Matches in SemOpenAlex for { <https://semopenalex.org/work/W2565756654> ?p ?o ?g. }
Showing items 1 to 96 of
96
with 100 items per page.
- W2565756654 abstract "Introduction: Primary ciliary dyskinesia (PCD) is a genetic disease characterized by anomalies in ciliary structure/function, responsible for chronic pulmonary and rhinosinus disease. To date, no study involving only adults has been published Aims and objectives: to assess the clinical characteristics and respiratory function in adults with PCD Methods: Retrospective study in two French tertiary hospitals, focusing on adults (≥18 yrs) with a diagnosis of PCD based on presence of bronchiectasis with typical ultrastructural defect of cilia and/or situs inversus (SI). We assessed clinical symptoms, respiratory function, extent of bronchiectasis on CT scans, sputum microbiology and molecular analysis. Results: 78 patients (18-77 yrs) were included. Median follow up was 8.1 yrs. 94.8% of patients had chronic rhinosinusitis. Median nasal NO was 18.9 nL/min (IQR 84.7) and half of ultrastructural defects were dynein arm defects. Respiratory function was significantly lower in women (median FEV1=60% pred, IQR=26 vs. 77.5%, IQR=33, p P. aeruginosa (PA, n=21) infection (median FEV 1 =60.5% vs. 75.5%, p 1 (% pred) correlated with age (r=-0.24, p=0.03), chest CT score (r=-0.46, p 1 decline was -11mL/year (IQR=62.3) and was greater in women (−28.3mL/ yr, IQR=43.4, vs -3.8mL/yr in men, IQR=85.7, p=0.01) although age and PA status were similar. FEV 1 decline was not different between patients with or without chronic PA (−22 mL/yr vs -9 mL/yr, p=0.14). Moreover 3 patients had severe chronic respiratory failure Conclusions: PCD in adults is more severe in women and in patients with chronic PA infection." @default.
- W2565756654 created "2017-01-06" @default.
- W2565756654 creator A5029437047 @default.
- W2565756654 creator A5035869301 @default.
- W2565756654 creator A5040654388 @default.
- W2565756654 creator A5052183901 @default.
- W2565756654 creator A5054386308 @default.
- W2565756654 creator A5055621366 @default.
- W2565756654 creator A5077230360 @default.
- W2565756654 creator A5086074999 @default.
- W2565756654 creator A5087522944 @default.
- W2565756654 date "2015-09-01" @default.
- W2565756654 modified "2023-10-17" @default.
- W2565756654 title "Clinical characteristics and follow up in adult patients with primary ciliary dyskinesia" @default.
- W2565756654 doi "https://doi.org/10.1183/13993003.congress-2015.oa4494" @default.
- W2565756654 hasPublicationYear "2015" @default.
- W2565756654 type Work @default.
- W2565756654 sameAs 2565756654 @default.
- W2565756654 citedByCount "0" @default.
- W2565756654 crossrefType "proceedings-article" @default.
- W2565756654 hasAuthorship W2565756654A5029437047 @default.
- W2565756654 hasAuthorship W2565756654A5035869301 @default.
- W2565756654 hasAuthorship W2565756654A5040654388 @default.
- W2565756654 hasAuthorship W2565756654A5052183901 @default.
- W2565756654 hasAuthorship W2565756654A5054386308 @default.
- W2565756654 hasAuthorship W2565756654A5055621366 @default.
- W2565756654 hasAuthorship W2565756654A5077230360 @default.
- W2565756654 hasAuthorship W2565756654A5086074999 @default.
- W2565756654 hasAuthorship W2565756654A5087522944 @default.
- W2565756654 hasConcept C119060515 @default.
- W2565756654 hasConcept C126322002 @default.
- W2565756654 hasConcept C142724271 @default.
- W2565756654 hasConcept C2776178081 @default.
- W2565756654 hasConcept C2776301714 @default.
- W2565756654 hasConcept C2776938444 @default.
- W2565756654 hasConcept C2777714996 @default.
- W2565756654 hasConcept C2778206077 @default.
- W2565756654 hasConcept C2779104077 @default.
- W2565756654 hasConcept C2779134260 @default.
- W2565756654 hasConcept C2779379686 @default.
- W2565756654 hasConcept C2779734285 @default.
- W2565756654 hasConcept C2780405171 @default.
- W2565756654 hasConcept C2781069245 @default.
- W2565756654 hasConcept C2908520674 @default.
- W2565756654 hasConcept C3019717275 @default.
- W2565756654 hasConcept C534529494 @default.
- W2565756654 hasConcept C71924100 @default.
- W2565756654 hasConcept C75603125 @default.
- W2565756654 hasConcept C90924648 @default.
- W2565756654 hasConceptScore W2565756654C119060515 @default.
- W2565756654 hasConceptScore W2565756654C126322002 @default.
- W2565756654 hasConceptScore W2565756654C142724271 @default.
- W2565756654 hasConceptScore W2565756654C2776178081 @default.
- W2565756654 hasConceptScore W2565756654C2776301714 @default.
- W2565756654 hasConceptScore W2565756654C2776938444 @default.
- W2565756654 hasConceptScore W2565756654C2777714996 @default.
- W2565756654 hasConceptScore W2565756654C2778206077 @default.
- W2565756654 hasConceptScore W2565756654C2779104077 @default.
- W2565756654 hasConceptScore W2565756654C2779134260 @default.
- W2565756654 hasConceptScore W2565756654C2779379686 @default.
- W2565756654 hasConceptScore W2565756654C2779734285 @default.
- W2565756654 hasConceptScore W2565756654C2780405171 @default.
- W2565756654 hasConceptScore W2565756654C2781069245 @default.
- W2565756654 hasConceptScore W2565756654C2908520674 @default.
- W2565756654 hasConceptScore W2565756654C3019717275 @default.
- W2565756654 hasConceptScore W2565756654C534529494 @default.
- W2565756654 hasConceptScore W2565756654C71924100 @default.
- W2565756654 hasConceptScore W2565756654C75603125 @default.
- W2565756654 hasConceptScore W2565756654C90924648 @default.
- W2565756654 hasLocation W25657566541 @default.
- W2565756654 hasOpenAccess W2565756654 @default.
- W2565756654 hasPrimaryLocation W25657566541 @default.
- W2565756654 hasRelatedWork W1783177394 @default.
- W2565756654 hasRelatedWork W1925552813 @default.
- W2565756654 hasRelatedWork W1928003661 @default.
- W2565756654 hasRelatedWork W2012503365 @default.
- W2565756654 hasRelatedWork W2041452420 @default.
- W2565756654 hasRelatedWork W2409464057 @default.
- W2565756654 hasRelatedWork W2550504563 @default.
- W2565756654 hasRelatedWork W2581076778 @default.
- W2565756654 hasRelatedWork W2908224725 @default.
- W2565756654 hasRelatedWork W2910550624 @default.
- W2565756654 hasRelatedWork W2989310096 @default.
- W2565756654 hasRelatedWork W2994064040 @default.
- W2565756654 hasRelatedWork W3026284903 @default.
- W2565756654 hasRelatedWork W3031846089 @default.
- W2565756654 hasRelatedWork W3032895674 @default.
- W2565756654 hasRelatedWork W3083360368 @default.
- W2565756654 hasRelatedWork W3108837454 @default.
- W2565756654 hasRelatedWork W3181600407 @default.
- W2565756654 hasRelatedWork W3186132399 @default.
- W2565756654 hasRelatedWork W3202145198 @default.
- W2565756654 isParatext "false" @default.
- W2565756654 isRetracted "false" @default.
- W2565756654 magId "2565756654" @default.
- W2565756654 workType "article" @default.