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A Case of Kartagener's Syndrome Presenting With Severe Hypoxemia
Nida Gul1, Felicita M Tayong2,3, Ashbin Sapkota4
1Medicine, Lady Reading Hospital, Peshawar, PAK.
Abstract:
Kartagener syndrome is a rare hereditary disorder that follows an autosomal recessive pattern of inheritance. It falls under the group of primary ciliary dyskinesias (PCDs). It is typically characterized by a classic triad: bronchiectasis, persistent sinus infections, and a reversal of internal organ positioning known as situs inversus. Due to its non-specific symptoms and absence of a simple diagnostic test, Kartagener syndrome is frequently diagnosed late, often after significant respiratory damage has occurred. We report the case of a 15-year-old female patient who presented with recurrent respiratory tract infections, productive cough, fever, and cyanosis. Radiological investigations confirmed the presence of situs inversus, bronchiectasis, and longstanding sinus inflammation, thereby meeting the criteria for a diagnosis of Kartagener syndrome. Arterial blood gas analysis indicated acute on chronic respiratory failure, and sputum culture showed growth of Klebsiella pneumoniae. The patient was managed with bronchodilators, oxygen therapy, antibiotics, and chest physiotherapy. Kartagener syndrome should be suspected in young individuals with chronic respiratory symptoms and situs inversus. Timely diagnosis and appropriate intervention can significantly reduce morbidity and improve patient outcomes.
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