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Quoted phrase not found in phrase index: "Primary ciliary dyskinesia 5"
Page 1
Safety and efficacy of the epithelial sodium channel blocker idrevloride in people with primary ciliary dyskinesia (CLEAN-PCD): a multinational, phase 2, randomised, double-blind, placebo-controlled crossover trial.
Ringshausen FC, Shapiro AJ, Nielsen KG, Mazurek H, Pifferi M, Donn KH, van der Eerden MM, Loebinger MR, Zariwala MA, Leigh MW, Knowles MR, Ferkol TW; CLEAN-PCD investigators and study team. Ringshausen FC, et al. Lancet Respir Med. 2024 Jan;12(1):21-33. doi: 10.1016/S2213-2600(23)00226-6. Epub 2023 Aug 31. Lancet Respir Med. 2024. PMID: 37660715 Clinical Trial.
BACKGROUND: Mucociliary clearance is dysfunctional in people with primary ciliary dyskinesia, resulting in the accumulation of dehydrated mucus in the airways that is difficult to clear. ...INTERPRETATION: In this phase 2 crossover study, idrevloride in hyper …
BACKGROUND: Mucociliary clearance is dysfunctional in people with primary ciliary dyskinesia, resulting in the accumula …
Airway Disease in Children with Primary Ciliary Dyskinesia: Impact of Ciliary Ultrastructure Defect and Genotype.
Kinghorn B, Rosenfeld M, Sullivan E, Onchiri F, Ferkol TW, Sagel SD, Dell SD, Milla C, Shapiro AJ, Sullivan KM, Zariwala MA, Pittman JE, Mollica F, Tiddens HAWM, Kemner-van de Corput M, Knowles MR, Davis SD, Leigh MW. Kinghorn B, et al. Ann Am Thorac Soc. 2023 Apr;20(4):539-547. doi: 10.1513/AnnalsATS.202206-524OC. Ann Am Thorac Soc. 2023. PMID: 36442147 Free PMC article.
Rationale: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance, recurrent respiratory infections, progressive airway damage, and obstructive lung disease. ...Objectives: We sought to delineate the association of ciliary …
Rationale: Primary ciliary dyskinesia (PCD) is characterized by impaired mucociliary clearance, recurrent respiratory i …
Genetic factors contributing to human primary ciliary dyskinesia and male infertility.
Ji ZY, Sha YW, Ding L, Li P. Ji ZY, et al. Asian J Androl. 2017 Sep-Oct;19(5):515-520. doi: 10.4103/1008-682X.181227. Asian J Androl. 2017. PMID: 27270341 Free PMC article. Review.
Primary ciliary dyskinesia (PCD) is an autosomal-recessive disorder resulting from the loss of normal ciliary function. ...Further research in this field will impact the diagnostic strategy for male infertility, enabling clinicians to provide patients …
Primary ciliary dyskinesia (PCD) is an autosomal-recessive disorder resulting from the loss of normal ciliary fu …
Airway Inflammation in Children with Primary Ciliary Dyskinesia.
Sagel SD, Kupfer O, Wagner BD, Davis SD, Dell SD, Ferkol TW, Hoppe JE, Rosenfeld M, Sullivan KM, Tiddens HAWM, Knowles MR, Leigh MW. Sagel SD, et al. Ann Am Thorac Soc. 2023 Jan;20(1):67-74. doi: 10.1513/AnnalsATS.202204-314OC. Ann Am Thorac Soc. 2023. PMID: 35984413 Free PMC article.
Rationale: The role of airway inflammation in disease pathogenesis in children with primary ciliary dyskinesia (PCD) is poorly understood. Objectives: We investigated relationships between sputum inflammation measurements, age, lung function, bronchiectasis, …
Rationale: The role of airway inflammation in disease pathogenesis in children with primary ciliary dyskinesia (PCD) is …
Intermittent prophylactic antibiotics for bronchiectasis.
Spencer S, Donovan T, Chalmers JD, Mathioudakis AG, McDonnell MJ, Tsang A, Leadbetter P. Spencer S, et al. Cochrane Database Syst Rev. 2022 Jan 5;1(1):CD013254. doi: 10.1002/14651858.CD013254.pub2. Cochrane Database Syst Rev. 2022. PMID: 34985761 Free PMC article. Review.
We excluded studies where participants received high dose antibiotics immediately prior to enrolment or those with a diagnosis of cystic fibrosis, allergic bronchopulmonary aspergillosis (ABPA), primary ciliary dyskinesia, hypogammaglobulinaemia, sarcoidosis, …
We excluded studies where participants received high dose antibiotics immediately prior to enrolment or those with a diagnosis of cystic fib …
Clinical and Genetic Spectrum of Children with Primary Ciliary Dyskinesia in China.
Guo Z, Chen W, Wang L, Qian L. Guo Z, et al. J Pediatr. 2020 Oct;225:157-165.e5. doi: 10.1016/j.jpeds.2020.05.052. Epub 2020 Jun 2. J Pediatr. 2020. PMID: 32502479
OBJECTIVE: To report detailed knowledge about the clinical manifestations, ciliary phenotypes, genetic spectrum as well as phenotype/genotype correlation in primary ciliary dyskinesia (PCD) in Chinese children. ...CONCLUSIONS: This large PCD cohort in …
OBJECTIVE: To report detailed knowledge about the clinical manifestations, ciliary phenotypes, genetic spectrum as well as phenotype/ …
Risk factors for the deterioration of pulmonary function in primary ciliary dyskinesia.
Fein V, Maier C, Schlegtendal A, Denz R, Koerner-Rettberg C, Brinkmann F. Fein V, et al. Pediatr Pulmonol. 2023 Jul;58(7):1950-1958. doi: 10.1002/ppul.26417. Epub 2023 Apr 25. Pediatr Pulmonol. 2023. PMID: 37096790
The impact of both adherence and colonization was assessed for the long-term course of FEV1, the association of colonization with lung function also quarterly. ...RESULTS: Chronic colonization did not show any impact on the for long-term course of FEV(1) , but adher …
The impact of both adherence and colonization was assessed for the long-term course of FEV1, the association of colonization with lun …
Primary Ciliary Dyskinesia: Longitudinal Study of Lung Disease by Ultrastructure Defect and Genotype.
Davis SD, Rosenfeld M, Lee HS, Ferkol TW, Sagel SD, Dell SD, Milla C, Pittman JE, Shapiro AJ, Sullivan KM, Nykamp KR, Krischer JP, Zariwala MA, Knowles MR, Leigh MW. Davis SD, et al. Am J Respir Crit Care Med. 2019 Jan 15;199(2):190-198. doi: 10.1164/rccm.201803-0548OC. Am J Respir Crit Care Med. 2019. PMID: 30067075 Free PMC article.
RATIONALE: In primary ciliary dyskinesia, factors leading to disease heterogeneity are poorly understood. OBJECTIVES: To describe early lung disease progression in primary ciliary dyskinesia and identify associations between ultrastructur …
RATIONALE: In primary ciliary dyskinesia, factors leading to disease heterogeneity are poorly understood. OBJECTIVES: T …
Rhinosinusitis in Pediatric Primary Ciliary Dyskinesia: Impact of Disease.
Bhatt JM, Muhonen EG, Meier M, Sagel SD, Chan KH. Bhatt JM, et al. Otolaryngol Head Neck Surg. 2019 Nov;161(5):877-880. doi: 10.1177/0194599819874842. Epub 2019 Sep 10. Otolaryngol Head Neck Surg. 2019. PMID: 31500503
OBJECTIVES: Primary ciliary dyskinesia (PCD) is a genetic disorder characterized by abnormal respiratory cilia ultrastructure and/or function causing defective mucociliary clearance. ...Demographic factors, Lund-Mackay score, and PCD genotype were not found t …
OBJECTIVES: Primary ciliary dyskinesia (PCD) is a genetic disorder characterized by abnormal respiratory cilia ultrastr …
Efficacy and safety of azithromycin maintenance therapy in primary ciliary dyskinesia (BESTCILIA): a multicentre, double-blind, randomised, placebo-controlled phase 3 trial.
Kobbernagel HE, Buchvald FF, Haarman EG, Casaulta C, Collins SA, Hogg C, Kuehni CE, Lucas JS, Moser CE, Quittner AL, Raidt J, Rosthøj S, Sørensen AL, Thomsen K, Werner C, Omran H, Nielsen KG. Kobbernagel HE, et al. Lancet Respir Med. 2020 May;8(5):493-505. doi: 10.1016/S2213-2600(20)30058-8. Lancet Respir Med. 2020. PMID: 32380069 Clinical Trial.
BACKGROUND: Use of maintenance antibiotic therapy with the macrolide azithromycin is increasing in a number of chronic respiratory disorders including primary ciliary dyskinesia (PCD). However, evidence for its efficacy in PCD is lacking. ...METHODS: T …
BACKGROUND: Use of maintenance antibiotic therapy with the macrolide azithromycin is increasing in a number of chronic respiratory disord …
108 results