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.

Cistos aéreos que emergem anormalmente da traquéia e/ou brônquios de maior calibre são doença extremamente rara. Na literatura encontram-se poucas descrições de casos isolados de cistos paratraqueais associados a pneumopatias crônicas. Relatamos dois casos dessa entidade clínica, rara, em membros de uma mesma família: o pai das pacientes faleceu dessa doença com 43 anos de idade e as duas filhas encontram-se em tratamento. O quadro clínico é semelhante ao da supuração pulmonar crônica. O diagnóstico foi firmado através de broncovideoscopia e tomografia computadorizada de tórax com reconstrução em 3D e broncoscopia virtual. As pacientes estão sob observação e tratamento clínico paliativo.

The conventional system of pleural drainage currently in use in the treatment of pleural diseases and in the postoperative period following thoracic surgery is the same as that described by Kenyon in 1916. (1) This method consists IntroductionAfter any surgical procedure involving the opening of the pleura, the thoracic cavity must be drained in order to facilitate adequate pulmonary reexpansion and allow the outflow of blood, fluids and air.Use of a one-way flutter valve drainage system in the postoperative period following lung resection* 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.

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