Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome.

Rayner, Charlotte F. J.; Rutman, Andrew; Dewar, A.; Greenstone, Michael; Cole, Peter; Wilson, Robert R.

doi:10.1164/ajrccm.153.3.8630555

Cited by 67 publications

(48 citation statements)

References 25 publications

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“…It can be safely omitted only if the CBF is normal with a normal beat pattern. Even under these circumstances, orientation studies should be performed if clinical suspicion is high [30]. The epithelium should be fixed immediately in cacodylate-buffered 2.5% glutaraldehyde and postfixed in osmium tetroxide, then processed for transmission electron microscopy (TEM).…”

Section: Assessment Of Structurementioning

confidence: 99%

“…Disorientation results in a larger SD. Typical normal values are SD 10-15% and for PCD with disorientation, 20-25% [30,37]. However, ciliary disorientation can be transient, secondary to infection, so a second sample is taken after treatment to reduce inflammation and eradicate infection before a diagnosis of PCD secondary to disorientation is made.…”

Section: -Diagnostic Algorithm For Primary Ciliary Dyskinesia (Pcd)mentioning

confidence: 99%

See 1 more Smart Citation

Primary ciliary dyskinesia: diagnosis and standards of care

Bush¹,

Cole²,

Hariri³

et al. 1998

Eur Respir J

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347

285

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Primary ciliary dyskinesia (PCD) is characterized by disease of the upper and lower respiratory tract, in association with visceral mirror image arrangement in 50% of cases, due to abnormal structure and/or function of cilia. The purpose of this paper is to review the clinical features, diagnosis and management of PCD. Presentations include neonatal respiratory distress, recurrent lower respiratory tract infection, chronic rhinosinusitis and male infertility. PCD enters the differential diagnosis of bronchiectasis, atypical asthma, and unusually severe upper airway disease. Diagnosis is by a cascade of investigations, starting with the saccharin test in patients older than 10 yrs; ciliary beat frequency and pattern on light microscopy; and electron microscopy to assess ciliary morphology and orientation. It is important not to confuse primary and secondary ciliary abnormalities. Nasal nitric oxide is low in PCD, and this measurement shows promise as a screening test for PCD. Diagnosis is important, in order to prevent the development of bronchiectasis and to avoid any unnecessary otorhinolaryngological procedures. Regular follow-up is essential, and management should be multidisciplinary, with input from centres with a special interest in PCD, having access to paediatric and adult chest physicians, otolaryngologists and audiological physicians, physiotherapists, counselling services and fertility clinics. The prognosis is good, but morbidity can be considerable if PCD is incorrectly managed.

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Section: Assessment Of Structurementioning

confidence: 99%

Section: -Diagnostic Algorithm For Primary Ciliary Dyskinesia (Pcd)mentioning

confidence: 99%

Primary ciliary dyskinesia: diagnosis and standards of care

Bush¹,

Cole²,

Hariri³

et al. 1998

Eur Respir J

Self Cite

347

285

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show abstract

“…: Randomization of rotational orientation of basal bodies and cilia has been observed in individual instances of primary ciliary dyskinesia (40,41). In one systematic study, ciliary rotational disorientation in PCD patients was always accompanied by immotility or dynein deficiency (42).…”

Section: Flow-induced Rotational Orientationmentioning

confidence: 99%

Cilia orientation and the fluid mechanics of development

Marshall¹,

Kintner

2008

Current Opinion in Cell Biology

119

109

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SummaryMotile cilia produce large-scale fluid flows critical for development and physiology. Defects in ciliary motility cause a range of disease symptoms including bronchiectasis, hydrocephalus, and situs inversus. However, it is not enough for cilia to be motile and generate a flow -the flow must be driven in the proper direction. Generation of properly directed coherent flow requires that the cilia are properly oriented relative to tissue axes. Genetic, molecular, and ultrastructural studies have begun to suggest pathways linking cilia orientation to planar cell polarity and other long-range positional cues, and also suggest that cilia-driven flow can itself play a causal role in orienting the cilia that create it. Errors in cilia orientation have been observed in human ciliary disease patients, suggesting that orientation defects may constitute a novel class of ciliopathies with a distinct etiology at the cell biological level.

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“…Our finding is similar to the result of Rautiainen, in which the disorientation was 23.8+/-6.3° in nasal samples and 29.9+/-7.3° in sphenoid sinuses [7]. The disorientation of PCD patients was 38.7+/-7.8° in a study by De Iongh and Rutland [8] and 23.3+/-1.5° for central pairs and 23.5+/-2.5° for basal feet by Rayner et al [9], all of which were significantly greater than the normal groups. The photoelectric method could simultaneously measure ciliary beat frequency, phase shifts and correlation factors from small active ciliary areas [13].…”

Section: Discussionmentioning

confidence: 96%

“…It has been found that ciliary disorientation may occur secondary to inflammation caused by infection [6] and that the measured orientation of cilia may vary in normal subjects [6]-[8]. Ciliary disorientation alone can lead to the clinical syndrome of primary ciliary dyskinesia (PCD) [9]. The directions based on observations of ciliary cross-sections may differ from the metachronal wave directions of cilia beating in media.…”

Section: Introductionmentioning

confidence: 99%

Direct measurement of directional disorder for ciliary metachronal wave

Park²,

Lee

et al.

2001 Conference Proceedings of the 23rd Annual International Conference of the IEEE Engineering in Medicine and Biology Society

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Abstract-The interrelationship of cilia and the directional disorder of ciliary metachronal wave were analyzed using digital microscopic images. The degree of synchronization between ciliary beats was determined by the correlation factor between two different spots. Principal axes of inertia were applied to find out the uniphase directions of beating cilia. The standard deviation of wave directions in an ROI was defined as a measure of ciliary wave disorder (CWD). The pooled mean of CWD was 22.9+/-8.73° in ROIs of 8 µ m× 8µ m and 25.4+/-6.46° in 32µ m× 24µ m from the sphenoid sinus mucosa of 5 normal subjects. Our result shows that there is a considerable variation in metachronal wave directions of cilia beating on the epithelium.Keywords -Digital microscopic images, correlation map, uniphase directions of cilia, principal axis of inertia, ciliary wave disorder (CWD). I. INTRODUCTIONThe main task of mucociliary systems in the respiratory tract is to eliminate inhaled particles by the propulsion of mucus. The o verall propulsive effect depends upon the arrangement of the cilia, their metachronal relationships, as well as on the ciliary beat frequency (CBF) [1]. Some methods based on image analysis allow the determination of the CBF of a single cell or multiple ciliated cells simultaneously [2]-[4]. Disorientation of cilia leads to an impairment of mucociliary transport. To determine the coordination of ciliary beating, variations in the orientation of cilia have been previously measured by examining crosssections of the epithelium using an electron microscope [5]. It has been found that ciliary disorientation may occur secondary to inflammation caused by infection [6] and that the measured orientation of cilia may vary in normal subjects [6]-[8]. Ciliary disorientation alone can lead to the clinical syndrome of primary ciliary dyskinesia (PCD) [9]. The directions based on observations of ciliary cross-sections may differ from the metachronal wave directions of cilia beating in media. We have applied principal axis of inertia to the 2-dimensional correlation map calculated from sequential ciliary images. This method directly measures the phase relations and wave directions of multiple cilia beating in culture media. II. MATERIALS AND METHODSThe mucosa in the sphenoid sinus were collected from subjects of no respiratory diseases with informed consent. The ciliary movements were recorded using a CCD camera from an inverted microscope. A personal computer equipped with an image-grabber [IC-Comp ; Imaging Technology , Inc.] converted VCR signals into digital images with a depth of 8 bits. Under the magnification used, one pixel size equated to 0.1µm. The digitized images for each processing were acquired at 30Hz for about 4.27 seconds (128 frames). The whole field of 640 by 480 pixels was divided into equal sized blocks (spots) of 2 by 2 pixels. Gray-level values of all pixels in each spot were summed sequentially from the first frame to the last.Movements of cilia are coordinated in time and space by phase differen...

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Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome.

Cited by 67 publications

References 25 publications

Primary ciliary dyskinesia: diagnosis and standards of care

Primary ciliary dyskinesia: diagnosis and standards of care

Cilia orientation and the fluid mechanics of development

Direct measurement of directional disorder for ciliary metachronal wave

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