Congenital problems of mucociliary clearance: primary ciliary dyskinesia

Rimmer, Joanne

doi:10.4193/rhin12.126

Cited by 5 publications

(3 citation statements)

References 30 publications

(50 reference statements)

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“…Each respiratory cilium is comprised of a ring of nine peripheral microtubular doublets that surround a central longitudinal pair (known as a ‘9 + 2’ configuration) (Fig. ) . Radial spokes connect the peripheral doublets to the centre, while small nexin links connect these outer microtubules to one another.…”

Section: Pathophysiology: Normal Cilia and Abnormal Ciliamentioning

confidence: 99%

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Primary ciliary dyskinesia: Overlooked and undertreated in children

Hosie

Fitzgerald

Jaffé

et al. 2014

J Paediatrics Child Health

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Primary ciliary dyskinesia (PCD) is a multi-organ disorder associated with chronic oto-sino-pulmonary disease, neonatal respiratory distress, situs abnormalities and reduced fertility. Repeated respiratory tract infections leads to the almost universal development of bronchiectasis. These clinical manifestations are a consequence of poorly functioning motile cilia. However, confirming the diagnosis is quite difficult and is often delayed, so the true incidence of PCD may be significantly higher than current estimates. Nasal nitric oxide has been earmarked as a useful screening tool for identifying patients, but its use is limited in pre-school-aged children. Due to the rarity of PCD, the evidence base for management is somewhat limited, and treatment regimens are extrapolated from other suppurative lung disorders, like cystic fibrosis.

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Section: Pathophysiology: Normal Cilia and Abnormal Ciliamentioning

confidence: 99%

“…1). 19,20 Radial spokes connect the peripheral doublets to the centre, while small nexin links connect these outer microtubules to one another. Inner and outer dynein arm complexes project off the peripheral doublets and are responsible for generating mucociliary movement.…”

Section: Pathophysiology: Normal Cilia and Abnormal Ciliamentioning

confidence: 99%

Primary ciliary dyskinesia: Overlooked and undertreated in children

Hosie

Fitzgerald

Jaffé

et al. 2014

J Paediatrics Child Health

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“…In paediatric patients presenting with a long history of uncontrolled CRS with bilateral anterior rhinorrhoea and otitis media with effusion, one should consider evaluating the mucociliary clearance system for exclusion or diagnosis of PCD (370,371) .…”

Section: Rationalementioning

confidence: 99%

European position paper on diagnostic tools in rhinology

Rimmer

Hellings

Lund

et al. 2019

Rhin

Self Cite

114

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is the official Journal of the European and International Rhinologic Societies and appears quarterly in March, June, September and December. Cited in Pubmed, Current Contents, Index Medicus, Exerpta Medica and Embase Founded in 1963 by H.A.E. van Dishoeck, Rhinology is a worldwide non-profit making journal. The journal publishes original papers on basic research as well as clinical studies in the major field of rhinology, including physiology, diagnostics, pathology, immunology, medical therapy and surgery of both the nose and paranasal sinuses. Review articles and short communications are also pulished. All papers are peer-reviewed. Letters-to-the-editor provide a forum for comments on published papers, and are not subject to editorial revision except for correction of English language. In-depth studies that are too long to be included into a regular issue can be published as a supplement. Supple ments are not subject to peer-review.

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How to Assess, Control, and Manage Uncontrolled CRS/Nasal Polyp Patients

Sivasubramaniam

Harvey

2017

Curr Allergy Asthma Rep

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The role of endotyping in CRS has offered a better understanding of the underlying pathophysiology and allows for more targeted treatment. The understanding of systemic disorders and their role in CRS and the importance of topical treatment reaching the sinuses has also allowed for better control of these patients. We have provided some of the commonly identified causes for uncontrolled CRS and a sensible approach to assessing these patients. We have also focused on common areas of pitfalls in the surgery and choice of patients and the role for ongoing systemic treatment. The future of managing this difficult condition includes endotyping using inflammatory markers and individualizing the treatment to the patient by using specific monoclonal antibodies.

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Congenital problems of mucociliary clearance: primary ciliary dyskinesia

Cited by 5 publications

References 30 publications

Primary ciliary dyskinesia: Overlooked and undertreated in children

Primary ciliary dyskinesia: Overlooked and undertreated in children

European position paper on diagnostic tools in rhinology

How to Assess, Control, and Manage Uncontrolled CRS/Nasal Polyp Patients

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