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Quoted phrase not found in phrase index: "Primary ciliary dyskinesia 17"
Page 1
Primary Ciliary Dyskinesia: First Health-related Quality-of-Life Measures for Pediatric Patients.
Dell SD, Leigh MW, Lucas JS, Ferkol TW, Knowles MR, Alpern A, Behan L, Morris AM, Hogg C, DunnGalvin A, Quittner AL. Dell SD, et al. Ann Am Thorac Soc. 2016 Oct;13(10):1726-1735. doi: 10.1513/AnnalsATS.201603-198OC. Ann Am Thorac Soc. 2016. PMID: 27464304 Free PMC article.
RATIONALE: Primary ciliary dyskinesia (PCD) is a rare disease. There are no available data on disease-specific pediatric patient-reported outcomes. ...Cognitive testing (n = 47; 20 males; age at diagnosis, 0-11 yr; FEV(1), 49-124% predicted) confirmed …
RATIONALE: Primary ciliary dyskinesia (PCD) is a rare disease. There are no available data on disease-specific pediatri …
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), and more hos …
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare disorder of motile cilia associated with situs abnormalities. .. …
Cardiopulmonary assessment in primary ciliary dyskinesia.
Valerio G, Giallauria F, Montella S, Vaino N, Vigorito C, Mirra V, Santamaria F. Valerio G, et al. Eur J Clin Invest. 2012 Jun;42(6):617-22. doi: 10.1111/j.1365-2362.2011.02626.x. Epub 2011 Nov 29. Eur J Clin Invest. 2012. PMID: 22121832
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare, usually autosomal recessive disorder of ciliary dysfunction associated with lung involvement, which has a great impact on health. ...RESULTS: Forty per cent of patients with PCD had impaired lung …
BACKGROUND: Primary ciliary dyskinesia (PCD) is a rare, usually autosomal recessive disorder of ciliary dysfunct …
COVID-19: Extensive epithelial damage and ciliary dyskinesia in hospitalised patients.
Lee DDH, Cardinale D, Saman Y, Hirst RA, Wilson N, Corden V, Rutman A, de Haro T, Hynds RE, McHugh T, Rea P, Smith CM, Oâ Callaghan C. Lee DDH, et al. Rhinology. 2022 Apr 1;60(2):155-158. doi: 10.4193/Rhin21.233. Rhinology. 2022. PMID: 35112671
Infection with SARS-CoV-2 can cause severe respiratory disease and it is predicted that the COVID-19 pandemic will leave a substantial number of patients with long-term respiratory complications (1)....
Infection with SARS-CoV-2 can cause severe respiratory disease and it is predicted that the COVID-19 pandemic will leave a substantia …
Changing clinical characteristics of non-cystic fibrosis bronchiectasis in children.
Eralp EE, Gokdemir Y, Atag E, Ikizoglu NB, Ergenekon P, Yegit CY, Kut A, Ersu R, Karakoc F, Karadag B. Eralp EE, et al. BMC Pulm Med. 2020 Jun 16;20(1):172. doi: 10.1186/s12890-020-01214-7. BMC Pulm Med. 2020. PMID: 32546272 Free PMC article.
Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last 12 months. RESULTS: Mean FEV1 and FVC %predicted at presentation improved compared to historical cohort (76.6 17.1 vs. 63.3 22.1 and …
Clinical outcomes were evaluated in terms of lung function tests and annual pulmonary exacerbation rates at presentation and within the last …
Ciliary disorientation alone as a cause of primary ciliary dyskinesia syndrome.
Rayner CF, Rutman A, Dewar A, Greenstone MA, Cole PJ, Wilson R. Rayner CF, et al. Am J Respir Crit Care Med. 1996 Mar;153(3):1123-9. doi: 10.1164/ajrccm.153.3.8630555. Am J Respir Crit Care Med. 1996. PMID: 8630555
Ciliary disorientation has been proposed as a variant of primary ciliary dyskinesia (PCD); cilia have normal ultrastructure and normal or near normal ciliary beat frequency (CBF) but lack efficacy because their beat direction is disorientated. .
Ciliary disorientation has been proposed as a variant of primary ciliary dyskinesia (PCD); cilia have normal ult
Using electrical impedance tomography to characterize lung impairment of children with primary ciliary dyskinesia: A pilot cross-sectional study.
Pensabene M, Gambazza S, Carta F, Rocchi A, Lelii M, Madini B, Hassan V, Piotto M, Patria MF. Pensabene M, et al. Pediatr Pulmonol. 2023 Apr;58(4):1051-1058. doi: 10.1002/ppul.26293. Epub 2022 Dec 29. Pediatr Pulmonol. 2023. PMID: 36571235 Free article.
BACKGROUND: In children with primary ciliary dyskinesia (PCD), measures more sensitive than spirometry are needed to characterize underlying pulmonary impairment. ...Using EIT, the global inhomogeneity (GI(TOT) ) index showed moderate to strong correlation wi …
BACKGROUND: In children with primary ciliary dyskinesia (PCD), measures more sensitive than spirometry are needed to ch …
A retrospective analysis of the pathogens in the airways of patients with primary ciliary dyskinesia.
Roden L, Görlich D, Omran H, Peters G, Große-Onnebrink J, Kahl BC. Roden L, et al. Respir Med. 2019 Sep;156:69-77. doi: 10.1016/j.rmed.2019.08.009. Epub 2019 Aug 15. Respir Med. 2019. PMID: 31437650 Free article.
INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare genetically heterogeneous disorder of motile cilia, which leads to recurrent and chronic airway infections. ...The culture of P. aeruginosa correlated negatively with age adjusted FEV(1)% predicted
INTRODUCTION: Primary ciliary dyskinesia (PCD) is a rare genetically heterogeneous disorder of motile cilia, which lead …
Longitudinal lung function and structural changes in children with primary ciliary dyskinesia.
Magnin ML, Cros P, Beydon N, Mahloul M, Tamalet A, Escudier E, Clément A, Le Pointe HD, Blanchon S. Magnin ML, et al. Pediatr Pulmonol. 2012 Aug;47(8):816-25. doi: 10.1002/ppul.22577. Epub 2012 May 8. Pediatr Pulmonol. 2012. PMID: 22570319 Free article.
BACKGROUND AND OBJECTIVES: Functional and structural lung evaluations are part of the follow-up of patients with primary ciliary dyskinesia (PCD). We aimed to evaluate transversal and longitudinal relationships between lung function test (LFT) and chest compu …
BACKGROUND AND OBJECTIVES: Functional and structural lung evaluations are part of the follow-up of patients with primary ciliary
A longitudinal study characterising a large adult primary ciliary dyskinesia population.
Shah A, Shoemark A, MacNeill SJ, Bhaludin B, Rogers A, Bilton D, Hansell DM, Wilson R, Loebinger MR. Shah A, et al. Eur Respir J. 2016 Aug;48(2):441-50. doi: 10.1183/13993003.00209-2016. Epub 2016 Jun 10. Eur Respir J. 2016. PMID: 27288033 Free article.
Primary ciliary dyskinesia (PCD) in adults has not been well described. ...Lung function decline was estimated at FEV1 decline of 0.49% predicted per year. Lung function decline was associated with ciliary ultrastructure, with microtubular defec
Primary ciliary dyskinesia (PCD) in adults has not been well described. ...Lung function decline was estimated at FEV1
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