Primary ciliary dyskinesia
Conditions
Interventions
Gold Standard:
Index test:
Sponsors
Beijing Children's Hospital
Eligibility
Sex/Gender
All
Age
No minimum to 18 Years
Inclusion criteria
Inclusion criteria: PCD group inclusion criteria:accordance with PCD or Kartagener syndrome diagnostic criteriaThe diagnostic criteria of PCD: (1)at least 5 cilia transverse section and section were prepared under the transmission electron microscope; (2) cross section of each at least 10 non-adjacent ciliated cells (at least 50 ciliated cells); (3) ciliary structure abnormality including absence, shorter or reduced number of dynein arms, absence or shorter of radiation, microtubule translocation, absence of central sheath, ciliary agenesia, basal anomaly; (4) >10% ciliary structure abnormality.The diagnostic criteria of Kartagener syndrome: (1) accordance with the diagnostic criteria of the PCD, at the same time with situs inversus and (or) dextrocardia; (2) with clinical diagnosis of Kartagener syndrome, who has the following triad: ? bronchiectasis; ? sinusitis or nasal polyps; ? transposition of viscera and (or) dextrocardia.suspected PCD group inclusion criteria:Patients with (1) recurrent or persistent cough, expectoration, or wheeze; (2) recurrent or persistent nose running, ear purulence; (3) recurrent or chronic pneumonia, chronic bronchitis; (4) bronchiectasis with unknown causes; (5) with or without dextrocardia, situs inversus, but didn't confirmed with electron microscopy.Acute pneumonia group inclusion criteria:patients with fever, cough, dyspnea, pulmonary rales or other respiratory signs, history within 2 weeks, accompanied by chest imaging unilateral or bilateral abnormalities.
Exclusion criteria
Exclusion criteria: (1)Patients with other systemic diseases or other concurrent diseases, or impossible to complete the follow-up; (2)Patients who does not agree with the ciliary function test.
Design outcomes
Primary
| Measure | Time frame |
|---|---|
| ciliary beat frequency;Ciliary beat for; | — |
Secondary
| Measure | Time frame |
|---|---|
| FeNO;lung fuction;Lung imagin; | — |
Countries
China
Contacts
Public ContactBaoping Xu
Outcome results
None listed