Document Detail

Cilia motility and structure in primary and secondary ciliary dyskinesia.
MedLine Citation:
PMID:  20537282     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Primary ciliary dyskinesia (PCD) is a clinically uniform entity, although cilia motility and structure can vary among patients, making diagnosis difficult. Chronic sinusitis, bronchiectasis, sinus hypoplasia, secretory otitis media, and low fertility are common in PCD patients. The aim of this work was to correlate nasal ciliary activity with clinical and structural abnormalities in PCD. A secondary aim was to evaluate the usefulness of high-resolution digital high-speed video (DHSV) in the diagnosis of PCD.
METHODS: We analyzed nasal mucociliary transport and cilia ultrastructure by electron microscopy and studied nasal ciliary beat frequency (CBF) and beat pattern using high-resolution DHSV imaging in 34 healthy volunteers, 25 individuals with PCD (including 11 with Kartagener's syndrome [KS]with situs inversus), and 27 with secondary ciliary dyskinesia (SCD).
RESULTS: Nasal mucociliary transport was defective in the PCD and SCD patients. Ciliary immotility was observed in only six KS patients and was correlated with the absence of dynein. We observed a correlation between partial dynein deficiency and an uncoordinated, stiffly vibrating ciliary beat. Cilia activity and structure were normal in the SCD patients.
CONCLUSION: Nasal mucociliary transport showed a sensitivity of 100% for the diagnosis of PCD but has low specificity. High-resolution DHSV imaging has high sensitivity and specificity for the diagnosis of PCD. Video analysis is probably more useful than the study of mucociliary transport and cilia ultrastructure in screening for PCD. The absence of dynein was correlated with ciliary immotility and was more common in KS patients.
Miguel Armengot; Javier Milara; Manuel Mata; Carmen Carda; Julio Cortijo
Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  American journal of rhinology & allergy     Volume:  24     ISSN:  1945-8932     ISO Abbreviation:  Am J Rhinol Allergy     Publication Date:    2010 May-Jun
Date Detail:
Created Date:  2010-06-11     Completed Date:  2010-11-15     Revised Date:  2011-11-10    
Medline Journal Info:
Nlm Unique ID:  101490775     Medline TA:  Am J Rhinol Allergy     Country:  United States    
Other Details:
Languages:  eng     Pagination:  175-80     Citation Subset:  IM    
Rhinology Unit, General and University Hospital, Medical School, Valencia University, Valencia, Spain.
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MeSH Terms
Cell Movement
Cells, Cultured
Child, Preschool
Cilia / metabolism,  ultrastructure*
Diagnosis, Differential
Dyneins / deficiency,  metabolism
Kartagener Syndrome / diagnosis*,  genetics,  pathology*,  physiopathology
Microscopy, Electron
Microscopy, Video*
Middle Aged
Mucociliary Clearance / genetics
Nasal Mucosa / pathology*
Reg. No./Substance:

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