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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