Kartagener Syndrome: A First Report of Two Cases from Benin, West Africa.

Abstract

BACKGROUND: Kartagener Syndrome is a rare autosomal recessive genetic disorder, belonging to the larger group of diseases of primary ciliary dyskinesia, including situs inversus. This underdiagnosed disease, especially in developing settings, may be potentially responsible for the impairment of the quality of life of patients and can even be life-threatening. We report on two patients with this condition in Benin, in whom the diagnosis had not previously been made, despite a long history of upper and lower respiratory symptoms. The patients were admitted with complications, a superinfection of bronchiectasis complicated by pneumonia and purulent pleurisy in a 30-year-old male and by a refractory hypoxemia, acute pulmonary heart disease in another 32-year-old male. Treatment outcome was favourable in the first case, but less satisfactory in the second one.