MRCEM Notes

Kartagener's syndrome

Kartagener's syndrome (also known as primary ciliary dyskinesia) was first described in 1933 and most frequently occurs in examinations due to its association with dextrocardia (e.g. 'quiet heart sounds', 'small volume complexes in lateral leads')

Pathogenesis
  • dynein arm defect results in immotile cilia

Features
  • dextrocardia or complete situs inversus
  • bronchiectasis
  • recurrent sinusitis
  • subfertility (secondary to diminished sperm motility and defective ciliary action in the fallopian tubes)
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