Answer for CoW 14 May 2017
Kartagner ‘s syndrome
Findings
Chest radiography showing dextrocardia with aortic arch lying on right side of trachea with cystic bronchiectatic changes.Stomach bubble is to the anatomic right- situs inversus viscerum. Xray PNS showing sclerosed bilateral maxillary sinus.
Discussion
Kartagner ‘s syndrome - Triad of bronchiectasis, sinusitis, and situs inversus was first described by Siewert in 1903, although its usual eponym is Kartagener’s syndrome – after the Swiss paediatrician . A disease with congenital and generalised non-functioning of the cilia . About 50% of the people affected with primary ciliary dyskinesia have situs inversus. The tracheobronchial tree is ciliated to the level of the respiratory bronchioles, each ciliated cell having about 200 cilia. Mucociliary transport in the respiratory tract is important for normal respiratory function and resistance to respiratory infection . The typical clinical picture of PCD is a chronic productive cough which can usually be traced back to early childhood or infancy, chronic rhinitis often with nasal polyposis, chronic or recurrent maxillary sinusitis, and frequent ear infections in childhood. Bronchiectasis is not present at birth, but may develop early, sometimes even in childhood. The most common respiratory pathogens are Haemophilus Influenzae and Streptococcus pneumoniae. Most males are sterile, but many females have a lowered fertility. About 50% of patients have situs inversus viscerum. Patients with suspected primary ciliary dyskinesia should have their mucociliary clearance measured, and the cilia should be examined by microscopy. Nasal mucociliary clearance can be measured by the ‘nasal saccharin transit time’ test in which a saccharin pellet is placed on the anterior end of the inferior turbinate and the time taken for the subject to notice the taste is recorded.
Findings
Chest radiography showing dextrocardia with aortic arch lying on right side of trachea with cystic bronchiectatic changes.Stomach bubble is to the anatomic right- situs inversus viscerum. Xray PNS showing sclerosed bilateral maxillary sinus.
Discussion
Kartagner ‘s syndrome - Triad of bronchiectasis, sinusitis, and situs inversus was first described by Siewert in 1903, although its usual eponym is Kartagener’s syndrome – after the Swiss paediatrician . A disease with congenital and generalised non-functioning of the cilia . About 50% of the people affected with primary ciliary dyskinesia have situs inversus. The tracheobronchial tree is ciliated to the level of the respiratory bronchioles, each ciliated cell having about 200 cilia. Mucociliary transport in the respiratory tract is important for normal respiratory function and resistance to respiratory infection . The typical clinical picture of PCD is a chronic productive cough which can usually be traced back to early childhood or infancy, chronic rhinitis often with nasal polyposis, chronic or recurrent maxillary sinusitis, and frequent ear infections in childhood. Bronchiectasis is not present at birth, but may develop early, sometimes even in childhood. The most common respiratory pathogens are Haemophilus Influenzae and Streptococcus pneumoniae. Most males are sterile, but many females have a lowered fertility. About 50% of patients have situs inversus viscerum. Patients with suspected primary ciliary dyskinesia should have their mucociliary clearance measured, and the cilia should be examined by microscopy. Nasal mucociliary clearance can be measured by the ‘nasal saccharin transit time’ test in which a saccharin pellet is placed on the anterior end of the inferior turbinate and the time taken for the subject to notice the taste is recorded.
Note:
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!
We do not discourage differential diagnosis. But all the differentials must satisfy the findings noted in the case.
If you feel you have answered rightly but cannot find your name in the above list, please call 09551942599.
Did you Know?
The order in which the names appear in this winner's list is based on the time of submission. The first person to send the correct answer gets his/her name on top of the list!