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      Síndrome de Kartagener: relato de caso Translated title: Kartagener Syndrome: a case report

      case-report

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          Abstract

          A Síndrome de Kartagener é causada por uma doença autossômica recessiva rara, que apresenta uma tríade composta por pansinusite crônica, bronquiectasia e situs inversus com dextrocardia. A incidência desta desordem genética é estimada em 1/25.000. Nosso objetivo é, neste trabalho, aduzir dados novos àqueles que pesquisam o assunto, para que estejam atentos, acompanhando os pacientes e esperando o aparecimento eventual desta malformação. Foram realizadas tomografias computadorizadas do tórax, abdome e crânio, além da tomografia computadorizada de alta resolução do tórax, audiometria, imitanciometria e vídeo-naso-fibroscopia.

          Translated abstract

          The Kartagener Syndrome is a rare recessive autossomical disease including the triad: chronical sinusitis, bronchiectasis and situs inversus with dextrocardia. The incidence of this genetical disorder is estimated about 1/25,000. Our purpose in this case report is to include new informations for who search about this syndrome. Tomography of chest, abomen and cranium, audiometry, impedance and nasopharyngoscopy were done.

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          Most cited references14

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          Characterization of a chemotactic defect in patients with Kartagener syndrome.

          Kartagener syndrome (KS) is an autosomally inherited recessive condition characterized by situs inversus, bronchiectasis, and chronic sinusitis. Ciliary dynein, the mechanochemical force generator in ciliary movement, is deficient in patients with KS. We examined blood samples from two patients and tissue biopsy specimens from five patients and found: (1) no significant defect in neutrophil or monocyte chemotaxis in response to formylpeptide chemoattractant; (2) no alterations in centriolar structure, but significantly more centriole-associated microtubules in KS neutrophils and monocytes than in control leukocytes; and (3) a marked reduction in KS fibroblast chemotaxis in response to fibronectin compared with control fibroblasts. The significance of these cellular defects in KS is described.
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            Ultrastructure and function of cilia and spermatozoa flagella in a patient with Kartagener's syndrome.

            A 37-year-old man with the characteristic clinical signs of Kartagener's syndrome, including bronchiectasia, chronic paranasal sinusitis, and situs inversus, was found to have immotile cilia and sperm. Electron microscopy demonstrated that the majority of cilia lacked dynein arms and radial spokes and that various defects of microorgans existed in the sperm. The most frequent defect was total defect of axoneme followed by defect of inner dynein arms in the sperm. These findings suggest an association between the structural abnormality of absent inner dynein arms and immotility of cilia and sperm in Kartagener's syndrome.
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              Primary ciliary dyskinesia.

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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                rboto
                Revista Brasileira de Otorrinolaringologia
                Rev. Bras. Otorrinolaringol.
                ABORL-CCF Associação Brasileira de Otorrinolaringologia e Cirurgia Cérvico-Facial (São Paulo )
                0034-7299
                December 2003
                : 69
                : 6
                : 857-861
                Affiliations
                [1 ] Pontifícia Universidade Católica de São Paulo Brazil
                [2 ] Pontifícia Universidade Católica de São Paulo Brazil
                Article
                S0034-72992003000600021
                10.1590/S0034-72992003000600021
                df05a465-3840-47d1-af4c-46179a7202d3

                http://creativecommons.org/licenses/by/4.0/

                History
                Product

                SciELO Brazil

                Self URI (journal page): http://www.scielo.br/scielo.php?script=sci_serial&pid=0034-7299&lng=en
                Categories
                OTORHINOLARYNGOLOGY

                Otolaryngology
                Kartagener syndrome,Kartagener triad,sinusitis,dextrocardia,síndrome de Kartagener,triade de Kartagener,sinusite

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