UNUSUAL INHERITANCE OF PRIMARY CILIARY DYSKINESIA (KARTAGENERS-SYNDROME)

Primary ciliary dyskinesia syndrome is characterised by chronic sinusi tis, bronchiectasis, and, in 50% of cases, dextrocardia. It is general ly believed to be inherited as an autosomal recessive disorder. In thi s report, we describe a family consisting of a mother and her five mal e children, the offspring of three different fathers, all of whom have this syndrome. This argues for either an X linked or autosomal domina nt pattern of inheritance. Cytogenetic and FISH (fluorescent in situ h ybridisation) analyses were done on the mother and one son and were fo und to be normal.