Case report of a middle-aged man with bronchiectasis, situs inversus and cirrhosis
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Abstract
Ciliopathies are anatomical and functional disorders of the cilia in various organ systems such as the urinary and respiratory tracts. Ciliopathies involving the respiratory system include primary ciliary dyskinesia and situs inversus totalis. Patients present with recurrent infections of the middle ear/sinuses, and of the upper and lower respiratory tracts. Chronic infections result in atelectasis and bronchiectasis. When bronchiectasis occurs with impaired fertility and sinusitis, Young’s syndrome, Kartagener’s syndrome and cystic fibrosis may be considered. When bronchiectasis coexists with cirrhosis, alpha1antitrypsin deficiency is generally thought of. Our patient, a middle-aged teetotaller, presented with abdominal distension and pedal edema. His past history was significant for recurrent upper and lower respiratory tract infections and treatment with antituberculous therapy. Investigations suggested Kartagener’s syndrome. Cirrhosis due to hepatitis B contributed to his ascites. Bronchiectasis related corpulmonale and amyloidosis were ruled out. Chronic illnesses with new symptoms may unexpectedly yield other coexisting diseases.
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References
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