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Calcium stone lithoptysis in primary ciliary dyskinesia
Marcus P Kennedy1, Peadar G Noone, John Carson
1UNC Chapel Hill, Division of Pulmonary and Critical Care Medicine, Chapel Hill, NC 277599-7020, USA. kennedy@med.unc.edu
Respiratory Medicine
|June 8, 2006
Summary
Older adults with primary ciliary dyskinesia (PCD) may experience lithoptysis, a condition involving airway calcification and stone formation. This study highlights a potential association between PCD and lithoptysis in this age group.
Area of Science:
- Pulmonology
- Rare Diseases
- Medical Imaging
Background:
- Primary ciliary dyskinesia (PCD) is a rare genetic disorder affecting cilia function.
- Lithoptysis, the expectoration of calcified material, has not been previously linked to PCD.
- Initial reports of lithoptysis in older patients prompted this investigation.
Purpose of the Study:
- To investigate the association between lithoptysis and primary ciliary dyskinesia (PCD).
- To identify the prevalence of lithoptysis and airway calcification in adult PCD patients.
Main Methods:
- Prospective and retrospective analysis of 142 PCD patients (28 aged 40+).
- Evaluation for lithoptysis and radiographic evidence of calcification.
- Analysis of broncholiths using microscopy and X-ray analysis.
Main Results:
- Lithoptysis was reported in 5 patients (all aged 40+).
- Radiographic evidence of calcification was found in 4/5 patients with lithoptysis.
- Broncholiths were composed of calcite or calcium phosphate; Pseudomonas aeruginosa was common.
Conclusions:
- An association exists between lithoptysis and PCD in patients aged 40 and older.
- Calcite stone formation may be a response to chronic inflammation and infected secretions in PCD.
- Further research is needed to understand this biomineralization process in PCD.