ATS GUIDELINES Bundle

Primary Ciliary Dyskinesia

American Thoracic Society Quick-Reference GUIDELINES Apps

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➤ In patients presenting with a strong clinical phenotype for PCD, the ATS suggests using an extended genetic panel (>12 genes) as a diagnostic test over TEM ciliary testing and/or standard (≤12 genes) genetic panel testing (conditional recommendation, moderate certainty of evidence in test accuracy but very low certainty in the overall evidence). ➤ In cooperative patients 5 years of age or older with a clinical phenotype consistent with PCD and with CF excluded, the ATS suggests using nNO testing with chemiluminescense technology and standardized protocol for the diagnosis of PCD over TEM and/or genetic testing (conditional recommendation, moderate certainty in test accuracy but very low certainty in the overall evidence). ➤ The ATS suggests NOT using ciliary beat pattern analysis by high-speed video microscopy (HSVM) as a replacement diagnostic test in patients with a high probability of having PCD (conditional recommendation, low certainty in the diagnostic accuracy of the test but very low certainty in the overall evidence). ➤ The ATS suggests NOT using ciliary beat frequency (CBF) measurement as a diagnostic test in patients with a high probability of having PCD (conditional/weak recommendation, low certainty in the diagnostic accuracy of the test but very low certainty in the overall evidence). Diagnosis

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