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Query: UMLS:C0032285 (pneumonia)
 54,520 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The differential diagnosis of a sinubronchial syndrome includes cystic fibrosis, allergic bronchopulmonic aspergillosis, Kartagener's syndrome, hypogammaglobulinemia and bronchioli-associated processes. The latter include cryptogenic organising pneumonia, constrictive bronchiolitis obliterans as well as the respiratory bronchiolitis. Our case presented a persistent interstitial shadow in the x-rax of the thorax and a relevant, mixed ventilation disturbance in lung function. A surgically performed lung biopsy finally allowed the diagnosis of diffuse panbronchiolitis, which is seldom found in central Europe. This disease is of unknown etiology and responds to long-term macrolide therapy.
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PMID:[A 63 year old Turkish woman with recurrent pneumonia]. 1730 10

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.
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PMID:Primary ciliary dyskinesia: considerations regarding six cases of Kartagener syndrome. 1802 60

Kartagener syndrome is a rare, ciliopathic, autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract and fallopian tube. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis caused by pseudomonal infection. Situs inversus can be seen in about 50% of cases. Diagnosis can be made by tests to prove impaired cilia function, biopsy, and genetic studies. Treatment is supportive. In severe cases, the prognosis can be fatal if bilateral lung transplantation is delayed. We present a case of a 66-year-old woman with chronic recurrent upper respiratory infections, pseudomonal pneumonia, and chronic bronchiectasis who presented with acute respiratory failure. She was diagnosed with Kartagener syndrome based on her clinical presentation and genetic studies. She expired on ventilator with refractory respiratory and multiorgan failure.
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PMID:Kartagener syndrome. 2140 91

Kartagener's syndrome is a rare autosomal recessive genetic disorder that causes a defect in the action of the cilia lining the respiratory tract. Patients usually present with chronic recurrent rhinosinusitis, otitis media, pneumonia, and bronchiectasis caused by Pseudomonas infection. In this case report, we present a 12-year old female with Kartagener's syndrome from Tikur Anbessa Teaching Hospital which to our knowledge is the first of it's kind to be reported in Ethiopia. The clinical and imaging findings are discussed.
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PMID:KARTAGENER'S SYNDROME: A CASE REPORT. 2747 30


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