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.
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KS 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. The annual decrease in forced expiratory volume in the first second FEV enffrmedad has been calculated at 0. Thorax, 49pp. Hospital Universitario La Paz. Knowing left from right: Other Papers By First Author.
Inadequate sweeping of pulmonary secretions causes bronchial dilatations or bronchiectasis that are observed as tubular opacities or ovoids of variable sizes in chest x-ray, a less sensitive method for its detection with respect to HRCT.
Middle lobe and lingula. Erythematous oropharynx was observed along with whitish plaques, heart sounds in the right hemithorax, decreased vesicular breath akrtagener in both lungs, occasional wheezing in the right lung dde bibasal stertors. Document obtained during the study. Manes Kartagener 1who described the presence of paranasal sinusitis in patients in association with situs inversus and bronchiectasis observed by Siewert in Short Cases Respir Med, 2pp.
Chest radiographic findings depend on the severity of underlying bronchiectasis.
Kartagener syndrome | Radiology Reference Article |
Zur Pathologhese der Bronchiektasien. For other causes of lower-lobe bronchiectasis which are independent of a situs abnormality, see page on lower lobe bronchiectasis.
European Respiratory Society guidelines for the diagno sis of primary ciliary dyskinesia. Arch Dis Child, 56pp. A coronal view of bi-lobed right lung; B sagittal view of tri-lobed left lung. Respiratory infectious involvement in patients with KS is explained by the alteration of the cilia, which leads both to the malposition of some organs and to the structural and functional alteration of others. Management of bronchiectasis and chronic suppurative lung disease in Indigenous children and adults from rural and remote Australian communities.
Eur Respir J, 12pp. Additionally, a tomography showed findings that suggested bilateral maxillary antrostomy by endoscopic intervention. More on Kartagener’s syndrome and efnermedad contributions of Afzelius and A.
You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Situs and the fact that the organs are usually located to the right or left do not have yet a satisfactory explanation regarding their correlation with KS, and new theories have been proposed in this regard. 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. 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.
Leigh 47 Estimated H-index: La tomografia computarizada demostro dextrocardia y bronquiectasias. In addition, HRCT showed tomographic signs of infectious bronchiolitis.
Ciliary ultrastructure in primary ciliary dyskinesia and other respiratory conditions: You can change the settings or obtain more information by clicking here. Lancet,pp. However, a negative result does not rule out this disease, as not all the genes involved are known to date. Unable to process the form. Eur J Respir Dis, 66pp.
Síndrome de Kartagener. Diagnóstico en una paciente de 75 años | Archivos de Bronconeumología
CT provides good anatomical detail of the specific condition, while MRI can be useful to assess patients with cardiac abnormalities. No gender predilection is recognised. Eur Respir J, 7Medline. Ed you for updating your details. Zur Pathogenese der Bronchiektasien. CT tends to demonstrate bronchiectasis which may be variable in severity. A congenital ciliar abnormality as an etiogenic factor in chronic airway infections and male sterility.
Bronchiectasis in congenital diseases. Read it at Google Books – Find it at Amazon.
Primary ciliary dyskinesia immotile cilia syndrome. This article addresses the general aspects pathophysiology, embryology, clinical presentation and prognosis of the Kartagener syndrome KS.
However, the diagnostic precision of PKD is achieved with the elements mentioned above, all of which are difficult to access. Further work was undertaken by Jennifer Sturgess, an ultrastructuralist from Toronto who identified the radial spoke defect as a common feature of Kartagener syndrome and immotile cilia syndrome.
Laterality depends on enfermerad movement of the cilia present in the primitive node, which are oriented upwards and immersed in extraembryonic fluid; they also move to any side of the extraembryonic fluid that contains multiple substances, contributing to gene expression. Kartagener syndrome, current data on a classical disease. National Journal of Clinical Anatomy. A clinical study of 27 patients. Support Radiopaedia and see fewer ads.