Brazilian Journal of Pulmonology

ISSN (on-line): 1806-3756 | ISSN (printed): 1806-3713

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Primary ciliary dyskinesia: Considerations regarding six cases of Kartagener syndrome

Discinesia ciliar primária: Considerações sobre seis casos da síndrome de Kartagener

Hugo Alejandro Vega Ortega, Nelson de Araujo Vega, Bruno Quirino dos Santos, Guilherme Tavares da Silva Maia

J Bras Pneumol.2007;33(5):602-608

Abstract PDF PT PDF EN Portuguese Text

Primary ciliary dyskinesia (PCD), previously known as immotile cilia syndrome, is an autosomal recessive hereditary disease that includes various patterns of ciliary ultrastructural defects. The most serious form is Kartagener syndrome (KS), which accounts for 50% of all cases of PCD. The incidence of PCD ranges from 1:20,000 to 1:60,000. Since PCD causes deficiency or even stasis of the transport of secretions throughout the respiratory tract, it favors the growth of viruses and bacteria. As a result, patients have lifelong chronic and recurrent infections, typically suffering from bronchitis, pneumonia, hemoptysis, sinusitis, and infertility. Bronchiectasis and other chronic conditions infections can be the end result of the irreversible bronchial alterations, leading to chronic cor pulmonale and its consequences. Only half of the patients affected by PDC present all of the symptoms, a condition designated complete KS, compared with incomplete KS, typically defined as cases in which situs inversus does not occur. The diagnosis is made clinically and confirmed through transmission electron microscopy. Since there is no specific therapy for PCD, it is recommended that, upon diagnosis, secondary infections be treated with potent antibiotics and prophylactic interventions be implemented. In this paper, we report six cases of PCD (five cases of complete KS and one case of KS) and review the related literature, focusing on the diagnostic, therapeutic and clinical aspects of this disease.

 


Keywords: Kartagener syndrome; Ciliary motility disorders; Bronchiectasis; Dextrocardia.

 


Tracheobronchial cystic disease

Doença cística traqueobrônquica

Hugo Alejandro Vega Ortega, Nelson de Araújo Vega, Jece Comparini, Leonardo Vieira do Carmo, Luciano Penha Pereira

J Bras Pneumol.2001;27(6):349-354

Abstract PDF PT

Air cysts abnormally emerging from the trachea and/or larger bronchi is an extremely rare disease. Only a few isolated cases of paratracheal air cysts associated with chronic lung diseases have been described in the literature. The aim of this communication is to describe two cases of this rare, clinical entity in members of the same family. The father of the patients died from this disease at 43 years of age and his two daughters are currently under treatment. The clinical findings are similar to those of chronic pulmonary suppuration syndrome. The diagnosis was established by bronchovideoscopy, axial CT with 3D reconstitution and virtual bronchoscopy. The patients are under observation and palliative treatment is being administered.

 


Keywords: Lung diseases. Cysts. Trachea. Bronchi. Bronchoscopy. Computed tomography.

 


Use of a one-way flutter valve drainage system in the postoperative period following lung resection

Utilização da válvula unidirecional de tórax como sistema de drenagem no pós-operatório de ressecções pulmonares

Nelson de Araujo Vega, Hugo Alejandro Vega Ortega, Alfio José Tincani, Ivan Felizardo Contrera Toro

J Bras Pneumol.2008;34(8):559-566

Abstract PDF PT PDF EN Portuguese Text

Objective: To evaluate pleural drainage using a one-way flutter valve following elective lung resection. Methods: This was a prospective study, with descriptive analysis, of 39 lung resections performed using a one-way flutter valve to achieve pleural drainage during the postoperative period. Patients less than 12 years of age were excluded, as were those submitted to pneumonectomy or emergency surgery, those who were considered lost to follow-up and those in whom water-seal drainage was used as the initial method of pleural drainage. Lung expansion, duration of the drainage, hospital stay and postoperative complications were noted. Results: A total of 36 patients were included and analyzed in this study. The mean duration of pleural drainage was 3.0 ± 1.6 days. At 30 days after the surgical procedure, chest X-ray results were considered normal for 34 patients (95.2%). Postoperative complications occurred in 8 patients (22.4%) and were related to the drainage system in 3 (8.4%) of those. Conclusions: The use of a one-way flutter valve following elective lung resection was effective, was well tolerated and presented a low rate of complications.

 


Keywords: Drainage; Postoperative complications; Thoracic surgery.

 


 

 


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