12 results match your criteria: "Centre de référence des maladies pulmonaires rares (site constitutif)[Affiliation]"
Eur Respir J
September 2024
CHRU Tours, service de pneumologie et d'explorations fonctionnelles respiratoires, Tours, France
Chest
August 2024
Service de pneumologie, Centre de référence des maladies pulmonaires rares (site constitutif), hôpital Avicenne, Assistance Publique Hôpitaux de Paris, UMR Inserm U1272, Université Sorbonne Paris Nord, Bobigny, France.
Background: Sarcoidosis is an idiopathic systemic granulomatosis whose evolution is self-limiting in most cases. However, it can progress to organ damage that menaces the vital or functional prognosis of patients. Sarcoidosis itself, but also its comorbidities, can pose a threat to the patient, require rapid initiation of treatment, and justify emergency hospitalization.
View Article and Find Full Text PDFRespirology
August 2024
Université Paris Cité, Inserm, PHERE, F-75018 Paris, et Hôpital Bichat, APHP, Service de Pneumologie A, Centre constitutif du centre de référence des Maladies Pulmonaires Rares, FHU APOLLO, Paris, France.
Respir Med
October 2023
Biostatistics and Epidemiology Unit, Hôpitaux Universitaires Paris Centre, APHP, Université Paris Cité, Paris, France.
Background And Aim: Few questionnaires are available for routine assessment of dyspnea. The study aimed to design a self-administered questionnaire assessing the impact of chronic dyspnea on daily activities, named DYSLIM (Dyspnea-induced Limitation).
Methods: The development followed 4 steps: 1: selection of relevant activities and related questions (focus groups); 2: clinical study: internal and concurrent validity vs.
Eur Respir J
June 2023
CHRU Tours, Service de Pneumologie et d'Explorations Fonctionnelles Respiratoires, Tours, France
Background: Standard of care for interstitial lung disease (ILD) with a nonspecific interstitial pneumonia (NSIP) pattern proposes mycophenolate mofetil (MMF) as one of the first-step therapies while rituximab is used as rescue therapy.
Methods: In a randomised, double-blind, two-parallel group, placebo-controlled trial (NCT02990286), patients with connective tissue disease-associated ILD or idiopathic interstitial pneumonia (with or without autoimmune features) and a NSIP pattern (defined on NSIP pathological pattern or on integration of clinicobiological data and a NSIP-like high-resolution computed tomography pattern) were randomly assigned in a 1:1 ratio to receive rituximab (1000 mg) or placebo on day 1 and day 15 in addition to MMF (2 g daily) for 6 months. The primary end-point was the change in percent predicted forced vital capacity (FVC) from baseline to 6 months analysed by a linear mixed model for repeated measures analysis.
Eur Respir J
August 2022
Dept of Medicine and Surgery, University of Insubria, Varese, Italy.
Patients diagnosed with coronavirus disease 2019 (COVID-19) associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection frequently experience symptom burden post-acute infection or post-hospitalisation. We aimed to identify optimal strategies for follow-up care that may positively impact the patient's quality of life (QoL). A European Respiratory Society (ERS) Task Force convened and prioritised eight clinical questions.
View Article and Find Full Text PDFAm J Transplant
April 2022
Service de Pneumologie A, Centre de référence des maladies pulmonaires rares (site constitutif), APHP, Hôpital Bichat, Paris, France.
Carriers of germline telomerase-related gene (TRG) mutations can show poor prognosis, with an increase in common hematological complications after lung transplantation (LT) for pulmonary fibrosis. The aim of this study was to describe the outcomes after LT in recipients carrying a germline TRG mutation and to identify the predictors of survival. In a multicenter cohort of LT patients, we retrospectively reviewed those carrying pathogenic TRG variations (n = 38; TERT, n = 23, TERC, n = 9, RTEL1, n = 6) between 2009 and 2018.
View Article and Find Full Text PDFRespiration
May 2022
Sorbonne Université, Assistance Publique Hôpitaux de Paris, Hôpital Trousseau, Service de Pneumologie Pédiatrique, Centre de Référence des Maladies Respiratoires Rares, Paris, France.
Background: There is growing evidence of gender-specific phenotypic differences among patients with idiopathic pulmonary fibrosis (IPF), which may affect patient outcomes.
Objectives: We present the characteristics of patients with IPF at inclusion in the French Rare Disease Cohort - Interstitial Lung Disease (RaDiCo-ILD) with the aim of characterizing gender-specific phenotypic differences.
Methods: Patients with IPF who were enrolled in the national, multicentre RaDiCo-ILD cohort were included.
Eur Respir J
October 2021
Centre de référence des maladies pulmonaires rares (site constitutif), AP-HP, Service de Pneumologie, Hôpital Avicenne - Université Sorbonne Paris Nord, INSERM 1272, «Hypoxie et Poumon: pneumopathies fibrosantes, modulations ventilatoires et circulatoires», Bobigny, France.
Respir Med
July 2021
Centre de Référence des Maladies Pulmonaires Rares (site Constitutif), AP-HP, Service de Pneumologie, Hôpital Avicenne, Bobigny, France; Université Sorbonne Paris Nord, INSERM, 1272, « Hypoxie et Poumon: Pneumopathies Fibrosantes, Modulations Ventilatoires et Circulatoires », Bobigny, France. Electronic address:
ERJ Open Res
October 2020
Dept of Diseases of the Thorax, Ospedale GB Morgagni, Forli, Italy.
Presse Med
June 2020
Service de pneumologie, centre de référence des maladies pulmonaires rares (site constitutif), centre de compétence des maladies auto-immunes et systémiques rares, hôpital Avicenne, assistance publique-Hôpitaux de Paris, 93000 Bobigny, France; Inserm 1272, « Hypoxie et Poumon: pneumopathies fibrosantes, modulations ventilatoires et circulatoires », université de Sorbonne Paris Nord, 93000 Bobigny, France. Electronic address: