Kartagener’s Syndrome: A Narrative Review on its Clinical Implications and Management

Abstract

Kartagener’s Syndrome, alternatively known as Primary Ciliary Dyskinesia (PCD) is a rare and intricate inherited disorder that impacts the structure and function of cilia, leading to compromised mucociliary clearance. The defining features of this condition consist of chronic sinusitis, situs inversus totalis, and bronchiectasis, forming a characteristic triad. Kartagener’s Syndrome presents numerous challenges in its diagnosis and management, and its clinical implications have significant ramifications for affected individuals. This review aims to comprehensively review and analyse the clinical impact of this syndrome, focusing on the diagnosis, treatment, and management modalities. The ciliary dysfunction in Kartagener’s Syndrome disrupts the mucus clearance and pathogens from the respiratory tract, resulting in chronic infections, progressive lung damage, and respiratory failure in severe cases. Through a narrative review of literature, reviews, and case studies, the authors have explored clinicians’ diagnostic challenges and the advances in genetic testing methods that aid in early and accurate diagnosis. The authors have discussed the multidisciplinary approach to manage Kartagener’s Syndrome, which involves respiratory therapies, antibiotics, and surgical interventions to improve the patient’s quality of life and prevent complications. The review paper also includes in-depth case studies of individuals with Kartagener’s Syndrome, highlighting the variability in clinical presentations and treatment outcomes. These case studies provide valuable insights into the diverse manifestations of this syndrome, further enhancing the existing understanding of the disease and its management. Ultimately, this review is directed to raise awareness among healthcare professionals about Kartagener’s Syndrome, its clinical implications, and its diagnostic and treatment challenges. By shedding light on this rare and intricate genetic disorder, the authors here promote early recognition and proactive management, leading to improved outcomes and better quality of life for individuals with Kartagener’s Syndrome.

Keywords

• bronchiectasis
• cilia
• primary ciliary dyskinesia
• respiratory tract infections
• situs inversus