VARIANTS OF KARTAGENERS SYNDROME IN THE SAME FAMILY

Abstract

A family is presented which demonstrates variation of Kartagener''s syndrome in that one sibling had situs inversus, sinusitis, under developed paranasal sinuses, chronic bronchitis and left lower lobe fibrosis. Another sibling without situs inversus had sinusitis, agenesis of the frontal sinuses, under development of the remaining sinuses, nasal polyposis and bronchiectasis. Two other siblings had sinusitis only. The familial tendency indicates an antenatal influence. This may be due to genetic factors, though the horizontal distribution suggests an environmental factor operating at the time of early cell division. As a result there probably is a respiratory tissue defect which renders these individuals more susceptible to recurrent infections and secondary chronic nasal and pulmonary disease. The importance of early recognition and proper prophylactic therapy is reemphasized.