Skip to main content

Main menu

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • COVID-19 submission information
    • Peer reviewer login
  • Alerts
  • Podcasts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • ERJ Early View
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Open access
    • COVID-19 submission information
    • Peer reviewer login
  • Alerts
  • Podcasts
  • Subscriptions

A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia

J. F. Papon, A. Coste, F. Roudot-Thoraval, M. Boucherat, G. Roger, A. Tamalet, A. M. Vojtek, S. Amselem, E. Escudier
European Respiratory Journal 2010 35: 1057-1063; DOI: 10.1183/09031936.00046209
J. F. Papon
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
A. Coste
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
F. Roudot-Thoraval
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
M. Boucherat
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
G. Roger
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
A. Tamalet
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
A. M. Vojtek
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
S. Amselem
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
E. Escudier
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Figures

  • Tables
  • Fig. 1—
    • Download figure
    • Open in new tab
    • Download powerpoint
    Fig. 1—

    Representative images of the ultrastructure of respiratory cilia. a) Cross-section of normal cilia showing the “9+2” microtubule doublet configuration with presence of dynein arms. b) Absence of outer dynein arms. c) Absence of both dynein arms. d) Absence of outer dynein arms and questionable inner dynein arms. e) Absence of inner dynein arms. f) Absence of inner dynein arms and axonemal disorganisation. g) Questionable inner dynein arms. h) Absence of central microtubules. Magnification ×60,000.

Tables

  • Figures
  • Table 1—

    Transmission electron microscopy(TEM) feasibility in 1,450 samples according to the age group, site of biopsy, children’s age range and year of diagnostic test

    Analysis of ciliary ultrastructure by TEM
    FeasibleUnfeasibleTotal
    Age group
     Child samples562 (58.5)403 (41.5)965
     Adult samples424 (87.4)*61 (12.6)485
    Site of biopsy
     Nose458 (72)#178 (28)636
     Bronchus528 (64.8)286 (35.2)814
    Children's age range yrs
     0–4180 (54)153 (46)333
     5–9177 (53)159 (47)336
     10–17205 (69)*91 (31)296
    Year of diagnostic test
     1985–1989230 (72)91 (28)321
     1990–1994172 (63)101 (37)273
     1995–1999294 (69)132 (31)426
     2000–2006290 (67)140 (33)430
    • Data are presented as n (%) or n. #: the significant difference disappeared after adjustment for age of patients. *: p<0.05.

  • Table 2—

    Characteristics of the groups as defined by ciliary ultrastructure in the 820 patients with feasible transmission electron microscopy analysis

    Groupsp-value
    IIIIIIIV
    NormalHeterogeneous defectsMain defectQuestionable ultrastructure
    Patients533 (65)15 (1.8)245 (29)27 (3.3)
    Abnormal cilia %
     Median528.5100#ND<0.0001
     IQR2–1025–3451–100ND
     Range0–2021–4220.5–100ND
    Sex ratio F/M0.680.360.780.8ns
    Children/adults ratio1.090.872.06#1.7<0.001
    Situs inversus28 (5.2)1 (6.7)78 (31.8)#2 (7.4)<0.0001
    Airway infections
     Isolated upper239 (44.8)6 (40)1 (0.4)4 (14.8)
     Isolated lower241 (45.2)7 (46.7)1 (0.4)5 (18.5)
     Sinopulmonary53 (10)2 (13.3)243 (99.2)¶18 (66.7)¶<0.001
    • Data are presented as n (%), unless otherwise indicated. IQR: interquartile range; F/M: female/male; ND: not determined; ns: nonsignificant. p-values are for global comparison between the four groups; if p<0.05, two-by-two comparisons were performed. #: indicates that only this group was significantly different from the other groups; ¶: indicates that the two groups were not different from each other but were significantly different from the other two groups.

  • Table 3—

    Phenotypic features according to the main ciliary defects as defined by transmission electron microscopy(group III, n = 245)

    Main ultrastructural defectp-value
    ODAIDACC
    Patients n1594046
     Associated defects
      IDA
       Normal81
       Defect56
       Questionable22
      Radial spoke
       Normal9
       Defect31
    Abnormal cilia %
     Median100#100#37<0.0001
     IQR78–100100–10026.7–60.2
     Range21–10030–10020.5–85
    Sex ratio F/M0.910.660.65ns
    Children/adults ratio2.7432.83ns
    Situs inversus65 (40.9)¶13 (32.5)0 (0)¶<0.001
    Patients with 100% abnormal cilia110 (69.2)32 (80)0 (0)+<0.001
    • Data are presented as n (%), unless otherwise indicated. ODA: outer dynein arm; IDA: inner dynein arm; CC: central complex; IQR: interquartile range; F/M: female/male; ns: nonsignificant. p-values are for global comparison between the four groups; if p<0.05, two-by-two comparisons were performed. #: indicates that the two groups were not different from each other but were significantly different from the other two groups; ¶: indicates that the two groups were different from each other and were also significantly different from the other two groups; +: indicates that only this group was significantly different from the other groups.

PreviousNext
Back to top
View this article with LENS
Vol 35 Issue 5 Table of Contents
European Respiratory Journal: 35 (5)
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Citation Tools
A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia
J. F. Papon, A. Coste, F. Roudot-Thoraval, M. Boucherat, G. Roger, A. Tamalet, A. M. Vojtek, S. Amselem, E. Escudier
European Respiratory Journal May 2010, 35 (5) 1057-1063; DOI: 10.1183/09031936.00046209

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

Share
A 20-year experience of electron microscopy in the diagnosis of primary ciliary dyskinesia
J. F. Papon, A. Coste, F. Roudot-Thoraval, M. Boucherat, G. Roger, A. Tamalet, A. M. Vojtek, S. Amselem, E. Escudier
European Respiratory Journal May 2010, 35 (5) 1057-1063; DOI: 10.1183/09031936.00046209
del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
Full Text (PDF)

Jump To

  • Article
    • Abstract
    • PATIENTS AND METHODS
    • RESULTS
    • DISCUSSION
    • Support statement
    • Statement of interest
    • Acknowledgments
    • References
  • Figures & Data
  • Info & Metrics
  • PDF
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • Health-related quality of life and unmet needs in patients with primary ciliary dyskinesia
Show more Original Articles: Primary ciliary dyskinesia

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About the ERJ

  • Journal information
  • Editorial board
  • Reviewers
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Instructions for authors
  • Publication ethics and malpractice
  • Submit a manuscript

For readers

  • Alerts
  • Subjects
  • Podcasts
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Print ISSN:  0903-1936
Online ISSN: 1399-3003

Copyright © 2022 by the European Respiratory Society