Kartagener Syndrome, case report

Abstract

Introduction: Kartagener Syndrome is a clinical variant of primary ciliary dyskinesia It is a rare autosomal recessive disease, one in every 32,000 births, characterized by a triad of bronchiectasis, chronic sinusitis and situs inversus partial o total. We present the case female with this disease and review the diagnostic, therapeutic strategies and prognosis. Case description: A 20-year-old female student denies toxic habits with history of repeated respiratory infections since childhood, nasal polypectomy; presents persistent cough with purulent expectoration, mild dyspnea, sporadic febrile episodes, and constitutional symptoms. On physical examination: regular general condition, tachypnea, tachycardia hypoxemia sat O2 89% baseline, lungs were auscultated crackles at the left base. Tests: hemogram (leukocytosis with neutrophilia), 3 sputum smears (negative), Sudan IV (negative), amylase (negative), chest X-ray showing dextrocardia and bronchiectasis in the lingula high resolution chest tomography, showed bilateral cystic bronchiectasis predominantly in lingual abdominal ultrasound revealed: situs inversus, electrocardiogram: right axis, Conclusion: Kartagener Syndrome is a rare disease that has a clinical presentation similar to other respiratory tract conditions. It is important to know its pathophysiology and clinical characteristics to establish a proper diagnosis and guarantee specialized multidisciplinary treatment. This clinical entity should be considered when finding partial or total situs inversus to specify a diagnosis.