Question

Primary ciliary dyskinesia (PCD) is a rare genetic disease. Affected individuals exhibit impaired functioning of ciliated cells.

Answer 1

Answer: The respiratory wall or mucosa is made up of the epithelium and supporting lamina propria. The epithelium of respiratory tract is tall columnar pseudostratified with CILIA and goblet cells The cilia aids in sweeping away dusts and bacteria that adheres to the mucous on the epithelium. Therefore people with Primary ciliary dyskinesia are prone to Respiratory infections.

Explanation: Primary ciliary dyskinesia also called immotile ciliary syndrome is a rare genetic disease that affects the movement of cilia lining the respiratory tract. The major consequences of this dysfunction is reduced or absent mucus clearance from the lungs which subsequently leads to chronic recurrent respiratory infections

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Answer 2

Answer:

Cilia are motile in the lungs responsible for keeping the airways clear of dirt and mucus by their characteristic beating motion and rhythmic waving allowing a person to breathe easily and without irritation. These are present in both the lungs as well as middle ear.

As in Primary ciliary dyskinesia , there are defects in the action of cilia in the lining of the respiratory tract , middle ear , sinuses , eustachian tube etc the cilia cannot peforms its regular role .Infections can lead to an irreversible scarring and obstruction in the bronchi resulting in :

1) Shortness of breath.

2) Recurring chest colds.

3) Sinusitis.

4) Coughing , gagging , choking,

5)Middle ear infections