Purpose
Kartagener syndrome is part of the larger group of disorders referred to as primary ciliary dyskinesias (about 50% of patients with PCD have situs viscerum inversus and are classified as affected of Kartagener syndrome).
In 1933,
Kartagener described the triad consisting of dextrocardia,
chronic vasomotor rhinitis,
and bronchiectasis as a particular clinicopathological entity.
He also emphasized the familial and hereditary character of this syndrome (it is inherited via an autosomal recessive pattern),
which now bears his name,
Kartagener syndrome.1
Camner et al.
first suggested ciliary...
Methods and Materials
We reviewed all available images from chest x-rays,
chest CT,
paranasal sinus x-rays and sinus CT studies of 12 patients whounderwent rigorous evaluation at our institution.
KS was suspected on the basis of clinical features and/or situs viscerum inversus.
The patients with the suspect or diagnosis of KS,
underwent imaging studies for clinical reasons (ie,
because of chronic cough and/or persistent focal abnormality seen on a chest radiograph that was unresponsive to medical treatment).
The diagnosisof PCD was confirmed on the basis of a strong...
Results
KS may be suspected because of respiratory disease or mirror image arrangement (conventional diagnostic clues).
Diagnosis is frequently made late,
in part because it presents with symptoms (rhinitis,
secretory otitis media,
cough) which are common in children.
The common diagnostic features of KS are: mirror image arrangement ,
continuous rhinorrhoea from the first day of life,
respiratory distress or neonatal pneumonia with no obvious predisposing cause,
chronic productive or “wet” cough, atypical “asthma”,
non-responsive to treatment,
“idiopathic” bronchiectasis,
rhinosinusitis (daily rhinitis is typical,
without remission,...
Conclusion
The probability or diagnostic suspicion of PCD increases when patients have had chronic respiratory infections since birth and present situs inversus.
In a subset of patients with KS,
sinus x-rays,
sinus CT scan and chest x-rays and high-resolutionCT of the chest findings were highlighted in terms of more accurate delineationof the distribution and nature of chronic respiratory disease secondaryto failure of the mucociliary clearance apparatus.
These findingsmay help in the evaluation of patients with a KS phenotype.
Unless appropriate management guidelines are established,
the morbidity...
References
Kartagener M.Zur pathogenese der bronchiectasien.
I Mitteilung:bronchiectasien bei situs viscerum inversus.Betr Klin Tuberk.1933;83:498-501.
Camner P,
Mossberg B,
Afzelius BA.Evidence of congenitally nonfunctioning cilia in the tracheobronchial tract in two subjects.Am Rev Respir Dis.Dec1975;112(6):807-9.
Afzelius BA.A human syndrome caused by immotile cilia.Science.Jul 231976;193(4250):317-9.
Guichard C,
Harricane MC,
Lafitte JJ,
et al.
Axonemal dynein intermediate-chain gene (DNAI1) mutations result in situs inversus and primary ciliary dyskinesia (Kartagener syndrome).Am J Hum Genet2001; 68:1030 -1035
Nadel HR,
Stringer DA,
Levison H,
Turner JA,
Sturgess JM.The immotile cilia syndrome: radiological...