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Inaugural dropped head syndrome and camptocormia in inflammatory myopathies: a retrospective study.
Robert M, Lessard LER, Bouhour F, Petiot P, Fenouil T, Svahn J, Fiscus J, Fabien N, Perard L, Robinson P, Durieu I, Coury F, Streichenberger N, Hot A, Gallay L. Robert M, et al. Among authors: durieu i. Rheumatology (Oxford). 2024 Feb 1;63(2):506-515. doi: 10.1093/rheumatology/kead347. Rheumatology (Oxford). 2024. PMID: 37462538 Free PMC article.
CONCLUSION: The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in terms of clinical and outcome specificities, and proposes an adapted diagnostic and care management approach to prevent long-term compl …
CONCLUSION: The analysis of IM patients with inaugural DHS/CC delineates two groups of patients according to the age at first symptoms in te …
Characteristics of Patients With Late-Onset Pompe Disease in France: Insights From the French Pompe Registry in 2022.
Lefeuvre C, De Antonio M, Bouhour F, Tard C, Salort-Campana E, Lagrange E, Behin A, Sole G, Noury JB, Sacconi S, Magot A, Nadaj-Pakleza A, Lacour A, Beltran S, Spinazzi M, Cintas P, Renard D, Michaud M, Bedat-Millet AL, Prigent H, Taouagh N, Arrassi A, Hamroun D, Attarian S, Laforêt P; for Pompe Study Group. Lefeuvre C, et al. Neurology. 2023 Aug 29;101(9):e966-e977. doi: 10.1212/WNL.0000000000207547. Epub 2023 Jul 7. Neurology. 2023. PMID: 37419682
BACKGROUND AND OBJECTIVES: The French Pompe disease registry was created in 2004 for study of the natural course of the disease in patients. It rapidly became a major tool for assessing the long-term efficacy of enzyme replacement therapy (ERT) after the market release of …
BACKGROUND AND OBJECTIVES: The French Pompe disease registry was created in 2004 for study of the natural course of the disease in patients. …
Characterisation of a high-risk profile for maternal thrombotic and severe haemorrhagic complications in pregnant women with antiphospholipid syndrome in France (GR2): a multicentre, prospective, observational study.
Murarasu A, Guettrot-Imbert G, Le Guern V, Yelnik C, Queyrel V, Schleinitz N, Ferreira-Maldent N, Diot E, Urbanski G, Pannier E, Lazaro E, Souchaud-Debouverie O, Orquevaux P, Belhomme N, Morel N, Chauvet E, Maurier F, Le Besnerais M, Abisror N, Goulenok T, Sarrot-Reynauld F, Deroux A, Pasquier E, de Moreuil C, Bezanahary H, Pérard L, Limal N, Langlois V, Calas A, Godeau B, Lavigne C, Hachulla E, Cohen F, Benhamou Y, Raffray L, de Menthon M, Tieulié N, Poindron V, Mouthon L, Larosa M, Eléfant E, Sentilhes L, Molto A, Deneux-Tharaux C, Costedoat-Chalumeau N; GR2 Study Group. Murarasu A, et al. Lancet Rheumatol. 2022 Dec;4(12):e842-e852. doi: 10.1016/S2665-9913(22)00308-3. Epub 2022 Nov 24. Lancet Rheumatol. 2022. PMID: 38261392
FINDINGS: Between May 26, 2014, and April 17, 2020, 168 pregnancies in 27 centres met the inclusion criteria for the study. 89 (53%) of 168 women had a history of thrombosis. The median term at inclusion was 8 weeks gestation. 16 (10%) of 168 women (95%CI 5-15) had a throm …
FINDINGS: Between May 26, 2014, and April 17, 2020, 168 pregnancies in 27 centres met the inclusion criteria for the study. 89 (53%) of 168 …
Sustained effectiveness of elexacaftor-tezacaftor-ivacaftor in lung transplant candidates with cystic fibrosis.
Martin C, Reynaud-Gaubert M, Hamidfar R, Durieu I, Murris-Espin M, Danner-Boucher I, Chiron R, Leroy S, Douvry B, Grenet D, Mely L, Ramel S, Montcouquiol S, Lemonnier L, Burnet E, Paillasseur JL, Da Silva J, Burgel PR. Martin C, et al. Among authors: durieu i. J Cyst Fibros. 2022 May;21(3):489-496. doi: 10.1016/j.jcf.2022.01.012. Epub 2022 Feb 2. J Cyst Fibros. 2022. PMID: 35123901
BACKGROUND: Elexacaftor-tezacaftor-ivacaftor induces rapid clinical improvement in patients with cystic fibrosis (CF) and advanced pulmonary disease, often leading to suspend the indication for lung transplantation. Yet no long-term data is available in lung transplant can …
BACKGROUND: Elexacaftor-tezacaftor-ivacaftor induces rapid clinical improvement in patients with cystic fibrosis (CF) and advanced pulmonary …
Rapid Improvement after Starting Elexacaftor-Tezacaftor-Ivacaftor in Patients with Cystic Fibrosis and Advanced Pulmonary Disease.
Burgel PR, Durieu I, Chiron R, Ramel S, Danner-Boucher I, Prevotat A, Grenet D, Marguet C, Reynaud-Gaubert M, Macey J, Mely L, Fanton A, Quetant S, Lemonnier L, Paillasseur JL, Da Silva J, Martin C; French Cystic Fibrosis Reference Network Study Group. Burgel PR, et al. Among authors: durieu i. Am J Respir Crit Care Med. 2021 Jul 1;204(1):64-73. doi: 10.1164/rccm.202011-4153OC. Am J Respir Crit Care Med. 2021. PMID: 33600738

The mean (95% confidence interval) absolute increase in the ppFEV(1) was +15.1 (+13.8 to +16.4; P < 0.0001), and the mean (95% confidence interval) in weight was +4.2 kg (+3.9 to +4.6; P < 0.0001). The number of patients requiring long-term oxygen, noninvasive ventil

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The mean (95% confidence interval) absolute increase in the ppFEV(1) was +15.1 (+13.8 to +16.4; P < 0.0001), and the mean (95% confidence

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Long-term safety and efficacy of tezacaftor-ivacaftor in individuals with cystic fibrosis aged 12 years or older who are homozygous or heterozygous for Phe508del CFTR (EXTEND): an open-label extension study.
Flume PA, Biner RF, Downey DG, Brown C, Jain M, Fischer R, De Boeck K, Sawicki GS, Chang P, Paz-Diaz H, Rubin JL, Yang Y, Hu X, Pasta DJ, Millar SJ, Campbell D, Wang X, Ahluwalia N, Owen CA, Wainwright CE; VX14-661-110 study group. Flume PA, et al. Lancet Respir Med. 2021 Jul;9(7):733-746. doi: 10.1016/S2213-2600(20)30510-5. Epub 2021 Feb 10. Lancet Respir Med. 2021. PMID: 33581080
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability, and efficacy of tezacaftor-ivacaftor in participants aged 12 years or older with cystic fibrosis who were homozygous or heterozygous for t …
Here, we present results of study 661-110 (EXTEND), a 96-week open-label extension study that assessed long-term safety, tolerability …
First Wave of COVID-19 in French Patients with Cystic Fibrosis.
Corvol H, de Miranda S, Lemonnier L, Kemgang A, Reynaud Gaubert M, Chiron R, Dalphin ML, Durieu I, Dubus JC, Houdouin V, Prevotat A, Ramel S, Revillion M, Weiss L, Guillot L, Boelle PY, Burgel PR. Corvol H, et al. Among authors: durieu i. J Clin Med. 2020 Nov 10;9(11):3624. doi: 10.3390/jcm9113624. J Clin Med. 2020. PMID: 33182847 Free PMC article.
Ten developed complications (including acute respiratory distress syndrome in two post-lung transplant patients), but all recovered and were discharged home without noticeable short-term sequelae. Overall, French CF patients were rarely diagnosed with COVID-19. Further res …
Ten developed complications (including acute respiratory distress syndrome in two post-lung transplant patients), but all recovered and were …
Twice- or Once-Daily Dosing of Direct Oral Anticoagulants, a systematic review and meta-analysis.
Mainbourg S, Cucherat M, Provencher S, Bertoletti L, Nony P, Gueyffier F, Mismetti P, Grange C, Durieu I, Kilo R, Laporte S, Grenet G, Lega JC; META-EMBOL group. Mainbourg S, et al. Among authors: durieu i. Thromb Res. 2021 Jan;197:24-32. doi: 10.1016/j.thromres.2020.10.011. Epub 2020 Oct 15. Thromb Res. 2021. PMID: 33161284 Review.
CONCLUSION: Our study does not support a global difference in term of efficacy and safety of the BID and QD regimens of DOAC in MOS, NVAF, VTE and ACS....
CONCLUSION: Our study does not support a global difference in term of efficacy and safety of the BID and QD regimens of DOAC in MOS, …
Quantifying Long-Term Changes in Lung Function and Exacerbations after Initiation of Azithromycin in Cystic Fibrosis.
Denis A, Touzet S, Diabaté L, Durieu I, Lemonnier L, Poupon-Bourdy S, Iwaz J, Reynaud Q, Rabilloud M. Denis A, et al. Among authors: durieu i. Ann Am Thorac Soc. 2020 Feb;17(2):195-201. doi: 10.1513/AnnalsATS.201812-882OC. Ann Am Thorac Soc. 2020. PMID: 31604024
Rationale: In cystic fibrosis, information on the efficacy of azithromycin past 12 months of treatment is still scarce.Objectives: The study sought to quantify the changes in lung function and the number of intravenous antibiotic courses (IVACs) after initiation of azithromycin i …
Rationale: In cystic fibrosis, information on the efficacy of azithromycin past 12 months of treatment is still scarce.Objectives: The study …
33 results