title: Calcium stone lithoptysis in primary ciliary dyskinesia.
abstract: BACKGROUND
  An association between lithoptysis and primary ciliary dyskinesia (PCD) has not been previously reported. However, reports of lithoptysis from 2 older patients (>60 yr) prompted a study of this association.
  
  
  METHODS
  We performed a prospective study of all PCD patients presenting to our institution between August 2003 and March 2006, seeking the symptom of lithoptysis or calcium deposition on radiology. A retrospective analysis of all PCD patients presenting prior to August 2003 was also performed. Patients age > or = 40 previously reviewed were recontacted. If a history of lithoptysis or calcium deposition was present, we further reviewed radiographic, microbiologic, and biochemical data, including serum calcium and phosphate. Broncholiths were analyzed by light and electron microscopy- and electron-dispersive X-ray analysis.
  
  
  RESULTS
  In total, 142 patients (n=28 age > or = 40) were included, 41 in the prospective and 91 in the retrospective study. Lithoptysis was reported in 5 patients (all age > or = 40). Chest CT scans identified calcification (4/5), involving bronchiectatic airways in 3 patients and focal nodular calcification in 1 patient. Two other patients (age 46, 59) were identified with airway calcification without lithoptysis. Available broncholiths from 2 of these patients were composed of calcite, whereas a broncholith from 1 patient with focal nodular calcification contained calcium phosphate. Sputum was positive for Pseudomonas aeruginosa in all 7 patients, but negative for mycobacterial and fungal cultures.
  
  
  CONCLUSIONS
  There is an association between lithoptysis and PCD in patients age > or = 40. We hypothesize that calcite stone formation is a biomineralization response to chronic airway inflammation and retention of infected airway secretions in PCD in a subset of PCD patients.
authors.affiliation: UNC Chapel Hill, Division of Pulmonary and Critical Care Medicine, Chapel Hill, NC 277599-7020, USA. kennedy@med.unc.edu
authors.forename: Marcus P
authors.initials: MP
authors.lastname: Kennedy
authors.forename: Peadar G
authors.initials: PG
authors.lastname: Noone
authors.forename: John
authors.initials: J
authors.lastname: Carson
authors.forename: Paul L
authors.initials: PL
authors.lastname: Molina
authors.forename: Andrew
authors.initials: A
authors.lastname: Ghio
authors.forename: Maimoona A
authors.initials: MA
authors.lastname: Zariwala
authors.forename: Susan L
authors.initials: SL
authors.lastname: Minnix
authors.forename: Michael R
authors.initials: MR
authors.lastname: Knowles
mesh_terms: D000328:Adult; D001987:Bronchiectasis / Q000150:complications / Q000000981:diagnostic imaging / Q000382:microbiology; D002118:Calcium / Q000032:analysis; D002137:Calculi / Q000150:complications* / Q000382:microbiology / Q000473:pathology; D005260:Female; D006801:Humans; D007619:Kartagener Syndrome / Q000150:complications* / Q000382:microbiology / Q000473:pathology; D008171:Lung Diseases / Q000150:complications* / Q000382:microbiology / Q000473:pathology; D008297:Male; D008875:Middle Aged; D011446:Prospective Studies; D011552:Pseudomonas Infections / Q000150:complications / Q000000981:diagnostic imaging / Q000473:pathology; D011859:Radiography; D012189:Retrospective Studies; D013183:Sputum / Q000737:chemistry
chemical_list: D002118:Calcium