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Ciliary Dyskinesis: The Immotile Cilia Syndrome
Author(s) -
Parker Gregg S.,
Mehlum David L.,
Bacher-Wetmore Bea
Publication year - 1983
Publication title -
the laryngoscope
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.181
H-Index - 148
eISSN - 1531-4995
pISSN - 0023-852X
DOI - 10.1002/lary.1983.93.5.573
Subject(s) - cilium , ultrastructure , microtubule , anatomy , dynein , electron micrographs , medicine , biology , pathology , microbiology and biotechnology , electron microscope , physics , optics
In the past 5 years much has been learned about the syndrome of ciliary dyskinesis, commonly referred to as immotile cilia syndrome. This syndrome appears to be a congenital defect in the ultrastructure of the cilia, which results in one of three basic defects: lack of dynein arms, absence of radial spokes, or transposition of microtubules. Three cases are presented with electron micrographs; they illustrate the diverse clinical presentations of this disease entity as well as some of the structural abnormalities. The normal and abnormal anatomy of the cilia is discussed and some explanation is offered as to why these structural abnormalities present with such a variety of clinical expressions.