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Master Thesis

Dyskinésie ciliaire primitive chez l’adulte : étude rétrospective sur 78 patients

Abstract : Introduction : Primary ciliary dyskinesia (PCD) is a genetic disease characterized by anomalies in ciliary structure, responsible for chronic pulmonary and rhinosinus disease. No study involving only adults has been published. Methods : Retrospective study in two French tertiary hospitals, focusing on adults with a diagnosis of PCD based on presence of bronchiectasis with typical ultrastructural defect of cilia and/or situs inversus (SI). Results : 78 patients (18-77 yrs, median follow-up 8.1 yrs) were included. FEV1 was significantly lower in women (median 60% pred vs. 77.5%, p<0.05) and in patients with chronic airway P. aeruginosa (PA, n=21) infection (median 60.5% vs. 75.5%, p<0.05). FEV1 (% pred) correlated with age (p=0.03), chest CT score (p<0.001) but not with age at diagnosis, SI or ultramicroscopy. FEV1 decline was -11mL/year and was greater in women (-28.3mL/ yr vs -3.8mL/yr p=0.01, although age and PA status were similar) but not in patients with chronic PA (-22mL/yr vs -9 mL/yr, p=0.14). Conclusions : PCD in adults is more severe in women and in patients with chronic PA infection.
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Master Thesis
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Submitted on : Friday, September 25, 2015 - 10:36:46 AM
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  • HAL Id : dumas-01205233, version 1

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Justine Frija Masson. Dyskinésie ciliaire primitive chez l’adulte : étude rétrospective sur 78 patients. Médecine humaine et pathologie. 2015. ⟨dumas-01205233⟩

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