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Adequacy of Clinical and Radiological Evidence for the Management of Kartagener Syndrome
Himangshu Hazarika1, Garima Talukdar2, Anjan J Talukdar3
1Internal Medicine, Primary Health Centre Bihmari Bongaon, Biswanath Chariali, IND.
Abstract:
Kartagener syndrome, a clinical variant of primary ciliary dyskinesia (PCD), is a rare autosomal recessive genetic disorder caused by defective motility of cilia lining the respiratory tract, fallopian tubes, and middle ear. This results in impaired mucociliary clearance and leads to recurrent upper and lower respiratory tract infections, chronic sinusitis, otitis media, and bronchiectasis. We report a case of a 36-year-old woman with longstanding recurrent sinopulmonary infections, productive cough, hemoptysis, and dyspnea, who was ultimately diagnosed with left lower lobe bronchiectasis and situs inversus totalis. Bronchoscopy with bronchoalveolar lavage grew Pseudomonas aeruginosa. In the absence of advanced confirmatory tests for PCD, such as nasal nitric oxide measurement, saccharin test, high-speed video microscopy, transmission electron microscopy, and genetic analysis, the diagnosis was made on the basis of clinical and radiological findings alone. This case underscores the practical challenges of diagnosing Kartagener syndrome in resource-limited settings with constrained access to advanced diagnostic tools, gold-standard genetic tests, specialized personnel, and comprehensive healthcare infrastructure. In our setting, access was limited to basic hematological and biochemical tests and a conventional chest X-ray. It further examines whether a strong clinico-radiological correlation can be sufficient to guide appropriate treatment and management in such contexts.
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