Primary Ciliary Dyskinesia and the HeartMarcus P Kennedy MD, FCCP aBarry J Plant MD a
Cilia are evolutionarily conserved organelles and motile respiratory cilia have a complex (9 + 2) axonemal structure to generate functional ciliary motility.16, 17 Motile cilia have microtubules composed of alpha and beta monomers of tubulin (Fig. 1).17 Outer dynein arms (ODAs) and inner dynein...
Primary ciliary dyskinesia (PCD) is a genetic disease that causes abnormalities in ciliary structure and/or function. Ciliated cells line the upper and lower respiratory tracts and the Eustachian tube. Impairment of mucus clearance at these sites leads to sinusitis, repeated pulmonary infections, ...
Secondary ciliary dyskinesia may be seen in diseases associated with acute and chronic airway inflammation and infection. This review will focus primarily on PCD, the genetically transmitted form of the disease, with a brief review of the structure and function of normal and dysfunctional cilia, ...
The following signs and symptoms may be present in patients with primary ciliary dyskinesia: Nasal mucosal congestion Mucopurulent nasal discharge Nasal obstruction Mouth breathing and halitosis Nasal polyps Inflammation of tympanic membranes Perforation with purulent discharge Hearing loss Respirato...
Primary Ciliary DyskinesiaJason Lobo, MD [Assistant Professor of Medicine], Maimoona A Zariwala, PhD [Associate Professor of Medicine and Laboratory Medicine], and Peadar G Noone, MD, FCCP, FRCPI [Professor of Medicine]The Division of Pulmonary Diseases, University of North Carolina Chapel Hill,...
For more information visit www.intechopen.com 1 Primary Ciliary Dyskinesia/Kartagener Syndrome - Clinical and Genetic Aspects Masumi Akita Division of Morphological Science, Biomedical Research Center, Saitama Medical University, Iruma-gun, Saitama Japan 1. Introduction The epithelium of the respiratory ...
Congenital heart disease and other heterotaxic defects in a large cohort of patients with primary ciliary dyskinesia. Circulation 2007;115:2814–21. 28. Nakhleh N, Francis R, Giese RA, et al. High prevalence of respiratory ciliary dysfunction in congenital heart disease patients with heterotaxy....
ARTICLE Next generation massively parallel sequencing of targeted exomes to identify genetic mutations in primary ciliary dyskinesia: Implications for application to clinical testing Jonathan S. Berg, MD, PhD1,2, James P. Evans, MD, PhD1,2, Margaret W. Leigh, MD3, Heymut Omran, MD4, Chris ...
H.Brost, H.Ott, C.Pfannenstiel, T. G.Wenzl, G.Heimann. (2003) Aspirin intolerance in boy with primary ciliary dyskinesia. Allergy 58 , 160-160 /Brost H., Ott H., Pfannenstiel T. i wsp. Aspirin intolerance in boy with primary ciliary dyskinesia. Allergy 2003; 58: 160....