Primary ciliary dyskinesia and humoral immunodeficiency--is there a missing link?
BACKGROUND
Primary ciliary dyskinesia (PCD) and humoral mmunodeficiency (HID) are both rare disorders which cause recurrent upper and lower respiratory tract infections.


OBJECTIVE
To examine the concurrence of PCD and HID in a patient cohort with known PCD.


METHODS
Retrospective review of the patient files.


RESULTS
We describe 11 patients of a cohort of 168 patients with PCD (6.5%) with a combination of PCD and some form of HID. The patients all presented with typical clinical symptoms for PCD, however the role of the concomitant immunological abnormalities is not clear.


CONCLUSIONS
PCD and HID coincided in 6.5% of the patients. We suggest that a common pathophysiological pathway results in both disorders.
Department of Pediatrics, Pediatric Pulmonology, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium. Electronic address: mieke.boon@uzleuven.be.
Mieke
M
Boon
Department of Pediatrics, Pediatric Pulmonology, University Hospital Gasthuisberg, Herestraat 49, 3000 Leuven, Belgium.
Kris
K
De Boeck
Department of Otorhinolaryngology, Head and Neck Surgery, University Hospital Gasthuisberg Leuven, Herestraat 49, 3000 Leuven, Belgium.
Mark
M
Jorissen
Department of Pediatrics, Pediatric Immunology, University Hospital Gasthuisberg, Herestraat 49, Leuven, Belgium.
Isabelle
I
Meyts
D000293:Adolescent; D016022:Case-Control Studies; D002648:Child; D005541:Forced Expiratory Volume / Q000502:physiology; D006801:Humans; D056724:Immunity, Humoral / Q000502:physiology*; D007153:Immunologic Deficiency Syndromes / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D007619:Kartagener Syndrome / Q000150:complications* / Q000276:immunology / Q000503:physiopathology; D008875:Middle Aged; D012189:Retrospective Studies
Antibody; Cilia; Humoral immunodeficiency; Primary ciliary dyskinesia