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1 Paediatric Respiratory Medicine, Imperial College of Science, Technology and Medicine at National Heart and Lung Institute, Royal Brompton Hospital, and 2 Host Defence Unit, Imperial College of Science, Technology and Medicine at National Heart and Lung Institute, London
CORRESPONDENCE: A. Bush, Dept of Paediatric Respiratory Medicine, Imperial College of Science, Technology and Medicine, National Heart and Lung Institute Thoracic Medicine, Royal Brompton Hospital, Sydney Street, London, UK, SW3 6NP. Fax: 44 207 3518763
Keywords: ciliary orientation, immotile cilia syndrome, Kartagener's syndrome, neonatal pneumonia, primary ciliary dyskinesia, rhinosinusitis
Received: December 30, 1999
Accepted September 15, 2000
Primary ciliary dyskinesia (PCD) syndrome associated with abnormal
ciliary orientation but with normal ciliary ultrastructure has been described
in adults, but there are no normal ranges for orientation in infants, despite
the fact that half of all patients with PCD present in the new-born period.
Nasal brush biopsies were obtained from eight infants (three males),
mean age 13.1 months, range 7–23, in order to determine ciliary orientation.
They had no upper or lower airway disease and normal organ arrangement and
were undergoing general anaesthesia for other reasons. Two infants with typical
PCD syndrome but normal ultrastructure of individual cilia also had orientation
studies.
In the eight normal subjects, a mean of 254 central pairs was examined,
range 82–453. The mean ciliary orientation was 14.9 degrees, range 12.9–17.5.
The two infants with PCD syndrome but normal ultrastructure of individual
cilia had ciliary orientation of (Case 1) 44.5 degrees (range
10.6–64.5) in 218 central pairs; and on a second occasion, 28.9
degrees, (range 9.0–47.5) in 259 central pairs; for Case 2,
24.4 degrees, (range 13.1–38.4) in 196 central pairs.
The normal range for ciliary orientation is similar in infants to that
described in other work in adults. The two cases of phenotypic primary ciliary
dyskinesia in the presence of normal ciliary ultrastructure but abnormal ciliary
orientation in infants supports the contention that measurement of ciliary
orientation should be part of the assessment of ciliary structure and function
in cases of possible primary ciliary dyskinesia, in particular when the ultrastructure
of individual cilia appear to be normal.
This article has been cited by other articles:
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  J. K. Marthin, J. Mortensen, T. Pressler, and K. G. Nielsen Pulmonary Radioaerosol Mucociliary Clearance in Diagnosis of Primary Ciliary Dyskinesia Chest, September 1, 2007; 132(3): 966 - 976. [Abstract] [Full Text] [PDF]
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R. Corbelli, B. Bringolf-Isler, A. Amacher, B. Sasse, M. Spycher, and J. Hammer Nasal Nitric Oxide Measurements To Screen Children for Primary Ciliary Dyskinesia Chest, October 1, 2004; 126(4): 1054 - 1059. [Abstract] [Full Text] [PDF]
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 M A Chilvers, A Rutman, and C O'Callaghan Functional analysis of cilia and ciliated epithelial ultrastructure in healthy children and young adults Thorax, April 1, 2003; 58(4): 333 - 338. [Abstract] [Full Text] [PDF]
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 A Bush, C O'Callaghan, and A Boon Primary ciliary dyskinesia Arch. Dis. Child., November 1, 2002; 87(5): 363 - 365. [Full Text] [PDF]
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