What proportion of patients with primary ciliary dyskinesia have this syndrome?
50%. Liberality is random.
What are the three classic clinical features of primary ciliary dyskinesia?
Chronic sinusitis, bronchiectasis and infertility (males), increased risk of ectopic pregnancy (females)
What is the pathologic abnormality responsible for this condition?
Structural ciliary abnormalities including: complete or partial absence of dynein arms; radial spoke defect; microtubular transposition
CT confirms situs inversus (mirror image of thoracic and upper abdominal content). The lung window setting the varicose to cystic bronchiectasis is well demonstrated. Also, the tree-in-bud sign is well visible in both lower lobes suggests current endobronchial infection.