Secondary ciliary dyskinesia is absent after ciliogenesis in culture

Abstract

Ultrastructural secondary ciliary dyskinesia (SCD) was measured using transmission electron microscopy in 301 biopsies and 439 samples after ciliogenesis in the sequential monolayer-suspension culture. Biopsies were taken in the context of exclusion of primary ciliary dyskinesia. SCD was frequently found in the biopsies: only 30% of the samples were normal (SCD < 5%), the mean percentage of SCD abnormalities was 11.9 +/- 12.9%. In 1/8 of the samples severe SCD (> 25%) was present. The most frequently encountered SCD abnormality was the membrane bleb, followed by the various peripheral microtubular abnormalities. With increasing total SCD the absence of the central pair became more important. After ciliogenesis in culture SCD was virtually absent: 1.0 +/- 1.8% for all 439 samples, 96% of the samples were within limits of normality (SCD < 5%). Moderate (15-25%) and severe SCD (> 25%) were never found. In more than 50% of the samples not one abnormality was found. There was no relation between the SCD in the biopsy and that after ciliogenesis. The absence of SCD after ciliogenesis is a major advantage for the diagnosis of PCD, specifically in cases with central pair abnormalities, peripheral microtubular pair abnormalities and those without a primary ultrastructural abnormality.