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Year : 2021  |  Volume : 28  |  Issue : 1  |  Page : 27-30

Kartagener's syndrome in a young female: A rare diagnosis in a resource-limited facility

1 Department of Radiology, Faculty of Clinical Sciences, Bayero University, Kano, Nigeria
2 Department of Radiology, University of Abuja, University of Abuja Teaching Hospital, Gwagwalada, Abuja, Nigeria

Correspondence Address:
Dr. Yusuf Lawal
Department of Radiology, Faculty of Clinical Sciences, Bayero University, Kano
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DOI: 10.4103/wajr.wajr_24_20

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Kartagener's syndrome (KS) is a subset of a larger group of ciliary motility disorders called primary ciliary dyskinesia. It is a genetic disease with an autosomal recessive inheritance characterized by inefficient or absent mucociliary clearance. It is a very rare congenital malformation comprising a classical triad of situs inversus, bronchiectasis, and sinusitis. A 22-year-old single female Nigerian came to our health facility with complaints of recurrent productive, non-foul-smelling cough, nasal discharge, and occasional shortness of breath since early childhood. She had a positive history of recurrent hospital visitations and chronic use of antibiotics but with few hospitalizations for recurrent chest infection. Chest examination revealed a maximally audible apex beat on the right side of her chest. Chest radiograph showed dextrocardia, while a chest computer tomography scan revealed cystic and varicose bronchiectatic changes with peribronchial thickening and multiple tiny interstitial nodules, mainly in the bilateral middle and lower lung fields. The patient had a fair response on inhaled steroids, nasal steroid spray, antibiotics, mucolytics, and bronchodilators. She is on follow-up clinic visits and close monitoring for potential complications. Patients with KS exist in resource-poor settings like northern Nigeria, largely being managed as cases of chronic sinusitis, pneumonia, or asthma. Although there is no rapid, reliable, non-invasive diagnostic test for KS, accurate diagnosis is crucial if the risks of complications from advanced disease and reduced quality of life are to be averted.

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