1/11. bronchiolitis in Kartagener's syndrome.The association of diffuse bronchiolitis in patients with Kartagener's syndrome (KS) has not been reported previously. The aim of this study was to present the morphological characteristics of bronchiolitis in patients with KS. Eight patients (four males, four females; mean age 37.9 /-18.7 yrs), clinically diagnosed as KS with the classical triad of chronic pansinusitis, bronchiectasis and situs in versus with dextrocardia, were evaluated. Routine chest radiography showed bronchiectasis and dextrocardia in all patients. Chest computed tomography (CT) showed diffuse centrilobular small nodules up to 2 mm in diameter throughout both lungs in six out of eight patients. Pulmonary function tests revealed marked obstructive impairment in all patients (forced expiratory volume in one second 57.0 /-11.3%, residual volume/total lung capacity 45. /-12.7%, maximum midexpiratory flow 0.92 /-0.72 L x s(-1), forced vital capacity 74.1 /-12.2% (all mean /- SD)). The examination of cilial movement of the bronchus revealed immotility in all of the five patients examined. The ultrastructure showed ciliary dynein arm defects in all patients. Histopathological examination of lung specimens obtained at autopsy or by video-assisted thoracoscopic surgery showed obliterative thickening of the walls of the membranous bronchioli with infiltration of lymphocytes, plasma cells and neutrophils, but most of the distal respiratory bronchioli were spared and alveolar spaces were overinflated. Pathologically, the diffuse centrilobular small nodules on the chest CT mainly corresponded to membranous bronchiolitis. This is the first report demonstrating that the association of diffuse bronchiolitis might be one of the characteristic features of the lung in Kartagener's syndrome.- - - - - - - - - - ranking = 1keywords = dextrocardia (Clic here for more details about this article) |
2/11. Anaesthesia for Kartagener's syndrome.Kartagener's syndrome is a hereditary syndrome involving a combination of dextrocardia (situs inversus), bronchiectasis and sinusitis, transmitted as an autosomal recessive trait. We describe a patient who had three anaesthetics over a period of a few months. Discussion relates to anaesthetic considerations in the syndrome and to recent findings relating to the molecular mechanisms of left-right development.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
3/11. General anaesthesia for lobectomy in an 8-year-old child with Kartagener's syndrome.Kartagener's syndrome (KGS) is an autosomal recessive disorder characterized by the clinical triad of bronchiectasis, sinusitis and dextrocardia (situs inversus). Reports of general anaesthesia in a child with KGS are rare in medical literature. We describe the case of a young boy who underwent lobectomy under general anaesthesia using a single lumen tube. Anaesthetic implications of this syndrome are mentioned. Key points in the management of anaesthesia in KGS are discussed.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
4/11. Radiofrequency catheter ablation of an accessory pathway in a patient with Wolff-Parkinson-White and Kartagener's syndrome.We report a case of a manifest left free wall accessory pathway in a patient with Kartagener's syndrome and recurrent episodes of orthodromic atrioventricular reentrant tachycardia. To the best of our knowledge, it is the first report of wolff-parkinson-white syndrome associated with Kartagener's syndrome. situs inversus and mirror image dextrocardia occurred with no additional detectable cardiac structural abnormalities. Diagnostic and therapeutic electrophysiological study was carried out via transaortic approach and a left-to-right reversal of monoplane fluoroscopic image.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
5/11. Anaesthetic considerations in Kartagener's syndrome -- a case report.Kartagener's syndrome is a rare disorder characterized by the triad of situs inversus, including dextrocardia, bronchiectasis and paranasal sinusitis. We report the anaesthetic management of a patient with Kartagener's syndrome and postrenal transplant immunosuppression, presenting for repair of uterovaginal prolapse. Combined spinal epidural anaesthesia was administered to this patient. The anaesthetic considerations of this rare disorder and the relative advantages of the regional technique over general anaesthesia in this situation are discussed.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
6/11. kartagener syndrome: an unusual cause of respiratory distress in the newborn.BACKGROUND: kartagener syndrome (KS) a rare genetic disorder belongs to a group of disorders referred to as primary ciliary dyskinesia (PCD) where the cilia covering the respiratory epithelium is either immotile or beat in an uncoordinated fashion. It is characterized by a triad of dextrocardia (with or without situs inversus), chronic sinusitis and bronchiectasis as a result of poor mucociliary clearance of mucus and bacteria. This may lead to respiratory distress in the newborn period. METHOD: This is a case report of a 14-day old male who presented with respiratory distress (which was noticed soon afterbirth) and features suggestive of KS. CONCLUSION: KS should be suspected in neonates presenting with respiratory distress, pneumonia and no risk factors for infection.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
7/11. Kartagener's syndrome with motile cilia and immotile spermatozoa: axonemal ultrastructure and function.A 35-yr-old infertile man with chronic sinobronchial disease and dextrocardia (Kartagener's syndrome) was found to have immotile sperm and motile nasal cilia in vitro. Ciliary beat frequency in vitro was normal, but in vivo nasal mucociliary clearance was markedly prolonged. Quantitative electron microscopy demonstrated a severe reduction in spermatozoal outer and inner dynein arms compared with normal (p less than 0.001) but normal numbers of outer doublets, central microtubules, and radial spokes were seen. In 2 samples of nasal cilia collected 14 months apart, the number of inner dynein arms was significantly reduced from normal (p less than 0.001), but normal numbers of radial spokes and microtubule structures were seen. Ciliary outer dynein arms were slightly reduced in 1 specimen (p less than 0.001) but were normal in the other. It is suggested that the reduction in the number of ciliary inner dynein arms does not affect ciliary motility in vitro but that, under the increased load of mucus in vivo, this defect prevents the cilia from functioning normally. The difference in axonemal ultrastructure between cilia and spermatozoa from the same patient further suggests a separate genetic control of their structural components.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
8/11. Two cases of Kartagener's syndrome.Two cases of the syndrome of bronchiectasis, chronic sinusitis and dextrocardia with situs inversus (Kartagener's syndrome) are described. This rare disorder may be important in further understanding of cilial function in humans.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
9/11. Unusual inheritance of primary ciliary dyskinesia (Kartagener's syndrome).Primary ciliary dyskinesia syndrome is characterised by chronic sinusitis, bronchiectasis, and, in 50% of cases, dextrocardia. It is generally believed to be inherited as an autosomal recessive disorder. In this report, we describe a family consisting of a mother and her five male children, the offspring of three different fathers, all of whom have this syndrome. This argues for either an X linked or autosomal dominant pattern of inheritance. Cytogenetic and FISH (fluorescent in situ hybridisation) analyses were done on the mother and one son and were found to be normal.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
10/11. Anomalous right coronary artery in a patient with Kartagener's syndrome.We report a case of an anomalous right coronary artery arising from the morphological left sinus of valsalva in a patient with Kartagener's syndrome. literature review has revealed only a small number of cases of anomalous coronary arteries in patients with dextrocardia and none previously reported in Kartagener's syndrome.- - - - - - - - - - ranking = 0.5keywords = dextrocardia (Clic here for more details about this article) |
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