PCD

 

Primary

Ciliary

Dyskinesia

inherited, i.e., not due to an outside source such as smoking or infection pertaining to the ciliaimproper movement

 

Kartagener´s Syndrome

The complete triad of PCD with:

  • Situs inversus (reversed organs)
  • chronic sinusitis (sinus infections)
  • chronic bronchitis (lung infections)

What do "Kartagener's Syndrome" and "Primary Ciliary Dyskinesia" mean?

PCD is a genetic disease, affecting the upper and lower airways of the lungs.

Through an inherited defective gene, the cilia in these people are either immotile (unable to move) or dysmotile (they move incorrectly). As a result, it is impossible to keep the lungs and sinuses clear of fluids, which can lead to congestion and infection.

This produces the following symptoms:

  • frequent cough
  • chronic runny nose
  • frequent middle ear infections, sometimes with some hearing loss

 

Normal cilia movement

Cilia with PCD

Die Grafik zeigt zilientragende Zellen und flüssigkeitsproduzierende Becherzellen. Der Zilienschlag und somit der Sekrettransport erfolgt rhythmisch in Richtung Mund oder Nase.

50% of PCD patients have Situs inversus, a situation where their organs are completely reversed (heart on the right, etc.).
For these patients, the disease is called Kartagener's Syndrome.

 

Bei PCD sind die sekretproduzierenden Becherzellen vermehrt und die Zilien überwiegend unbeweglich.