Primary ciliary dyskinesia: a review.
(2005) In Ultrastructural Pathology 29(3). p.217-220- Abstract
- The entity sinusitis, bronchiectasis, and situs inversus is since long named Kartagener syndrome. Nowadays the designation used is primary ciliary dyskinesia (PCD), which implies cilia with decreased or total absence of motility, which may result in sinusitis, chronic bronchitis, bronchiectasis, and male infertility. A large number of deficiencies detectable on the ultrastructural level give rise to PCD. There may also be aberrations not detected up to the present. The normal left-right asymmetry of the body is thought to be due to the beating of the cilia in the embryonic (Hensen's) node. Total immotility of the cilia should therefore result in random asymmetry of the body that is situs inversus in 50% of the cases. It has also been... (More)
- The entity sinusitis, bronchiectasis, and situs inversus is since long named Kartagener syndrome. Nowadays the designation used is primary ciliary dyskinesia (PCD), which implies cilia with decreased or total absence of motility, which may result in sinusitis, chronic bronchitis, bronchiectasis, and male infertility. A large number of deficiencies detectable on the ultrastructural level give rise to PCD. There may also be aberrations not detected up to the present. The normal left-right asymmetry of the body is thought to be due to the beating of the cilia in the embryonic (Hensen's) node. Total immotility of the cilia should therefore result in random asymmetry of the body that is situs inversus in 50% of the cases. It has also been claimed that 50% of cases with PCD have situs inversus. However, several deficiencies apparently do not cause total immotility, and all ultrastructural variants are not associated with situs inversus in 50% of the cases. Several of the deficiencies are difficult to detect. Optimal fixation and handling are therefore obligatory. The genetic changes behind the variants are now being studied in several laboratories. Patients with PCD have very low levels of nasal nitric oxide, which is of increasing diagnostic importance. Other established diagnostic methods are the saccharine test and determination of ciliary beat frequency. (Less)
Please use this url to cite or link to this publication:
https://lup.lub.lu.se/record/141984
- author
- Carlén, Birgitta LU and Stenram, Unne LU
- organization
- publishing date
- 2005
- type
- Contribution to journal
- publication status
- published
- subject
- keywords
- nitric oxide, primary ciliary dyskinesia, left-right asymmetry, cytofila, Kartagener syndrome
- in
- Ultrastructural Pathology
- volume
- 29
- issue
- 3
- pages
- 217 - 220
- publisher
- Taylor & Francis
- external identifiers
-
- wos:000230599600007
- scopus:22244443066
- ISSN
- 1521-0758
- DOI
- 10.1080/01913120590951220
- language
- English
- LU publication?
- yes
- additional info
- The information about affiliations in this record was updated in December 2015. The record was previously connected to the following departments: Pathology, (Lund) (013030000)
- id
- 13303592-ada5-4bee-9c16-90e131e907b5 (old id 141984)
- alternative location
- http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=Retrieve&db=pubmed&dopt=Abstract&list_uids=16036877
- date added to LUP
- 2016-04-01 17:06:50
- date last changed
- 2022-01-29 00:28:22
@article{13303592-ada5-4bee-9c16-90e131e907b5,
abstract = {{The entity sinusitis, bronchiectasis, and situs inversus is since long named Kartagener syndrome. Nowadays the designation used is primary ciliary dyskinesia (PCD), which implies cilia with decreased or total absence of motility, which may result in sinusitis, chronic bronchitis, bronchiectasis, and male infertility. A large number of deficiencies detectable on the ultrastructural level give rise to PCD. There may also be aberrations not detected up to the present. The normal left-right asymmetry of the body is thought to be due to the beating of the cilia in the embryonic (Hensen's) node. Total immotility of the cilia should therefore result in random asymmetry of the body that is situs inversus in 50% of the cases. It has also been claimed that 50% of cases with PCD have situs inversus. However, several deficiencies apparently do not cause total immotility, and all ultrastructural variants are not associated with situs inversus in 50% of the cases. Several of the deficiencies are difficult to detect. Optimal fixation and handling are therefore obligatory. The genetic changes behind the variants are now being studied in several laboratories. Patients with PCD have very low levels of nasal nitric oxide, which is of increasing diagnostic importance. Other established diagnostic methods are the saccharine test and determination of ciliary beat frequency.}},
author = {{Carlén, Birgitta and Stenram, Unne}},
issn = {{1521-0758}},
keywords = {{nitric oxide; primary ciliary dyskinesia; left-right asymmetry; cytofila; Kartagener syndrome}},
language = {{eng}},
number = {{3}},
pages = {{217--220}},
publisher = {{Taylor & Francis}},
series = {{Ultrastructural Pathology}},
title = {{Primary ciliary dyskinesia: a review.}},
url = {{http://dx.doi.org/10.1080/01913120590951220}},
doi = {{10.1080/01913120590951220}},
volume = {{29}},
year = {{2005}},
}