Chest
Original Research: Genetic and Developmental DisordersDifferences in Disease Expression Between Primary Ciliary Dyskinesia and Cystic Fibrosis With and Without Pancreatic Insufficiency
Section snippets
Materials and Methods
Patients with confirmed diagnosis of PCD or CF, who were treated at the Hadassah Medical Center from 2007 to 2011 and for whom spirometry, HRCT scan, sputum cultures, and pancreatic sufficiency test data were available, were included in the study. The study was approved by the Hadassah Medical Center Institutional Review Board, and written informed consent was waived (Committee on Research Involving Human Subjects of the Hebrew University—Hadassah Medical School, 0324-08-HMO).
The CF and PCD
Results
A total of 164 patients, 34 with PCD and 130 with CF (88 with CF-PI [67.7% of patients with CF, 53.7% of the study population] and 42 with CF-PS [32.3% of CF and 25.6% of all patients]), were enrolled. Table 2 summarizes the clinical presentation of all patients. Patients with PCD and CF-PI were similar in age and younger than the CF-PS group (P = .074 and 0.014, respectively). Additionally, PCD was diagnosed at a significantly later age compared with the total CF group (P = .002) but at a
Discussion
To our knowledge, this is the first study comparing disease expression between PCD and CF, analyzing separately CF-PI and CF-PS. Patients with PCD, like those with CF-PS, do not suffer from pancreatic insufficiency and are generally diagnosed at a later age compared with patients with CF-PI. In PCD, in contrast to results seen for both CF groups, no correlation between FEV1 and TBS or between FEV1 and age was found. For PCD, these data provide further support that FEV1 is not a strong predictor
Acknowledgments
Author contributions: Prof Kerem is guarantor of the manuscript, taking responsibility for the integrity of the data and accuracy of the data analysis.
Dr Cohen-Cymberknoh: contributed to conception and design of the study, acquisition of data, analysis of the results, and revision of the manuscript and provided final approval of the version to be published.
Dr Simanovsky: contributed to performing the CT scans, analysis, calculations of TBS, and revision of the manuscript and provided final
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Drs Cohen-Cymberknoh and Simanovsky contributed equally to this manuscript.
Funding/Support: This study was funded by departmental grants from Hadassah Hebrew University Hospital.
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