Literature DB >> 11082770

Secondary ciliary dyskinesia is absent after ciliogenesis in culture.

M Jorissen1, T Willems, B Van der Schueren, E Verbeken.   

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.

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Mesh:

Year:  2000        PMID: 11082770

Source DB:  PubMed          Journal:  Acta Otorhinolaryngol Belg        ISSN: 0001-6497


  6 in total

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Authors:  M A Chilvers; A Rutman; C O'Callaghan
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2.  Ciliary ultrastructure in two sisters with Kartagener's syndrome.

Authors:  Kayoko Tanaka; Akihisa Sutani; Yuka Uchida; Yoshihiko Shimizu; Michio Shimizu; Masumi Akita
Journal:  Med Mol Morphol       Date:  2007-03-29       Impact factor: 2.309

Review 3.  Value of transmission electron microscopy for primary ciliary dyskinesia diagnosis in the era of molecular medicine: Genetic defects with normal and non-diagnostic ciliary ultrastructure.

Authors:  Adam J Shapiro; Margaret W Leigh
Journal:  Ultrastruct Pathol       Date:  2017-09-15       Impact factor: 1.094

4.  Mucociliary transport using 99mTc-albumin colloid: a reliable screening test for primary ciliary dyskinesia.

Authors:  K De Boeck; M Proesmans; L Mortelmans; B Van Billoen; T Willems; M Jorissen
Journal:  Thorax       Date:  2005-05       Impact factor: 9.139

5.  Diagnosis and management of primary ciliary dyskinesia.

Authors:  Claudius Werner; Jörg Große Onnebrink; Heymut Omran
Journal:  Cilia       Date:  2015-01-22

6.  Primary ciliary dyskinesia: critical evaluation of clinical symptoms and diagnosis in patients with normal and abnormal ultrastructure.

Authors:  Mieke Boon; Anne Smits; Harry Cuppens; Martine Jaspers; Marijke Proesmans; Lieven J Dupont; Francois L Vermeulen; Sabine Van Daele; Anne Malfroot; Veronique Godding; Mark Jorissen; Kris De Boeck
Journal:  Orphanet J Rare Dis       Date:  2014-01-22       Impact factor: 4.123

  6 in total

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