Chest
ORIGINAL RESEARCHPRIMARY CILIARY DYSKINESIAPulmonary Radioaerosol Mucociliary Clearance in Diagnosis of Primary Ciliary Dyskinesia
Section snippets
Aim
The aim of this three-part study was as follows: (1) to describe PRMC in a preliminary study by testing it on a population of patients with known PCD; (2) following that, to assess the diagnostic validity (ie, sensitivity, specificity, and positive and negative predictive values) in patients referred for a PCD workup in a blinded trial by testing PRMC against nasal ciliary motility studies; and (3) after acceptance of PRMC as a valid method based on the results of substudies 1 and 2, to
Patients
We studied 95 patients, all of whom were referred to the National PCD Centre in Copenhagen for workup due to a clinical suspicion of PCD. Prior to study inclusion, cystic fibrosis was ruled out. Tests were performed at least 4 to 6 weeks after the occurrence of an acute upper or lower respiratory tract infection. In case of inconclusive tests, repeated testing was performed after treatment with antibiotics for 6 weeks. PRMC was allowed to be performed up to three times and a ciliary motility
Results
A total of 95 patients (age range, 5 to 74 years) were included in this three-part study. Among the 95 patients, the 15 patients from substudy 1 were already known to have PCD, in substudy 2 PCD was diagnosed in an additional 36 patients, and in substudy 3 PCD was diagnosed in an additional 6 patients. PCD was ruled out in 26 patients in substudies 2 and 3. Hence, the study population consisted of 57 patients with a confirmed diagnosis of PCD, 26 non-PCD patients, and 12 patients who had been
Discussion
This is the first study of PRMC comprising a larger population of PCD patients and also the first study in which PRMC has been applied in a routine clinical setting for diagnosing PCD. Our study of PRMC studies applied to known PCD patients confirmed PRMC to be a highly valid functional test for detecting impaired tracheobronchial clearance in patients in whom PCD had previously been diagnosed by the presence of abnormal nasal ciliary motility and ultrastructure.
In the prospective blinded trial
Conclusion
In this study, we have evaluated PRMC as a functional test for mucociliary clearance in patients suspected of having PCD. PRMC is at present the only method for testing the mucociliary function in ciliated parts of the lower airways of an individual and is not influenced by local nasal secondary mucociliary defects.
We conclude from this study, that PRMC (1) has a high diagnostic validity and repeatability of interpretation in the diagnosis and exclusion of PCD in a selected group of patients
Appendix
The procedure for the calculation of reference values for PRMC and RSDs based on a multiple regression model5 is as follows:
ACKNOWLEDGMENT
We thank the bioanalytical technologists (especially Ulla Kernchen and Lene Højby) in the Department of Clinical Physiology and Nuclear Medicine for their help in performing the radioaerosol clearance studies; and bioanalytical technologist Marianne Moller Andersen in the Department of Pediatrics for performing the ciliary function analysis.
References (21)
- et al.
Nasal nitric oxide measurements to screen for primary ciliary dyskinesia
Chest
(2004) - et al.
Disorders of ciliary motility
Am J Med Sci
(2001) Primary ciliary dyskinesia
Acta Otorhinolaryngol Belg
(2000)- et al.
Longitudinal study of lung function in a cohort of primary ciliary dyskinesia
Eur Respir J
(1997) Bronchoscintigraphy and pulmonary clearance of 99mTc-albumin colloid in the study of mucociliary clearance
Dan Med Bull
(1998)- et al.
Lung mucociliary clearance
Eur J Nucl Med
(1994) - et al.
Nasal mucociliary transport, number of ciliated cells, and beating pattern in naturally acquired common colds
Eur J Respir Dis Suppl
(1983) - et al.
The effect of bacterial products on ciliary function
Am Rev Respir Dis
(1988) - et al.
Ciliary function analysis for the diagnosis of primary ciliary dyskinesia: advantages of ciliogenesis in culture
Acta Otolaryngol
(2000) - et al.
Secondary ciliary dyskinesia is absent after ciliogenesis in culture
Acta Otorhinolaryngol Belg
(2000)
Cited by (0)
Dr. Marthin was supported during this work by a grant from SanCop Foundation.
The authors have reported to the ACCP that no significant conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.