Primary ciliary dyskinesia natural history, complications and prognosis
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Hafsa Ghaffar, M.B.B.S[2]
Overview
The long-term effects of primary ciliary dyskinesia (PCD) are dependent on respiratory symptoms. PCD is not considered to be life-threatening, but persistent lung and airway disease can lead to permanent damage. Recurrent ear infections can lead to hearing loss, which is sometimes permanent, prompt diagnosis and early treatment improve long-term outcomes.
Natural History, Complications, and Prognosis
Natural History
Primary ciliary dyskinesia develops earlier in life, prognosis depends on the severity of associated symptoms. It is usually diagnosed after workup of persistent rhinorrhea, sinusitis, recurrent pneumonia, persistent otitis media, or infertility. Age on onset directly correlates with disease severity and prognosis.
Complications
Chronic childhood infections can be very devastating, but the range and severity of clinical symptoms are expansive. Fortunately, by adulthood primary ciliary dyskinesia usually becomes less problematic and many patients may have near-normal lives. However, cross-sectional data imply that spirometry deteriorates in patients over time. Clinical manifestations include chronic upper and lower airway disease resulting from ineffective mucociliary clearance. Males demonstrate infertility secondary to immotile spermatozoa.[1]
Prognosis
- Prognosis is generally based on early diagnosis and prompt treatment. Regular chest physiotherapy with bronchodilators or spirometry could improve lung function.
- Children should have regular doctor checkups.