Kartagener syndrome is a subset of primary ciliary dyskinesia, an autosomal recessive condition characterised by an abnormal ciliary structure or function. Kartagener syndrome, current data on a classical disease. Case report. Keywords: Kartagener Syndrome; Primary. Resumen El sindrome de Kartagener es una enfermedad genetica poco frecuente que se hereda de forma autosomica recesiva, con una afectacion progresiva.

Author: Kagakazahn Taubar
Country: Austria
Language: English (Spanish)
Genre: Politics
Published (Last): 7 December 2006
Pages: 77
PDF File Size: 4.86 Mb
ePub File Size: 19.47 Mb
ISBN: 834-2-29825-990-3
Downloads: 71953
Price: Free* [*Free Regsitration Required]
Uploader: Brazil

Karttagener is part of the PKD spectrum related to an autosomal recessive genetic disorder that affects ciliary motility and predisposes to problems of laterality, rhinosopulmonary infections and impaired fertility.

Kartagener’s syndrome is an inherited disease characterized by the clinical triad of bronchiectasis, sinusitis and situs inversus caused by an ultrastructural defect in the cilia that results in impaired mucociliary clearance.

Situs inversus totalis – a case report.

Case reports

Late presentation of Kartagener’s syndrome. Am Rev Respir Dis,pp. The movement of the fluid allows for different substance concentrations which accumulate on one side only and, therefore, for the expression of different genes in each hemi-embryo.

Kartagenwr it at Google Books – Find it at Amazon. Reduction in bronchial subdivision in bronchiectasis. He also presented unquantified fever; coughing with greenish expectoration; exertional dyspnea; odynophagia; generalized arthralgia; nasal congestion, and asthenia. The incidence of primary ciliary dyskinesia PKD is estimated at 1 case per 10 to 20 births based on surveys of situs inversus and bronchiectasis ; however, its frequency is difficult to determine due to the diagnostic difficulty related to nonspecific ee pictures.


No images or control laboratory exams were made. En el presente articulo se describe la compleja interrelacion entre la variacion genetica y un tratamiento inespecifico apropiado del sindrome.

Own elaboration based on Lucas et al.

Sensitivity and specificity for diagnosing primary ciliary dyskinesia. Thorax, 49pp. Primary nasal origin of exhaled nitric oxide and absence in Kartagener’s syndrome. Diagnosis in a year-old woman.

Primary d dyskinesia in adults. Once the over-aggregated infectious process was confirmed in a patient with risk factors for pseudomonas infection, antibiotic therapy was adjusted with piperacillin tazobactam 4.

I Mitteilung Bronchiekctasien bei situs viscerum inversus. Manes Kartagener 1who described the presence of paranasal sinusitis in patients in association with situs inversus and bronchiectasis observed by Siewert in Noone 30 Estimated H-index: Alterations in the genes that cause PKD lead to random situs half of the individuals with situs inversus and the other half with situs solitus.

Kartagener syndrome, current data on a classical disease.

If a person with recurrent respiratory infections attends consultation, and also presents structural alterations in the lungs, PKD should be kwrtagener. Log in Sign up. While investigating rare cases of men with immotile spermatozoa, he noted a frequency of situs inversus and dysfunction of other ciliated organs 8. Finally, prognosis depends on lung involvement.


Clinical suspicion of KS occurs when the heart is auscultated on the right and the liver is palpated on the left.

Eur J Respir Kartgaener, 66pp. Middle lobe and lingula. A clinical study of 27 patients. Typical symptoms are persistent moist cough, sputum production, nasal congestion and chronic wheezing, with recurrent ear, nose and throat ENT infections, being the most frequent reason for consultation. About Blog Go ad-free.

Kartagener syndrome | Radiology Reference Article |

Case 5 Case 5. A congenital ciliar abnormality as an etiogenic factor in chronic airway infections and male sterility. Facultad de Medicina, Universidad Nacional de Colombia; Case 9 Case 9. It was Afzelius who made the connection with the syndrome previously described by Kartagener. Berdon 9 Estimated H-index: Confirmation is achieved through imaging methods that prove visceral heterotaxia, indirect methods related to scan of ciliary malfunction nasal nitric oxide, video microscopy and ciliary biopsy that demonstrates the defect of the ciliary ultrastructure.