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Ciliary abnormalities

  • Chapter
Biopsy Pathology of the Bronchi

Part of the book series: Biopsy Pathology Series ((BPS))

Abstract

Cilia extend from the apical surfaces of ciliated cells into the airway. Each cilium is made up of many components and a defect in any of the constituent elements will likely lead to failure of ciliary movement. Ciliary abnormalities are of two main types: acquired lesions, which result from sublethal injury to the ciliated cells, and inborn genetic errors. In most cases it is possible to determine whether or not a patient has an acquired or genetic defect. In this regard the patient history is very helpful. Furthermore, genetic defects affect all of the cilia permanently, whereas acquired defects usually affect a proportion of the cilia during or for some time following the insult. However, discrimination between genetic and acquired defects is complicated by the fact that symptoms induced by the former, such as lack of mucociliary clearance, often lead to chronic inflammation which may readily induce multiple and widespread acquired defects in the cilia (Cornillie et al., 1984). This was likely in the case reported by Howell et al. (1980) when, in addition to a lack of dynein arms (a genetic defect), numerous apparently acquired defects were also seen in the cilia of a 12-year-old boy who had repeated respiratory tract infections. Likewise, genetically abnormal cilia were described in patients who developed bronchiectasis, yet numerous seemingly acquired ciliary lesions were also noted, superimposed upon the genetic defects (Wakefield and Waite, 1980). No specific axonemal defects which could be genetically derived and not simply the result of the insult due to recurrent infections or medications have been found in patients with cystic fibrosis (Kollberg et al., 1978; Katz and Holsclaw, 1980; Robertson, 1982; see also Section 8.2).

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Authors and Affiliations

  1. School of Medicine, University of Maryland, Baltimore, Maryland, USA

    Elizabeth M. Mcdowell B Vet Med, PhD (Cantab) (Professor of Pathology)

  2. Veterans Administration Medical Center, University of Michigan School of Medicine, Ann Arbor, Michigan, USA

    Theodore F. Beals MD (Assistant Professor of Pathology, Director of Clinical Electron Microscopy Program)

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  1. Elizabeth M. Mcdowell B Vet Med, PhD (Cantab)
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  2. Theodore F. Beals MD
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© 1986 E.M. McDowell and T.F. Beals

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Mcdowell, E.M., Beals, T.F. (1986). Ciliary abnormalities. In: Biopsy Pathology of the Bronchi. Biopsy Pathology Series. Springer, Boston, MA. https://doi.org/10.1007/978-1-4899-3398-0_7

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  • DOI: https://doi.org/10.1007/978-1-4899-3398-0_7

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