Ivan H Huang, Kenneth N Dang, Saarang Kashyap, Alexander J Sweidan
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Management of Primary Ciliary Dyskinesia in a Kartagener's Syndrome Patient With Total Situs Inversus.
Kartagener's syndrome is a rare, autosomal recessive disorder characterised by primary ciliary dyskinesia (PCD) resulting in impaired mucociliary clearance and situs inversus. Common symptoms are chronic recurrent rhinosinusitis, bronchiectasis, otitis media and pneumonia. We report the case of a 62-year-old female patient with complete situs inversus of the chest and abdominal organs. Bronchoscopic washout of the lungs was performed for therapeutic benefit and culturing for targeted antibacterial therapy. Patients affected by Kartagener's syndrome are vulnerable to repeated respiratory infections and lower BMI due to impaired mucociliary clearance from PCD. Multimodal airway clearance through pulmonary hygiene is a research-backed strategy in treating this condition. This case highlights the importance of a comprehensive and effective management approach for patients with symptomatic Kartagener's syndrome and other forms of primary ciliary dyskinesia (PCD).
期刊介绍:
Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.