Extract
Despite recent advances in diagnostic methods, diagnosis of primary ciliary dyskinesia (PCD) remains complex. We need a combination of different diagnostic tests, and all have their limitations [1]. In 2009, the first European Respiratory Society (ERS) Task Force on PCD in children published recommendations [2], suggesting that: 1) nasal nitric oxide (nNO) should be measured to screen for PCD in patients aged ≥5 years [3]; and 2) video microscopy (VM) analysis of ciliary beat pattern and frequency [4] plus electron microscopy (EM) [5] should be the key confirmatory diagnostic tests. Genetic testing was not recommended as part of the initial diagnostic testing, but as an additional test for inconclusive cases. The recommended test combination was nNO, VM and EM for patients aged ≥5 years and VM plus EM for younger patients.
Abstract
Adherence to the 2009 ERS task force diagnostic recommendations was low. To further improve PCD diagnosis, we must be more diligent and engaging in implementing the new evidence-based guidelines published in 2017. http://bit.ly/2zvjpBh
Footnotes
Conflict of interest: F.S. Halbeisen has nothing to disclose.
Conflict of interest: A. Shoemark has nothing to disclose.
Conflict of interest: A. Barbato has nothing to disclose.
Conflict of interest: M. Boon reports grants from Horizon 2020, outside the submitted work.
Conflict of interest: S. Carr reports personal fees for advisory board work, acting as registry trial PI, and travel and accommodation expenses for meetings from Vertex Pharmaceuticals, advisory board honoraria from Chiesi Pharmaceuticals, personal fees for lectures from Actavis Pharmaceuticals, and has acted as trial PI for Pharmaixis Pharmaceuticals, outside the submitted work.
Conflict of interest: S. Crowley has nothing to disclose.
Conflict of interest: R. Hirst has nothing to disclose.
Conflict of interest: B. Karadag has nothing to disclose.
Conflict of interest: C. Koerner-Rettberg has nothing to disclose.
Conflict of interest: M.R. Loebinger reports personal fees for advisory board work from Bayer, Polyphor and Raptor, personal fees for advisory board work lectures from Griffols, and is UK chief investigator for Parion, outside the submitted work.
Conflict of interest: J.S. Lucas reports grants, personal fees for advisory board work, non-financial support for meeting attendance and equipment donations from Aerocrine/Circassia, grants and personal fees for advisory board work from Vertex, grants from Parion, outside the submitted work.
Conflict of interest: B. Maitre has nothing to disclose.
Conflict of interest: H. Mazurek has nothing to disclose.
Conflict of interest: U. Ozcelik has nothing to disclose.
Conflict of interest: V. Martinu has nothing to disclose.
Conflict of interest: N. Schwerk has nothing to disclose.
Conflict of interest: G. Thouvenin has nothing to disclose.
Conflict of interest: S.A. Tschanz has nothing to disclose
Conflict of interest: P. Yiallouros has nothing to disclose.
Conflict of interest: M. Goutaki has nothing to disclose.
Conflict of interest: C.E. Kuehni has nothing to disclose.
Support statement: This work was funded by Schweizerischer Nationalfonds zur Förderung der Wissenschaftlichen Forschung (grant 320030_173044). Funding information for this article has been deposited with the Crossref Funder Registry.
- Received March 14, 2019.
- Accepted June 11, 2019.
- Copyright ©ERS 2019
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