Axonemal utrastructural abnormalities: primary [genetic] versus secondary [acquired] ciliary dyskinesia coiled by control

ABSTRACT

Primary ciliary dyskinesia [PCD] is an autosomal disorder that includes Kartagner's syndrome, immotile cilia syndrome, ciliary dysmotility and primary orientation defects. It is characterized clinically by sinusitis, otitis media, bronchiectasis, male infertility and often dextrocardia. The present work represents a .comparative quantitative study of the respiratory ciliary mucosa in PCD [6 cases], secondary ciliary dyskinesia [SCD] [6 cases] and 12 cases of normal non-inflamed mucosa as a control group. The light microscopy, the ultrastructure constituents and the deviation angle of the cilia were studied. There was no difference by light microscopy while the differences on the ciliary constituents were statistically insignificant [p>0.005]. It was found that the mean ciliary deviation angle in both the control group and the SCD were less than 30 while in PCD it was 43. The pattern of the histogram can aid in the diagnosis. The interpretation of the different ultrastructural findings should be associated with the deviation angle, along with the history of sinusitis, otitis media and respiratory tract infection since birth