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Quoted phrase not found in phrase index: "Ciliary dyskinesia, primary, 38"
Page 1
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. ...However, variable phenotypes were also …
OBJECTIVE: To report detailed knowledge about the clinical manifestations, ciliary phenotypes, genetic spectrum as well as phenotype/ …
Etiology and clinical characteristics of a non-cystic fibrosis bronchiectasis cohort in a middle eastern population.
Shafiq I, Wahla AS, Uzbeck MH, Zoumot Z, Abuzakouk M, Elkhalifa S, Bodi G, Almazrouei KM, Bodi K, Isse S. Shafiq I, et al. BMC Pulm Med. 2023 Jul 10;23(1):250. doi: 10.1186/s12890-023-02543-z. BMC Pulm Med. 2023. PMID: 37430275 Free PMC article.
Forty-eight (18.5%) patients were labelled idiopathic, while Primary Ciliary Dyskinesia (PCD) accounted for 23 (8.8%) cases. Pseudomonas aeruginosa was the most common colonizing organism (32.7%), followed by Haemophilus influenzae (9.2%) and Methicillin-Sens …
Forty-eight (18.5%) patients were labelled idiopathic, while Primary Ciliary Dyskinesia (PCD) accounted for 23 (8.8%) c …
Situs Ambiguus Is Associated With Adverse Clinical Outcomes in Children With Primary Ciliary Dyskinesia.
Kaspy KR, Dell SD, Davis SD, Ferkol TW, Rosenfeld M, Sagel SD, Milla C, Olivier KN, Barber AT, Wee W, Lin FC, Li L, Rampakakis E, Zariwala MA, Knowles MR, Leigh MW, Shapiro AJ. Kaspy KR, et al. Chest. 2024 May;165(5):1070-1081. doi: 10.1016/j.chest.2023.12.005. Epub 2023 Dec 9. Chest. 2024. PMID: 38072392
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare disorder of motile cilia associated with situs abnormalities. ...Compared with participants without SA, those with SA showed lower median BMI z scores (P = .03), lower FVC z scores (P = .01) …
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare disorder of motile cilia associated with situs abnormalities. .. …
Matrix metalloproteinases and airway remodeling and function in primary ciliary dyskinesia.
Pifferi M, Bush A, Caramella D, Metelli MR, Di Cicco M, Piras M, Gherarducci G, Capristo C, Maggi F, Peroni D, Boner AL. Pifferi M, et al. Respir Med. 2017 Mar;124:49-56. doi: 10.1016/j.rmed.2017.02.001. Epub 2017 Feb 2. Respir Med. 2017. PMID: 28284321 Free article.
OBJECTIVE: To evaluate whether they are important in the pathophysiology of primary and secondary ciliary dyskinesia (PCD, SCD). METHODS: We measured sputum bacteriology, lung CT changes, MMPs, TIMPs and lung function in 86 patients (51 PCD, 35 SCD) in a cros …
OBJECTIVE: To evaluate whether they are important in the pathophysiology of primary and secondary ciliary dyskinesia (P …
Pulmonary functions, nasal symptoms, and quality of life in patients with primary ciliary dyskinesia (PCD).
Gut G, Bar-Yoseph R, Hanna M, Brandl N, Alisha I, Rizik S, Pollak M, Hakim F, Amirav I, Bentur L, Gur M. Gut G, et al. Pediatr Pulmonol. 2024 Mar;59(3):688-694. doi: 10.1002/ppul.26814. Epub 2023 Dec 20. Pediatr Pulmonol. 2024. PMID: 38116904
BACKGROUND: Several factors may influence quality of life (QOL) for patients with primary ciliary dyskinesia (PCD). We aimed to evaluate the association between pulmonary functions, nasal symptoms and QOL in PCD patients. ...RESULTS: Twenty-seven patients (56 …
BACKGROUND: Several factors may influence quality of life (QOL) for patients with primary ciliary dyskinesia (PCD). We …
Characterising the nutritional status of children with primary ciliary dyskinesia.
Marino LV, Harris A, Johnstone C, Friend A, Newell C, Miles EA, Lucas JS, Calder PC, Walker WT. Marino LV, et al. Clin Nutr. 2019 Oct;38(5):2127-2135. doi: 10.1016/j.clnu.2018.08.034. Epub 2018 Sep 7. Clin Nutr. 2019. PMID: 30243504 Free article.
INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare, heterogeneous genetic disorder where impaired mucociliary clearance is caused by dysfunctional motile cilia leading to bronchiectasis. ...RESULTS: There was a weak positive association between height-f …
INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare, heterogeneous genetic disorder where impaired mucociliary cle …
Familial Recurrence Patterns in Congenitally Corrected Transposition of the Great Arteries: An International Study.
Tortigue M, Nield LE, Karakachoff M, McLeod CJ, Belli E, Babu-Narayan SV, Prigent S, Boet A, Conway M, Elder RW, Ladouceur M, Khairy P, Kowalik E, Kalfa DM, Barron DJ, Mussa S, Hiippala A, Temple J, Abadir S, Le Gloan L, Lachaud M, Sanatani S, Thambo JB, Gronier CG, Amedro P, Vaksmann G, Charbonneau A, Koutbi L, Ovaert C, Houeijeh A, Combes N, Maury P, Duthoit G, Hiel B, Erickson CC, Bonnet C, Van Hare GF, Dina C, Karsenty C, Fournier E, Le Bloa M, Pass RH, Liberman L, Happonen JM, Perry JC, Romefort B, Benbrik N, Hauet Q, Fraisse A, Gatzoulis MA, Abrams DJ, Dubin AM, Ho SY, Redon R, Bacha EA, Schott JJ, Baruteau AE. Tortigue M, et al. Circ Genom Precis Med. 2022 Jun;15(3):e003464. doi: 10.1161/CIRCGEN.121.003464. Epub 2022 May 12. Circ Genom Precis Med. 2022. PMID: 35549293
RESULTS: Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associated with primary ciliary dyskinesia in 11 patients. ...Familial clusters as well as evidence of an association between c …
RESULTS: Laterality defects and atrioventricular block at diagnosis were observed in 29.9% and 9.3%, respectively. ccTGA was associat …
Clinical impact of Pseudomonas aeruginosa colonization in patients with Primary Ciliary Dyskinesia.
Cohen-Cymberknoh M, Weigert N, Gileles-Hillel A, Breuer O, Simanovsky N, Boon M, De Boeck K, Barbato A, Snijders D, Collura M, Pradal U, Blau H, Mussaffi H, Price M, Bentur L, Gur M, Aviram M, Picard E, Shteinberg M, Livnat G, Rivlin J, Hiller N, Shoseyov D, Amirav I, Kerem E. Cohen-Cymberknoh M, et al. Respir Med. 2017 Oct;131:241-246. doi: 10.1016/j.rmed.2017.08.028. Epub 2017 Sep 1. Respir Med. 2017. PMID: 28947038 Free article.
BACKGROUND: Airway infections in Primary Ciliary Dyskinesia (PCD) are caused by different microorganisms, including pseudomonas aeruginosa (PA). ...The colonized group had significantly worse CT-Brody scores (36.07 24.38 vs. 25.56 24.2, p = 0.03 …
BACKGROUND: Airway infections in Primary Ciliary Dyskinesia (PCD) are caused by different microorganisms, including pse …
Objective sputum colour assessment and clinical outcomes in bronchiectasis: data from the European Bronchiectasis Registry (EMBARC).
Aliberti S, Ringshausen FC, Dhar R, Haworth CS, Loebinger MR, Dimakou K, Crichton ML, De Soyza A, Vendrell M, Burgel PR, McDonnell M, Skrgat S, Maiz Carro L, de Roux A, Sibila O, Bossios A, van der Eerden M, Kauppi P, Wilson R, Milenkovic B, Menendez R, Murris M, Borekci S, Munteanu O, Obradovic D, Nowinski A, Amorim A, Torres A, Lorent N, Van Braeckel E, Altenburg J, Shoemark A, Shteinberg M, Boersma W, Goeminne PC, Elborn JS, Hill AT, Welte T, Blasi F, Polverino E, Chalmers JD; EMBARC Registry Investigators. Aliberti S, et al. Eur Respir J. 2024 Apr 18;63(4):2301554. doi: 10.1183/13993003.01554-2023. Print 2024 Apr. Eur Respir J. 2024. PMID: 38609095 Free PMC article.
In the European Bronchiectasis Registry (EMBARC), we tested whether sputum colour would be associated with disease severity and clinical outcomes. METHODS: We used a prospective, observational registry of adults with bronchiectasis conducted in 31 countries. ...Compared to …
In the European Bronchiectasis Registry (EMBARC), we tested whether sputum colour would be associated with disease severity and clinical out …
Lung structure-function correlation in patients with primary ciliary dyskinesia.
Boon M, Vermeulen FL, Gysemans W, Proesmans M, Jorissen M, De Boeck K. Boon M, et al. Thorax. 2015 Apr;70(4):339-45. doi: 10.1136/thoraxjnl-2014-206578. Epub 2015 Feb 11. Thorax. 2015. PMID: 25673230
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare disease, characterised by chronic airway infection. ...RESULTS: LCI was abnormal in 28 of 38 patients and a moderate correlation was observed between LCI and FEV1 (r=-0.519, p=0.001). ...
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare disease, characterised by chronic airway infection. ...RESULTS: …
25 results