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ALL / Respiratory Medicine
Kartagener's syndrome
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)
Investigation
- low nasal nitric oxide
- reflects impaired ciliary function and is a sensitive screening marker
- measured by aspirating air from the nasal cavity during velum closure
- abnormal ciliary beat pattern on high-speed video microscopy
- genetic testing
+PassMedicine Notes
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
- dextrocardia or complete situs inversus
- bronchiectasis
- recurrent sinusitis
- subfertility (secondary to diminished sperm motility and defective ciliary action in the fallopian tubes)
- low nasal nitric oxide
- reflects impaired ciliary function and is a sensitive screening marker
- measured by aspirating air from the nasal cavity during velum closure
- abnormal ciliary beat pattern on high-speed video microscopy
- genetic testing
