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Table 1 A comparison between European Respiratory Society (ERS) and North American primary ciliary dyskinesia (PCD) Foundation guidelines

From: Why, when and how to investigate primary ciliary dyskinesia in adult patients with bronchiectasis

  ERS 2017 [36] North American PCD Foundation 2016 [17]
Structure Evidence-based guidelines Consensus statement
Patients included Infants and adults Infants and adults
Diagnostic criteria distinguished by age Not done Newborns (0–1 month)
Children (1 month – 5 years)
Children 5–18 years and adults
Diagnostic criteria A diagnostic algorithm is proposed Two major clinical criteria PLUS at least one diagnostic test
Diagnostic outcome PCD positive, PCD highly likely and PCD extremely unlikely PCD positive and PCD negative
nNO No consensus on appropriate thresholds < 77 nL/min on 2 occasions, > 2 months apart, with CF excluded
HSVA Several European centres employ HSVA due to high expertise No American centres can reliably perform HSVA due to lack of expertise
Cell culture Recommended to improve accuracy of HSVA and TEM to rule out a false positive diagnosis or support a highly likely diagnosis Not mentioned
IF Not included in the diagnostic algorithm due to lack of studies at time of guideline Not included in the diagnostic criteria due to lack of studies at time of guideline
TEM Can be used to confirm a diagnosis but advise against TEM in isolation to exclude PCD because some patients with PCD have apparently normal ultrastructure Can be used to confirm a diagnosis but advise against TEM in isolation to exclude PCD because some patients with PCD have apparently normal ultrastructure
Genotyping Can be used to confirm a diagnosis but cannot be used to exclude a diagnosis because evidence on sensitivity and specificity lacks Can be used to confirm a diagnosis but cannot be used to exclude a diagnosis because evidence on sensitivity and specificity lacks
  1. PCD Primary ciliary dyskinesia, nNO Nasal nitric oxide, CF Cystic fibrosis, HSVA High-speed video analysis, IF Immunofluorescence, TEM Transmission electron microscopy