Kartagener Syndrome.


 

A 43-year-old man presented with cough and expectoration for 7 days. Since birth he had had recurrent episodes of respiratory tract infections, for which he used to take drugs from his family doctor. He was married for 15 years but had borne no children. On examination, apex beat was in the sixth right intercostal space with heart sounds being heard on the right side of the chest. Electrocardiogram showed evidence of dextrocardia . Cardiac apex, along with aortic arch and gastric bubble on chest radiograph were seen on the right side . High-resolution CT of chest and upper abdomen showed bronchiectasis with situs inversus totalis . Radiograph and CT of sinuses showed mucosal thickening in maxillary sinuses and hypoplastic frontal sinuses. Fibreoptic bronchoscopy showed transposition of right and left bronchus. Semen analysis (done thrice) showed decreased sperm viability with no motility. Saccharin test for mucociliary clearance gave a time of 48 min (normal <15 min).

k1k2Situs_inversus_due_to_Kartagener's_syndrome

A diagnosis of Kartagener’s syndrome was made. Kartagener’s syndrome is characterised by the triad of chronic sinusitis, bronchiectasis, and situs inversus. It is classified under the group of disorders called primary ciliary dyskinesias. Patients generally present with recurrent upper and lower respiratory tract infection because of ineffective mucociliary clearance. As sperm mortility is dependent on ciliary function, males are generally sterile.

Source: Lancet

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