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Unraveling the Complexities of Kartagener's Syndrome: A Case of Bronchiectasis, Isolated Dextrocardia, and Primary Ciliary Dyskinesia in an Adult With Chronic Respiratory Symptoms

Year: 2026

Type: article

Abstract: ABSTRACT Kartagener's Syndrome (KS), a rare autosomal recessive disorder and a subset of Primary Ciliary Dyskinesia (PCD), is characterized by chronic sinusitis, bronchiectasis, and, in approximately 50% of cases, situs inversus. This condition arises from genetic mutations that impair motile cilia function, leading to defective mucociliary clearance, recurrent respiratory infections, and progressive lung damage. While KS is typically diagnosed in childhood, its recognition in adults is often delayed due to symptom overlap with common respiratory conditions such as asthma, chronic obstructive pulmonary disease (COPD), or cystic fibrosis. This case report describes a 40‐year‐old male who presented with a 10–12‐day history of productive cough, intermittent left‐sided chest pain, and a longstanding history of recurrent respiratory infections and chronic sinusitis. Diagnostic imaging, including chest X‐rays and computed tomography (CT) scans, revealed bilateral bronchiectasis, right lung atelectasis, and isolated dextrocardia, with all other organs maintaining normal anatomical positions (situs solitus), an atypical presentation of KS. Laboratory findings indicated mild neutrophilic leukocytosis, and sputum cultures confirmed Pseudomonas aeruginosa infection, a common pathogen in bronchiectasis. The patient was managed with inhaled bronchodilators, corticosteroids, targeted antibiotic therapy, and chest physiotherapy to enhance mucociliary clearance. A multidisciplinary team, including pulmonologists and cardiologists, was engaged to address the complex, multisystem nature of KS and provide long‐term care. This case underscores the diagnostic challenges of identifying KS in adults, particularly with atypical features like isolated dextrocardia, and emphasizes the critical need for comprehensive clinical evaluation, advanced imaging, and consideration of rare genetic disorders in patients with chronic respiratory symptoms.

Source: Clinical Case Reports

DOI: 10.1002/ccr3.72086

Authors: Ibrahim Khalil, Sakib Abrar, Shah Tanvir Ahmed, Md. Imran Hossain

Institutions: Dhaka Medical College and Hospital, University of Dhaka

Language: English

Topic: Cystic Fibrosis Research Advances

Subfield: Pulmonary and Respiratory Medicine

Field: Medicine

Domain: Health Sciences

SDG: Good health and well-being

Open Access status: gold

Estimated APC paid: $1,820