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: Akijas Nilkis
Country: Montenegro
Language: English (Spanish)
Genre: Software
Published (Last): 6 January 2010
Pages: 40
PDF File Size: 15.96 Mb
ePub File Size: 19.29 Mb
ISBN: 417-9-40488-366-3
Downloads: 3046
Price: Free* [*Free Regsitration Required]
Uploader: Aralkree

Management of bronchiectasis and chronic suppurative lung disease in Indigenous children and adults from rural and remote Australian communities.

CT tends to demonstrate bronchiectasis which may be variable in severity. About Blog Go ad-free. Check for errors and try again. 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.

Cilia, primary ciliary dyskinesia and molecular genetics.


Q J Med, 67pp. The patient reported a history of symptomatic focal epilepsy secondary to brain abscess at age 23 as a consequence of a previous enfermedas complication the etiology was not proven. Eur J Respir Dis, 66pp.

On the other hand, thin-section chest CT is the gold standard to detect bronchiectasis, although thick-section CT can also be used. The annual decrease in forced expiratory volume in the first second FEV 1 has been calculated at 0. Late presentation of Kartagener’s syndrome. En envermedad presente articulo se describe la compleja interrelacion entre la variacion genetica y un tratamiento inespecifico apropiado del sindrome.


There was a problem providing the content you requested

Case 6 Case 6. Additionally, a tomography showed findings that suggested bilateral maxillary antrostomy by endoscopic intervention. Otolaryngol Head Neck Surg,pp. Thorax, 49pp.

Log in Sign up. Own elaboration based on Lucas et al. No images or control laboratory exams were made. Articles Cases Courses Quiz. Paranasal sinus tomography with thickening of the mucosal lining, partially occupied by a soft tissue density mass.

If a person with ehfermedad respiratory infections attends consultation, and also presents structural alterations in the lungs, PKD should be considered. Reduction enfedmedad bronchial subdivision in bronchiectasis. Ciliary movement depends on the kattagener configuration of the cilia, which is formed by arrangements of ten pairs of microtubules, assembled by various proteins such as dynein and others.

Knowing left from right: The differential diagnoses that should be considered depend on the moment of symptom onset: 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.

Chest radiographic findings depend on the severity of underlying bronchiectasis. Bronchopulmonary symptoms in primary ciliary dyskinesia. 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 clinical pictures.

A coronal view of bi-lobed right lung; B sagittal view of tri-lobed left lung. Thank you for updating your details. Imaging plays a key role in proving the kaftagener findings that support KS.


Síndrome de Kartagener. Diagnóstico en una paciente de 75 años | Archivos de Bronconeumología

The infectious respiratory compromise presented by patients with KS can be explained by cilia alteration, enfermsdad leads to the malposition of some organs, as well as to structural and functional alteration of others. To improve our services and products, we use “cookies” own or third parties authorized to show advertising related to client preferences through the analyses of navigation customer behavior.

Case 7 Case 7. Leigh 47 Estimated H-index: BodoutchianHitender JainTania Velez.

Case reports

KS is part of the Enfermefad spectrum related to an autosomal recessive genetic disorder that affects ciliary motility and predisposes to problems of laterality, rhinosopulmonary infections and impaired fertility. Prognostic Value of Frequent Exacerbations in Berdon 9 Estimated H-index: He also presented unquantified fever; coughing with greenish expectoration; exertional dyspnea; odynophagia; generalized arthralgia; nasal congestion, and asthenia.

High-resolution computed tomography of the chest with isomerism of the bronchial branching pattern. Synonyms or Alternate Spellings: The Kartagener syndrome KS comprises a triad of situs inversus, bronchiectasis and paranasal sinusitis, which is named after Dr.