Primary ciliary dyskinesia pathophysiology: Difference between revisions
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Revision as of 18:48, 18 September 2017
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Differentiating Primary ciliary dyskinesia from other Diseases |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
Genetics
This disease in genetically inherited. Both inner and/or outer dynein arms are dysfunction and thus the axoneme structure lacks the ability to move. Axonemes are the elongated structures that make up cilia and flagella. The dysfunction of the cilia begins during the embryologic phase of development. Since the cilia aid in the movement of growth factors resulting in the normal rotation of the internal organs during early embryological development, 50% of these individuals will develop situs inversus, as the laterality of the internal organs is determined by chance.