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Original Research: Genetic and Developmental Disorders |

Differences in Disease Expression Between Primary Ciliary Dyskinesia and Cystic Fibrosis With and Without Pancreatic InsufficiencyDifferences in Disease Expression

Malena Cohen-Cymberknoh, MD; Natalia Simanovsky, MD; Nurith Hiller, MD; Alex Gileles Hillel, MD; David Shoseyov, MD; Eitan Kerem, MD
Author and Funding Information

From the Department of Pediatrics (Drs Cohen-Cymberknoh, Gileles Hillel, and Shoseyov and Prof Kerem), the Cystic Fibrosis and Primary Ciliary Dyskinesia Center (Drs Cohen-Cymberknoh, and Shoseyov and Prof Kerem), and the Department of Radiology (Drs Simanovsky and Hiller), Hadassah Hebrew-University Medical Center, Mount Scopus, Jerusalem, Israel.

Correspondence to: Eitan Kerem, MD, Pediatric Division, Hadassah Hebrew-University Medical Center, POB 12000, 91120 Jerusalem, Israel; e-mail: kerem@hadassah.org.il


For editorial comment see page 674

Drs Cohen-Cymberknoh and Simanovsky contributed equally to this manuscript.

Funding/Support: This study was funded by departmental grants from Hadassah Hebrew University Hospital.

Reproduction of this article is prohibited without written permission from the American College of Chest Physicians. See online for more details.


Chest. 2014;145(4):738-744. doi:10.1378/chest.13-1162
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Background:  Impaired mucociliary clearance causes pulmonary disease in primary ciliary dyskinesia (PCD) and contributes to cystic fibrosis (CF) lung disease. Although the sinopulmonary disease is similar, morbidity and mortality are different. Both patients with PCD and patients with CF with pancreatic sufficiency (CF-PS) show no nutrient malabsorption and are diagnosed at a later age compared with patients with CF with pancreatic insufficiency (CF-PI).

Methods:  Clinical status, microbiology, FEV1, and high-resolution CT (HRCT) scans presented as total Brody score (CT-TBS) were compared for patients with PCD, CF-PI, and CF-PS, all treated at the same medical center, by the same team, and by a similar routine follow-up.

Results:  One hundred sixty-four patients, 34 with PCD, 88 with CF-PI, and 42 with CF-PS were enrolled. PCD was diagnosed at a similar age as CF-PS but significantly later than CF-PI. Mean FEV1 % predicted was similar for the three groups. The rate of FEV1 change with age in PCD was similar to CF-PS but significantly lower than in CF-PI. Severity of structural lung disease (CT-TBS) was similar for PCD and CF-PS and significantly higher in CF-PI. No correlation between TBS or Pseudomonas aeruginosa infection and FEV1 in PCD was seen, whereas a negative correlation with FEV1 was observed for both CF groups.

Conclusions:  Although in our study PCD was similar to CF-PS, the lack of correlation between FEV1 and age, CT-TBS, and P aeruginosa infection in PCD suggests that impaired mucociliary clearance is not the only cause for inducing pulmonary damage in these diseases. Furthermore, a comparison of disease characteristics for PCD and CF should distinguish between CF-PI and CF-PS as different entities.

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