0
Correspondence |

Manifold Embryonic Ciliary Functions in the Genesis of Kartagener Syndrome and HeterotaxySitus Inversus and Heterotaxy FREE TO VIEW

Jerome M. Reich, MD, FCCP
Author and Funding Information

From the Earle A. Chiles Research Institute.

CORRESPONDENCE TO: Jerome M. Reich, MD, FCCP, Earle A. Chiles Research Institute, Portland Providence Medical Center, 7400 SW Barnes Rd, A 622, Portland, OR 97225; e-mail: Reichje@isp.com


Editor’s Note: Authors are invited to respond to Correspondence that cites their previously published work. Those responses appear after the related letter. In cases where there is no response, the author of the original article declined to respond or did not reply to our invitation.

FINANCIAL/NONFINANCIAL DISCLOSURES: The author has reported to CHEST that no potential conflicts of interest exist with any companies/organizations whose products or services may be discussed in this article.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;146(5):e167. doi:10.1378/chest.14-0673
Text Size: A A A
Published online
To the Editor:

Physicians have been acquainted with the seemingly incongruous association of situs inversus with bronchiectasis for a century.1,2 Primary ciliary dyskinesia has been identified as the underlying cause of bronchiectasis and its accompanying maladies: sinusitis, hearing loss, and infertility in men and women (sperm and oviduct immotility) with Kartagener syndrome. Less well known is that a deficit in embryonic ciliary motility may result in situs errors and heterotaxy as quantified by Shapiro et al3 in this issue of CHEST (see page 1176). Cartwright et al4 demonstrated the mechanism whereby cilia determine left-right asymmetry. Slough et al5 delineated their cardiac role in mouse embryos with absent cilia in which endocardial cushions failed to develop, leading to heterotaxy. They suggested that cardiac cilia function as mechanosensors, integrating flow, cardiac function, and morphogenesis. This conceptual framework is both scientifically gratifying and a tribute to the generative power of natural selection, which has led to the varied and invaluable adaptive repurposing of the primitive mechanism of ciliary motility. Do the authors agree with this unifying formulation of the genesis of the multifarious features of Kartagener syndrome?

References

Zivert AK. Über einen fall von bronchiectasie bei einem patienten mit situs inversus viscerum. Berliner Klinische Wochenschrift. 1904;41:139-141.
 
Kartagener M. Zur pathogenese der bronchiektasien: bronchiektasien bei situs viscerum inversus. Beiträge zur Klinik der Tuberkulose. 1933;83(4):489-501. [CrossRef]
 
Shapiro AJ, Davis SD, Ferkol T, et al; on behalf of the Genetic Disorders of Mucociliary Clearance Consortium. Laterality defects other than situs inversus totalis in primary ciliary dyskinesia: insights into situs ambiguus and heterotaxy. Chest. 2014;146(5):1176-1186.
 
Cartwright JH, Piro O, Tuval I. Fluid-dynamical basis of the embryonic development of left-right asymmetry in vertebrates. Proc Natl Acad Sci U S A. 2004;101(19):7234-7239. [CrossRef] [PubMed]
 
Slough J, Cooney L, Brueckner M. Monocilia in the embryonic mouse heart suggest a direct role for cilia in cardiac morphogenesis. Dev Dyn. 2008;237(9):2304-2314. [CrossRef] [PubMed]
 

Figures

Tables

References

Zivert AK. Über einen fall von bronchiectasie bei einem patienten mit situs inversus viscerum. Berliner Klinische Wochenschrift. 1904;41:139-141.
 
Kartagener M. Zur pathogenese der bronchiektasien: bronchiektasien bei situs viscerum inversus. Beiträge zur Klinik der Tuberkulose. 1933;83(4):489-501. [CrossRef]
 
Shapiro AJ, Davis SD, Ferkol T, et al; on behalf of the Genetic Disorders of Mucociliary Clearance Consortium. Laterality defects other than situs inversus totalis in primary ciliary dyskinesia: insights into situs ambiguus and heterotaxy. Chest. 2014;146(5):1176-1186.
 
Cartwright JH, Piro O, Tuval I. Fluid-dynamical basis of the embryonic development of left-right asymmetry in vertebrates. Proc Natl Acad Sci U S A. 2004;101(19):7234-7239. [CrossRef] [PubMed]
 
Slough J, Cooney L, Brueckner M. Monocilia in the embryonic mouse heart suggest a direct role for cilia in cardiac morphogenesis. Dev Dyn. 2008;237(9):2304-2314. [CrossRef] [PubMed]
 
NOTE:
Citing articles are presented as examples only. In non-demo SCM6 implementation, integration with CrossRef’s "Cited By" API will populate this tab (http://www.crossref.org/citedby.html).

Some tools below are only available to our subscribers or users with an online account.

Related Content

Customize your page view by dragging & repositioning the boxes below.

Find Similar Articles
CHEST Journal Articles
PubMed Articles
  • CHEST Journal
    Print ISSN: 0012-3692
    Online ISSN: 1931-3543