Bench To Bedside: New Findings in Primary Ciliary Dyskinesia

By Joseph H. Sisson, MD; and Johnny L. Carson, PhD

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Natural and Treated History of PCD

Like much of our understanding of PCD, the small number of patients followed by any one center means that few data on the natural history of PCD are available. It is clear, however, that the natural history of PCD varies greatly and likely reflects the genetic heterogeneity of the syndrome. The best evidence for this is a study by Tamalet and colleagues,⁶⁷ who categorized 43 children with PCD based on cilia ultrastructural defects determined by electron microscopy. Those PCD individuals with absence of the cilia central complex had the worst outcomes, with higher incidences of respiratory tract infections and extensive bronchiectasis, and they often required surgical management of their bronchiectatic disease. In contrast, individuals with peripheral microtubule or dynein arm defects appeared to have a good prognosis. Certainly, many individuals with PCD live relatively normal lives, and occasionally PCD causes only minor complaints and is not recognized until late adulthood. In contrast, there are a few PCD individuals with advanced bronchiectasis in childhood that progresses to end-stage lung disease rapidly. Lung transplantation has been used successfully in PCD. Because of the infectious nature of advanced bronchiectasis, bilateral transplantation is the preferred approach to avoid contralateral infection of the new lung, similar to the transplantation strategy used for cystic fibrosis patients. Although it is controversial, living related lung donation has been used successfully in an individual with PCD.^68,69

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