Reversibility of bronchiectasis: case report of Kartagener’s syndrome

Abstract

Kartagener’s syndrome is an autosomal recessive disorder primarily manifesting as ciliary movement disorder. Kartagener’s syndrome is part of the larger group of disorders referred to as primary ciliary dyskinesias (PCD). Although the condition is usually inherited in an autosomal recessive pattern, and some specific gene defects have been recognized, it is clear that the syndrome shows substantial genetic heterogeneity. The incidence of this genetic disorder is estimated to be between 1 and 2 per 30 000 births. Symptoms result from defective cilia motility in the airways. The recurrent pulmonary infections are caused by the grossly impaired mucociliary transport in the respiratory tract causing stasis of the mucus within the bronchi. Progressive and significant lung damage occurs up to the time of diagnosis. Although the management of patients with Kartagener’s syndrome remains uncertain and evidence is limited, it is important to follow up these patients with an adequate and shared care system. This report presents a clinical case of Kartagener’s syndrome in a 25-year-old woman. Computed tomography showed dextrocardia and bronchiectasis. After 7 years, good treatment results were achieved: radiological findings and lung function were improved. The present clinical case demonstrated reversibility of bronchiectasis even in congenital Kartagener’s syndrome, thus indicating, that bronchiectasis progression is a complex interrelationship among genetic variation and a proper nonspecific management.