Ten-year experience using a plastic, disposable curette for the diagnosis of primary ciliary dyskinesia.
BACKGROUND
Primary ciliary dyskinesia (PCD) results in impaired mucociliary clearance. Patients with this disorder develop chronic sinopulmonary disease with recurrent sinusitis, otitis media, nasal polyposis, pneumonia, and, ultimately, bronchiectasis. Other associated findings of dysfunctional ciliary activity include situs inversus, dextrocardia, and infertility.


OBJECTIVE
To describe our 10-year experience using a small, plastic, disposable curette to perform a screening procedure for cilia function and to collect samples for electron microscopy.


METHODS
In the past 10 years, we screened infants and children with severe chronic sinusitis and other chronic recurrent upper respiratory tract problems for PCD by using a plastic, disposable curette to collect tissue samples from the nasal mucosa. Samples were placed in sterile saline and examined under light microscopy for the presence of cilia. Failure to note ciliary movement prompted another examination 1 month later. If no functional cilia were noted at the follow-up examination, a specimen was obtained and sent for electron microscopy.


RESULTS
We identified 7 patients with PCD; 2 had situs inversus totalis. Average age at diagnosis was 3 years. The most common symptom at presentation was frequent upper respiratory tract infections with severe otitis media (7 patients) and sinusitis (5 patients). Recurrent pneumonia was present in 6 patients. Dynein arm deficiency was the most common electron microscopic diagnosis.


CONCLUSIONS
Evaluating children for PCD by using a plastic, disposable curette is a relatively simple procedure that could be used by allergists in practice. Primary ciliary dyskinesia occurs frequently enough that physicians should consider it as part of the differential diagnosis in evaluating children with recurrent, severe sinopulmonary infections.
Section of Pediatric Allergy and Immunology, Department of Pediatrics, West Virginia University, School of Medicine, Morgantown, West Virginia 26506-9214, USA.
Jon E
JE
Welch
Mary Beth
MB
Hogan
Nevin W
NW
Wilson
D002648:Child; D002669:Child Welfare; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004209:Disposable Equipment; D004867:Equipment Design; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D007229:Infant Welfare; D007231:Infant, Newborn; D007619:Kartagener Syndrome / Q000175:diagnosis*; D008297:Male; D008403:Mass Screening / Q000295:instrumentation / Q000379:methods; D008854:Microscopy, Electron; D009297:Nasal Mucosa / Q000002:abnormalities / Q000000981:diagnostic imaging; D011859:Radiography; D012141:Respiratory Tract Infections / Q000175:diagnosis; D012189:Retrospective Studies; D012882:Skin Tests; D014903:West Virginia