Abstract

Four girls born to second cousin parents developed chronic chest infection and bronchiectasis in infancy. Three were studied in detail: they all had the same HLA haplotype, all showed random orientation of cilia or compound cilia in the respiratory tract, and all had low levels of the C1 and C2 components of the complement system. Although the cause of the respiratory disease in this family remains unclear, it is suggested that the low C1 levels may have contributed to the disease in two of the children while the low C2 levels were artefacts and the ciliary abnormalities were secondary to chronic chest infection.