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 |