Search

Your search keyword '"Lazor, R."' showing total 236 results
Start Over Author "Lazor, R."
236 results on '"Lazor, R."'

6. Syndrome des télomères courts chez l’adulte : une entité rare qu’il faut savoir évoquer [Short telomere syndrome in adults: a rare entity that should be evoked]

Author

Coukos, A., Daccord, C., Lazor, R., Blum, S., Naveiras, O., Unger, S., Vionnet, J., Gaide, O., Koutsokera, A., Moschouri, E., Sempoux, C., Good, J.M., Moradpour, D., Baerlocher, G.M., and Fraga, M.

Abstract

Short telomere syndrome (STS) is a group of rare, often underrecognized, diseases caused by defects in telomere-maintenance genes, leading to abnormal telomere shortening and associated with diverse multi-organ manifestations. In pediatric patients, STS typically presents with mucocutaneous or gastrointestinal lesions, bone marrow failure and neoplasia. In adulthood, aplastic bone marrow disease, liver disease and pulmonary fibrosis are classic clinical manifestations. At present, medical treatment options for STS remain limited. Danazol, a synthetic androgenic hormone, can slow down telomere shortening and thus limit the progression of the disease. Finally, hematopoietic, hepatic and pulmonary transplantation, sometimes combined, may be discussed in a multidisciplinary setting in certain situations.

Published
2022

7. Pneumopathie interstitielle associée à la sclérodermie systémique : nouveaux développements [New developments in systemic sclerosis-associated interstitial lung disease]

Author

Humair, G., Daccord, C., Beigelman-Aubry, C., and Lazor, R.

Abstract

Interstitial lung disease is a frequent complication of systemic sclerosis and has now become the leading cause of death in this disorder. It mainly occurs during the first five years after the diagnosis of systemic sclerosis. Various risk factors are associated with the occurrence of interstitial lung disease, including the presence of anti-topoisomerase I antibodies (Scl-70) and the diffuse cutaneous form of systemic sclerosis. The most common radio-pathological presentation is nonspecific interstitial pneumonia, followed by usual interstitial pneumonia. The classical immunosuppressive treatment of systemic sclerosis-associated interstitial lung disease is evolving, as recent studies suggest a beneficial effect of biological agents such as rituximab and tocilizumab, and antifibrotic drugs such as nintedanib.

Published
2020

8. Airway Complications in Lung Transplant Recipients with Telomere-Related Interstitial Lung Disease

Author

Choi, B., primary, Messika, J., additional, Courtwright, A., additional, Mornex, J., additional, Hirschi, S., additional, Roux, A., additional, Le Pavec, J., additional, Quêtant, S., additional, Froidure, A., additional, Lazor, R., additional, Reynaud-Gaubert, M., additional, Le Borgne, A., additional, Goldberg, H., additional, El-Chemaly, S., additional, and Borie, R., additional

Published
2021
Full Text
View/download PDF

16. Pneumopathie interstitielle dans la ­polyarthrite rhumatoïde : nouvelles données génétiques et perspectives thérapeutiques [Rheumatoid arthritis-associated interstitial lung disease : new genetic data and therapeutic perspectives]

Author

Valerio, F., Daccord, C., Letovanec, I., Hügle, T., and Lazor, R.

Subjects
respiratory system, Arthritis, Rheumatoid/complications, Arthritis, Rheumatoid/genetics, Arthritis, Rheumatoid/therapy, Humans, Idiopathic Pulmonary Fibrosis/genetics, Idiopathic Pulmonary Fibrosis/therapy, Lung Diseases, Interstitial/genetics, Lung Diseases, Interstitial/therapy, Treatment Outcome, respiratory tract diseases
Abstract

Diffuse interstitial lung disease (ILD) is one of the most frequent extra-articular manifestation of rheumatoid arthritis (RA) and is an important factor of morbidity and mortality. However, the physiopathological mechanisms underlying RA-associated ILD remain poorly understood, and disease management is difficult in the absence of effective treatments and international guidelines. The recent identification of genetic variants and mutations similar to those observed in idiopathic pulmonary fibrosis (IPF), a disease affecting exclusively the lung, provides new insights into the understanding of RA-associated ILD. Furthermore, new antifibrotic drugs approved for the treatment of IPF, including pirfenidone and nintedanib, could also prove to be effective for RA-associated ILD. Studies are ongoing to confirm this hypothesis.

Published
2019

17. Isolated mediastinal necrotizing granulomatous lymphadenopathy due to cat-scratch disease

Author

Lovis, A., Clerc, O., Lazor, R., Jaton, K., Greub, G., Lovis, A., Clerc, O., Lazor, R., Jaton, K., and Greub, G.

Abstract

We report a patient suffering from cat-scratch disease limited to mediastinal lymphadenitis. Although rare, cat-scratch disease should be considered in the differential diagnosis of mediastinal lymphadenitis, especially when patients were exposed to cats.

Published
2019

20. Oil spills risks assessed for offshore arctic pipeline

Author

Dinovitzer, A., Comfort, G., Lazor, R, and Hinnah, D.

Subjects
Gas transmission industry -- Research, Business, Petroleum, energy and mining industries
Abstract

The details of quantitative risk analysis conducted to find the best alternative for designing gas pipelines in the Alaskan Beaufort Sea are presented. The steel pipe-in-pipe design, which is superior to other designs, is suggested.

Published
2004

21. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss): A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Cottin , V., Bel , E., Bottero , P., Dalhoff , K., Humbert , M., Lazor , R., Sinico , R.A., Sivasothy , P., Wechsler , M.E., Groh , M., Marchand-Adam , S., Khouatra , C., Wallaert , B., Taillé , C., Delaval , P., Cadranel , J., Bonniaud , P., Prévot , G., Hirschi , S., Gondouin , A., Dunogué , B., Chatté , G., Briault , C., Pagnoux , C., Jayne , D., Guillevin , L., Cordier , J.-F., Centre de Référence des Maladies Pulmonaires Rares [Hôpital Louis Pradel - HCL], Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Department of Pneumology [Lyon], Hospices Civils de Lyon (HCL), Academic Medical Center - Academisch Medisch Centrum [Amsterdam] (AMC), University of Amsterdam [Amsterdam] (UvA), Universität zu Lübeck = University of Lübeck [Lübeck], AP-HP Hôpital Bicêtre (Le Kremlin-Bicêtre), Université de Lausanne = University of Lausanne (UNIL), Cambridge University Hospitals NHS Foundation Trust, Addenbrookes Hospital, University of Colorado Anschutz [Aurora], Pathologies Respiratoires : Protéolyse et Aérosolthérapie, Université de Tours (UT)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Pneumologie et Immuno-Allergologie [CHU LIlle], Pole Cardio-vasculaire et pulmonaire [CHU Lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille)-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Service de Pneumologie [Bichat], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Diderot - Paris 7 (UPD7)-Centre de Compétence pour les Maladies Pulmonaires Rares, Institut de recherche en santé, environnement et travail (Irset), Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Theranoscan, Université Pierre et Marie Curie - Paris 6 (UPMC), Lipides - Nutrition - Cancer (U866) (LNC), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon (ENSBANA), Service des maladies respiratoires [CHU de Dijon], Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Protéines de choc thermique : mort cellulaire, différenciation cellulaire et propriétés tumorigéniques (U866, Cancer, équipe 3), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon (ENSBANA)-Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM)-AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon (ENSBANA), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, European Respiratory Society ERS TF 2008-08, Cottin, V, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, Wechsler, M, Groh, M, Marchand Adam, S, Khouatra, C, Wallaert, B, Taillé, C, Delaval, P, Cadranel, J, Bonniaud, P, Prévot, G, Hirschi, S, Gondouin, A, Dunogué, B, Chatté, G, Briault, C, Pagnoux, C, Jayne, D, Guillevin, L, Cordier, J, Universität zu Lübeck [Lübeck], Université de Lausanne (UNIL), Université de Tours-Institut National de la Santé et de la Recherche Médicale (INSERM), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Service de Pneumologie - Oncologie Thoracique - Maladies Pulmonaires Rares [CHU Tenon], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Hospices Civils de Lyon ( HCL ) -Hospices Civils de Lyon ( HCL ), Hospices Civils de Lyon ( HCL ), Academic Medical Center [Amsterdam] ( AMC ), University of Amsterdam [Amsterdam] ( UvA ), Université de Lausanne ( UNIL ), University of Colorado Health Sciences Center and National Jewish Medical and Research Center, Université de Tours-Institut National de la Santé et de la Recherche Médicale ( INSERM ), Service de Pneumologie et Immuno-Allergologie, Centre Hospitalier Régional Universitaire [Lille] ( CHRU Lille ), Assistance publique - Hôpitaux de Paris (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Université Paris Diderot - Paris 7 ( UPD7 ) -Centre de Compétence pour les Maladies Pulmonaires Rares, Institut de recherche, santé, environnement et travail ( Irset ), Université d'Angers ( UA ) -Université de Rennes 1 ( UR1 ), Université de Rennes ( UNIV-RENNES ) -Université de Rennes ( UNIV-RENNES ) -École des Hautes Études en Santé Publique [EHESP] ( EHESP ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ) -Université des Antilles ( UA ), Service de pneumologie et réanimation [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Université Pierre et Marie Curie - Paris 6 ( UPMC ), Lipides - Nutrition - Cancer (U866) ( LNC ), Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon ( ENSBANA ), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand ( CHU Dijon ), Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon ( ENSBANA ) -Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ) -AgroSup Dijon - Institut National Supérieur des Sciences Agronomiques, de l'Alimentation et de l'Environnement-Ecole Nationale Supérieure de Biologie Appliquée à la Nutrition et à l'Alimentation de Dijon ( ENSBANA ), Université Claude Bernard Lyon 1 ( UCBL ), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA)

Subjects
Adult, Male, [SDV.EE]Life Sciences [q-bio]/Ecology, environment, Vasculiti, ANCA, Eosinophilic granulomatosis with polyangiitis, Immunology, Churg-Strauss syndrome, Middle Aged, Prognosis, Asthma, vasculitis, [ SDV.EE ] Life Sciences [q-bio]/Ecology, environment, Treatment Outcome, classification, diagnostic criteria, Eosinophilic granulomatosis with polyangiiti, Humans, Immunology and Allergy, Female, Immunosuppressive Agents, Retrospective Studies
Abstract

International audience; Objective To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Methods Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. Results The study population included 157 patients (mean age 49.4 ± 14.1), with a follow-up of 7.4 ± 6.4 years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p = 0.005) and with the presence of ANCA (p

Published
2017

22. Supplementary Material for: Zystische Lungenerkrankungen bei genetisch bedingten Syndromen mit Funktionsausfall von Tumorsuppressorgenen

Author

Daccord, C., Nicod, L.P., and Lazor, R.

Abstract

Die zystischen Lungenerkrankungen sind eine distinkte Gruppe seltener Lungenkrankheiten. Zwei von ihnen sind die Folge monogener Defekte von Tumorsuppressorgenen: Die Lymphangioleiomyomatose - entweder sporadisch oder im Rahmen des Tuberöse-Sklerose-Komplexes - und das Birt-Hogg-Dubé-Syndrom. Diese Krankheiten weisen Gemeinsamkeiten im klinischen Erscheinungsbild auf, vom Auftreten im jungen Erwachsenenalter über multiple pulmonale Zysten, rezidivierenden Pneumothorax und Hamartome der Haut bis hin zu Nierentumoren. Sie unterscheiden sich jedoch deutlich im Hinblick auf die Verteilung auf die Geschlechter, die Pathogenese, den Krankheitsverlauf und die Prognose. In der Erforschung beider seltenen Krankheiten werden derzeit rapide Fortschritte gemacht. Beim Management der Lymphangioleiomyomatose sind in den letzten zehn Jahren wesentliche Verbesserungen erzielt worden, nachdem die pathogenen Mechanismen erforscht, eine wirksame Therapie entdeckt und große Kohortenstudien sowie internationale Leitlinien erstellt wurden. Das Birt-Hogg-Dubé-Syndrom wurde erst vor kürzerer Zeit erstmals beschrieben; die Klärung der pathophysiologischen Hintergründe steht noch aus.

Published
2018
Full Text
View/download PDF

24. A 12-WEEK COMBINATION OF CLARITHROMYCIN AND PREDNISONE COMPARED TO A 24-WEEK PREDNISONE ALONE TREATMENT IN CRYPTOGENIC AND RADIATION-INDUCED ORGANIZING PNEUMONIA

Author

Petitpierre, N., Cottin, V., sylvain marchand-adam, Hirschi, S., Rigaud, D., Court-Fortune, I., Jouneau, S., Israël-Biet, D., Molard, A., Cordier, J. -F, Lazor, R., Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), Service de Pneumologie [Hôpital Louis Pradel – CHU Lyon] (Centre de Référence des Maladies Pulmonaires Rares), CHU Lyon-Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), CHU Strasbourg, Centre Hospitalier Universitaire de Saint-Etienne (CHU de Saint-Etienne), Institut de recherche en santé, environnement et travail (Irset), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Institut National de la Santé et de la Recherche Médicale (INSERM)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Université d'Angers (UA), CHU Pontchaillou [Rennes], Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Centre Hospitalier Régional Universitaire de Tours (CHRU TOURS), Université d'Angers (UA)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Hôpital Louis Pradel [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL)-CHU Lyon, Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), and Université d'Angers (UA)-Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )

Subjects
Original Article: Clinical Research, recurrence, glucocorticoids, cryptogenic organizing pneumonia, [SDV]Life Sciences [q-bio], treatment outcome, interstitial, clarithromycin, lung diseases
Abstract

Background: Some data suggest that anti-inflammatory macrolides may be effective to treat organizing pneumonia (OP) and prevent relapses, but no formal comparison with prednisone alone is available. To explore this issue, we retrospectively compared the efficacy of a 12-week combined regimen of clarithromycin and prednisone with a 24-week prednisone alone regimen in OP. Methods: A standard 12-week regimen of combined clarithromycin and prednisone was designed for the treatment of cryptogenic or radiation-induced OP, aiming at reducing the cumulated prednisone dose and the relapse rate. Its use was left to the discretion of the treating physicians, members of the Groupe d’Etudes et de Recherche sur les Maladies Orphelines Pulmonaires. Data were compared to a historical control group treated with a standard 24-week prednisone alone regimen. Results: 16 patients were treated with combined therapy and 21 with prednisone alone. Complete radiological remission was achieved in 63% of the combined therapy group and 81% of the prednisone alone group (p=0.38). Symptomatic relapses occurred in 81% of the combined therapy group, and 52% of the prednisone alone group (p=0.14). No side effect of clarithromycin was reported. Conclusions: In patients with cryptogenic or radiation-induced OP, a 12-week regimen of clarithromycin and prednisone showed no benefit on remission rate and relapse rate as compared to a 24-week prednisone only regimen. (Sarcoidosis Vasc Diffuse Lung Dis 2018; 35: 230-238)

Published
2018

25. Évolution après transplantation pulmonaire pour fibrose chez les patients porteurs d’une mutation du complexe télomérase

Author

Phillips Houlbracq, M., primary, Mal, H., additional, Cottin, V., additional, Hirschi, S., additional, Roux, A., additional, Wémeau-Stervinou, L., additional, Le Pavec, J., additional, Claustre, J., additional, Park, S., additional, Marchand-Adam, S., additional, Froidure, A., additional, Lazor, R., additional, Naccache, J.M., additional, Jouneau, S., additional, Nunes, H., additional, Reynaud-Gaubert, M., additional, Prevot, G., additional, Crestani, B., additional, Kannengiesser, C., additional, and Borie, R., additional

Published
2019
Full Text
View/download PDF

26. Crystal structure and phase transitions in new series of double perovskite oxides Ba2-xSrxCaTeO6 (0≤x≤2): X-ray diffraction and Raman spectroscopy studies

Author

M. Jahid, A. El Hachmi, R. Abkar, M. A. El Aamrani, O. Ait Sidi Ahmed, L. Bih, P. Lazor, R. Haloui, B. Manoun, S. Louihi

Subjects
Ba2-xSrxCaTeO6 (0≤x≤2), X-ray diffraction, Raman spectroscopy, Crystal structure, Phase transition
Abstract

The structure stability of double perovskite ceramics Ba2-xSrxCaTeO6 (0≤x≤2) has been studied using X-ray powder diffraction (XRD) and Raman spectroscopy. According to the Rietveld refinement three phases transition induced by composition were reported. The transition from the cubic symmetry to tetragonal (Fmm → I4/m) is observed between x= 0.4 and 0.6. The second transition from tetragonal symmetry to monoclinic symmetry with I2/m as space group takes place between x = 0.6 and 0.8. The third phase transition monoclinic to monoclinic (I2/m → P21/n) is located between x = 1.4 and 1.6. Furthermore, considerable changes of the composition dependence of the Raman modes recorded at ambient conditions confirm the phase transitions reported by X-ray studies., Journal of Applied Surfaces and Interfaces, Vol 1, No 1-3 (2017)

Published
2017
Full Text
View/download PDF

27. Revisiting the systemic vasculitis in eosinophilic granulomatosis with polyangiitis (Churg-Strauss). A study of 157 patients by the Groupe d'Etudes et de Recherche sur les Maladies Orphelines Pulmonaires and the European Respiratory Society Taskforce on eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Cottin, V, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, Wechsler, M, Groh, M, Marchand Adam, S, Khouatra, C, Wallaert, B, Taillé, C, Delaval, P, Cadranel, J, Bonniaud, P, Prévot, G, Hirschi, S, Gondouin, A, Dunogué, B, Chatté, G, Briault, C, Pagnoux, C, Jayne, D, Guillevin, L, Cordier, J, Sinico, Ra, Cordier, J., Cottin, V, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, Wechsler, M, Groh, M, Marchand Adam, S, Khouatra, C, Wallaert, B, Taillé, C, Delaval, P, Cadranel, J, Bonniaud, P, Prévot, G, Hirschi, S, Gondouin, A, Dunogué, B, Chatté, G, Briault, C, Pagnoux, C, Jayne, D, Guillevin, L, Cordier, J, Sinico, Ra, and Cordier, J.

Abstract

Objective: To guide nosology and classification of patients with eosinophilic granulomatosis with polyangiitis (EGPA) based on phenotype and presence or absence of ANCA. Methods: Organ manifestations and ANCA status were retrospectively analyzed based on the presence or not of predefined definite vasculitis features or surrogates of vasculitis in patients asthma, eosinophilia, and at least one systemic organ manifestation attributable to systemic disease. Results: The study population included 157 patients (mean age 49.4. ±. 14.1), with a follow-up of 7.4. ±. 6.4. years. Patients with ANCA (31%) more frequently had weight loss, myalgias, arthralgias, biopsy-proven vasculitis, glomerulonephritis on biopsy, hematuria, leukocytoclastic capillaritis and/or eosinophilic infiltration of arterial wall on biopsy, and other renal disease. A total of 41% of patients had definite vasculitis manifestations (37%) or strong surrogates of vasculitis (4%), of whom only 53% had ANCA. Mononeuritis multiplex was associated with systemic vasculitis (p. =0.005) and with the presence of ANCA (p. <. 0.001). Overall, 59% of patients had . polyangiitis as defined by definite vasculitis, strong surrogate of vasculitis, mononeuritis multiplex, and/or ANCA with at least one systemic manifestation other than ENT or respiratory. Patients with . polyangiitis had more systemic manifestations including arthralgias (p. =0.02) and renal disease (p. =0.024), had higher peripheral eosinophilia (p. =0.027), and a trend towards less myocarditis (p. =0.057). Using predefined criteria of vasculitis and surrogates of vasculitis, ANCA alone were found to be insufficient to categorise patients with vasculitis features. Conclusion: We suggest a revised nomenclature and definition for EGPA and a new proposed entity referred to as hypereosinophilic asthma with systemic (non vasculitic) manifestations

Published
2017

28. Use of variability in national and regional data to estimate the prevalence of lymphangioleiomyomatosis

Author

Harknett, E.C., Chang, W.Y.C., Byrnes, S., Johnson, J., Lazor, R., Cohen, M.M., Gray, B., Geiling, S., Telford, H., Tattersfield, A.E., Hubbard, R.B., Johnson, S.R., Harknett, E.C., Chang, W.Y.C., Byrnes, S., Johnson, J., Lazor, R., Cohen, M.M., Gray, B., Geiling, S., Telford, H., Tattersfield, A.E., Hubbard, R.B., and Johnson, S.R.

Abstract

Background: Understanding the true prevalence of lymphangioleiomyomatosis (LAM) is important in estimating disease burden and targeting specific interventions. As with all rare diseases, obtaining reliable epidemiological data is difficult and requires innovative approaches. Aim: To determine the prevalence and incidence of LAM using data from patient organizations in seven countries, and to use the extent to which the prevalence of LAM varies regionally and nationally to determine whether prevalence estimates are related to health-care provision. Methods: Numbers of women with LAM were obtained from patient groups and national databases from seven countries (n = 1001). Prevalence was calculated for regions within countries using female population figures from census data. Incidence estimates were calculated for the USA, UK and Switzerland. Regional variation in prevalence and changes in incidence over time were analysed using Poisson regression and linear regression. Results: Prevalence of LAM in the seven countries ranged from 3.4 to 7.8/million women with significant variation, both between countries and between states in the USA. This variation did not relate to the number of pulmonary specialists in the region nor the percentage of population with health insurance, but suggests a large number of patients remain undiagnosed. The incidence of LAM from 2004 to 2008 ranged from 0.23 to 0.31/million women/per year in the USA, UK and Switzerland. Conclusions: Using this method, we have found that the prevalence of LAM is higher than that previously recorded and that many patients with LAM are undiagnosed

Published
2017

33. [Pulmonary lymphangioleiomyomatosis: From pathogenesis to management]

Author

Chebib, N., Khouatra, Chahéra, Lazor, R., Archer, Fabienne, Leroux, Caroline, Gamondes, D., Thivolet-Bejui, F., Cordier, Jean-Francois, Cottin, Vincent, Infections Virales et Pathologie Comparée - UMR 754 (IVPC), Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon, Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), and Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL)

Subjects
Angiomyolipome, Adult, History, Lung Neoplasms, Lymphangioleiomyomatosis/diagnosis/epidemiology/etiology/therapy, Tuberous sclerosis, [SDV]Life Sciences [q-bio], Lung Neoplasms/diagnosis/epidemiology/etiology/therapy, MTOR protein, History, 20th Century, Angiomyolipoma, Proteine mTOR, Sclerose tubereuse de Bourneville, 21st Century, History, 21st Century, Lymphangioleiomyomatose, 20th Century, Humans, Female, Lymphangioleiomyomatosis, Sirolimus
Abstract

INTRODUCTION: Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting mainly young women. BACKGROUND: The respiratory manifestations are characterized by a progressive cystic destruction of the lung parenchyma. Extrapulmonary involvement includes benign renal tumours called angiomyolipomas and abdominal lymphatic masses called lymphangioleiomyomas. At the pathological level, the cellular proliferation found in LAM is in part due to the presence of mutations in the tumour suppressor genes TSC1 and TSC2 (Tuberous Sclerosis Complex). These mutations lead to the activation of the mTOR pathway, which is currently the main therapeutic target. mTOR inhibitors such as sirolimus or everolimus have shown a beneficial effect on the decline in pulmonary function and a reduction of angiomyolipoma size, but are necessary in only some patients. PERSPECTIVES: LAM cells have migratory properties mediated by the formation of new lymphatic vessels. They are also able to secrete metalloproteases, which enhance their invasiveness. Moreover, the expression of estrogen and progesterone receptors by LAM cells suggests a possible role for sex hormones in the pathogenesis of the disease. CONCLUSION: A better understanding of mTOR-independent mechanisms would allow the development of novel therapeutic approaches.

Published
2015

34. Respiratory manifestations of eosinophilic granulomatosis with polyangiitis (Churg-Strauss)

Author

Cottin, V, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, Wechsler, M, Groh, M, Marchand Adam, S, Khouatra, C, Wallaert, B, Taillé, C, Delaval, P, Cadranel, J, Bonniaud, P, Prévot, G, Hirschi, S, Gondouin, A, Dunogué, B, Chatté, G, Briault, A, Jayne, D, Guillevin, L, Cordier, J, Cordier, J., SINICO, RENATO ALBERTO, Cottin, V, Bel, E, Bottero, P, Dalhoff, K, Humbert, M, Lazor, R, Sinico, R, Sivasothy, P, Wechsler, M, Groh, M, Marchand Adam, S, Khouatra, C, Wallaert, B, Taillé, C, Delaval, P, Cadranel, J, Bonniaud, P, Prévot, G, Hirschi, S, Gondouin, A, Dunogué, B, Chatté, G, Briault, A, Jayne, D, Guillevin, L, Cordier, J, Cordier, J., and SINICO, RENATO ALBERTO

Abstract

The respiratory manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) have not been studied in detail. In this retrospective multicentre study, EGPA was defined by asthma, eosinophilia and at least one new onset extra-bronchopulmonary organ manifestation of disease. The study population included 157 patients (mean±SD age 49.4±14.1 years), with a mean±SD blood eosinophil count of 7.4±6.4×109 L-1 at diagnosis. There was a mean±SD of 11.8±18.2 years from the onset of asthma to the diagnosis of EGPA, of 1.4±8.4 years from the first onset of peripheral eosinophilia to the diagnosis of EGPA, and of 7.4±6.4 years from EGPA diagnosis to the final visit. Despite inhaled and oral corticosteroid treatment, the severity of asthma increased 3-6 months before the onset of the systemic manifestations. Asthma was severe in 57%, 48%, and 56% of patients at diagnosis, at 3 years, and at the final visit, respectively. Persistent airflow obstruction was present in 38%, 30%, and 46% at diagnosis, at 3 years, and at the final visit, respectively. In EGPA, asthma is severe, antedates systemic manifestations by a mean of 12 years, and progresses to long-term persistent airflow obstruction despite corticosteroids in a large proportion of patients, which affects long-term management and morbidity.

Published
2016

35. DNAI1 mutations explain only 2% of primary ciliary dyskinesia

Author

Failly M, Saitta A, Muñoz A, Falconnet E, Dossier C, SANTAMARIA, FRANCESCA, de Santi MM, Lazor R, DeLozier Blanchet CD, Bartoloni L, Blouin J.L., Failly, M, Saitta, A, Muñoz, A, Falconnet, E, Dossier, C, Santamaria, Francesca, de Santi, Mm, Lazor, R, DeLozier Blanchet, Cd, Bartoloni, L, and Blouin, J. L.

Subjects
children, Primary Ciliary Dykinesia, mutation
Published
2008

36. [Granulomatous lymphocytic interstitial lung disease in common variable immunodeficiency]

Author

Mp, Bianchi, Letovanec I, Spertini F, Laurent P Nicod, and Lazor R

Subjects
Common Variable Immunodeficiency, Granuloma, Sarcoidosis, Recurrence, Humans, Immunoglobulins, Lung Diseases, Interstitial, Prognosis, Bronchiectasis
Abstract

Common variable immunodeficiency (CVID) is the most frequent primary immune deficiency. Recurrent infections are classical consequences of CVID, but their impact has been largely reduced by immunoglobulin replacement. CVID is also associated with various inflammatory and autoimmune manifestations resulting from abnormal cellular immunity. The lungs are especially affected by a recently described entity called granulomatous lymphocytic interstitial lung disease (GLILD). GLILD currently constitutes an important cause of morbidity and mortality in these patients. It is distinct from bronchiectasis secondary to recurrent infections, and presents similarities but also striking differences with sarcoidosis.

Published
2013

38. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss) (EGPA) Consensus Task Force recommendations for evaluation and management

Author

Groh, M, Pagnoux, C, Baldini, C, Bel, E, Bottero, P, Cottin, V, Dalhoff, K, Dunogué, B, Gross, W, Holle, J, Humbert, M, Jayne, D, Jennette, J, Lazor, R, Mahr, A, Merkel, P, Mouthon, L, Sinico, R, Specks, U, Vaglio, A, Wechsler, M, Cordier, J, Guillevin, L, Guillevin, L., SINICO, RENATO ALBERTO, Groh, M, Pagnoux, C, Baldini, C, Bel, E, Bottero, P, Cottin, V, Dalhoff, K, Dunogué, B, Gross, W, Holle, J, Humbert, M, Jayne, D, Jennette, J, Lazor, R, Mahr, A, Merkel, P, Mouthon, L, Sinico, R, Specks, U, Vaglio, A, Wechsler, M, Cordier, J, Guillevin, L, Guillevin, L., and SINICO, RENATO ALBERTO

Abstract

Objective To develop disease-specific recommendations for the diagnosis and management of eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) (EGPA). Methods The EGPA Consensus Task Force experts comprised 8 pulmonologists, 6 internists, 4 rheumatologists, 3 nephrologists, 1 pathologist and 1 allergist from 5 European countries and the USA. Using a modified Delphi process, a list of 40 questions was elaborated by 2 members and sent to all participants prior to the meeting. Concurrently, an extensive literature search was undertaken with publications assigned with a level of evidence according to accepted criteria. Drafts of the recommendations were circulated for review to all members until final consensus was reached. Results Twenty-two recommendations concerning the diagnosis, initial evaluation, treatment and monitoring of EGPA patients were established. The relevant published information on EGPA, antineutrophil-cytoplasm antibody-associated vasculitides, hypereosinophilic syndromes and eosinophilic asthma supporting these recommendations was also reviewed. Discussion These recommendations aim to give physicians tools for effective and individual management of EGPA patients, and to provide guidance for further targeted research.

Published
2015

39. Treatment of idiopathic pulmonary fibrosis with ambrisentan: a parallel, randomized trial

Author

Raghu, G, Behr, J, Brown, K, Egan, J, Kawut, S, Flaherty, K, Martinez, F, Nathan, S, Wells, A, Collard, H, Costabel, U, Richeldi, L, de Andrade, J, Khalil, N, Morrison, L, Lederer, D, Shao, L, Li, X, Pedersen, P, Montgomery, A, Chien, J, O'Riordan, T, Amin, D, Baker, A, Baratz, D, Baughman, R, Cagino, A, Chan, A, Chapman, J, Cordova, F, Edelman, J, Enelow, R, Ettinger, N, Glassberg, M, Golden, J, Ilowite, J, Kreider, M, Kureishy, S, Lancaster, L, Limper, A, Strek, M, Padilla, M, Fisher, M, Riley, D, Mohabir, P, Safdar, Z, Sahn, S, Schaumberg, T, Scholand, M, Smith, C, Sussman, R, Yung, G, Saggar, R, Geffen, D, Zibrak, J, Alvarez, J, Chan, K, Ruzi, J, Mcconnell, J, Mehta, J, Verghese, G, Talwar, A, Haddad, T, Sood, N, Goldberg, H, Sundar, K, Ziedalski, T, Gibson, K, Chan, C, Lien, D, Fell, C, Fox, G, Poirier, C, Provencher, S, Wilcox, P, Vilayi Weiler, Z, Kramer, M, Yigla, M, Baloira, A, Parakova, Z, Kra, H, Schwarz, Y, Martinez, C, Ben Dov, I, Kahler, C, Xaubet, A, Skrickova, J, Kolek, V, Parfrey, H, Echave Sustaeta, J, Wuyts, W, Geiser, T, Muller Quernheim, J, Whyte, M, Pfeifer, M, Grohe, C, Bourdin, A, Olschewski, H, Sibille, Y, Snizek, T, Vytiska, J, Pesek, M, Crestani, B, Wallaert, B, Chanez, P, Biet, D, Pompidou, G, Dromer, C, Gläser, S, Wagner, U, Witt, C, Herth, F, Hoeffken, G, Coswig, F, Breuer, R, Kerem, E, Adir, Y, Agostini, C, Cremona, G, Vitulo, P, Poletti, V, Rottoli, P, Rybacki, C, Piotrowski, W, Morera, J, Hattotuwa, K, Warburton, C, Corris, P, Leonard, C, Booth, H, Britton, M, Marchand Adam, S, Marquette, C, Tamm, M, Lazor, R, Chalmers, G, Hirani, N, De Vuyst, P, Saltini, C, Harari, S, Maher, T, Campos, F, Ramirez, A, Wehbe, L, Altieri, H, Fuchigami, A, Salinas, C, Mattos, W, Posadas, R, Fiss, E, Diaz Castanon, J, Munoz, S, Ramirez, L, Chercoff, J, Fritscher, C, Cardoso, A, Moreira, M, Steidle, L, Arakaki, J, Florenzano, M, Leon, L, Bernardini, S, Gilberto, A, Duque, C, Awad, C, Severiche, D, Lucro, D, Grimaldos, F, Rubin, A, Barrera, C, Ore, D, Heredia, C, Mazzei, J, Matiz, C, Glanville, A, Hopkins, P, Smallwood, D, Veitch, E, Musk, M, Glaspole, I, Wood Baker, R, Veale, A, and Costabel, Ulrich (Beitragende*r)

Subjects
Adult, Male, medicine.medical_specialty, Ambrisentan, Endothelin A Receptor Antagonists, Settore MED/10 - Malattie dell'Apparato Respiratorio, Medizin, Placebo, Idiopathic pulmonary fibrosis, Aged, Aged, 80 and over, Disease Progression, Double-Blind Method, Female, Humans, Idiopathic Pulmonary Fibrosis, Lung, Middle Aged, Phenylpropionates, Prospective Studies, Pyridazines, Treatment Outcome, Internal medicine, 80 and over, Internal Medicine, medicine, Clinical endpoint, Prospective cohort study, business.industry, Hazard ratio, General Medicine, medicine.disease, Interim analysis, Pulmonary hypertension, Surgery, business, medicine.drug
Abstract

BACKGROUND Idiopathic pulmonary fibrosis (IPF) is characterized by formation and proliferation of fibroblast foci. Endothelin-1 induces lung fibroblast proliferation and contractile activity via the endothelin A (ETA) receptor. OBJECTIVE To determine whether ambrisentan, an ETA receptor-selective antagonist, reduces the rate of IPF progression. DESIGN Randomized, double-blind, placebo-controlled, event-driven trial. (ClinicalTrials.gov: NCT00768300). SETTING Academic and private hospitals. PARTICIPANTS Patients with IPF aged 40 to 80 years with minimal or no honeycombing on high-resolution computed tomography scans. INTERVENTION Ambrisentan, 10 mg/d, or placebo. MEASUREMENTS Time to disease progression, defined as death, respiratory hospitalization, or a categorical decrease in lung function. RESULTS The study was terminated after enrollment of 492 patients (75% of intended enrollment; mean duration of exposure to study medication, 34.7 weeks) because an interim analysis indicated a low likelihood of showing efficacy for the end point by the scheduled end of the study. Ambrisentan-treated patients were more likely to meet the prespecified criteria for disease progression (90 [27.4%] vs. 28 [17.2%] patients; P = 0.010; hazard ratio, 1.74 [95% CI, 1.14 to 2.66]). Lung function decline was seen in 55 (16.7%) ambrisentan-treated patients and 19 (11.7%) placebo-treated patients (P = 0.109). Respiratory hospitalizations were seen in 44 (13.4%) and 9 (5.5%) patients in the ambrisentan and placebo groups, respectively (P = 0.007). Twenty-six (7.9%) patients who received ambrisentan and 6 (3.7%) who received placebo died (P = 0.100). Thirty-two (10%) ambrisentan-treated patients and 16 (10%) placebo-treated patients had pulmonary hypertension at baseline, and analysis stratified by the presence of pulmonary hypertension revealed similar results for the primary end point. LIMITATION The study was terminated early. CONCLUSION Ambrisentan was not effective in treating IPF and may be associated with an increased risk for disease progression and respiratory hospitalizations. PRIMARY FUNDING SOURCE Gilead Sciences.

Published
2013

40. Treatment of Idiopathic Pulmonary Fibrosis With Ambrisentan

Author

Raghu, G, Behr, J, Brown, Kk, Egan, Jj, Kawut, Sm, Flaherty, Kr, Martinez, Fj, Nathan, Sd, Wells, Au, Collard, Hr, Costabel, U, Richeldi, L, de Andrade, J, Khalil, N, Morrison, Ld, Lederer, Dj, Shao, L, Li, X, Pedersen, Ps, Montgomery, Ab, Chien, Jw, O'Riordan, Tg, ARTEMIS IPF Investigators: Amin, D, Baker, A, Baratz, D, Baughman, R, Cagino, A, Chan, A, Chapman, J, Cordova, F, Edelman, J, Enelow, R, Ettinger, N, Glassberg, M, Golden, J, Ilowite, J, Kreider, M, Kureishy, S, Lancaster, L, Lederer, D, Limper, A, Morrison, L, Nathan, S, Strek, M, Padilla, M, Fisher, M, Riley, D, Mohabir, P, Safdar, Z, Sahn, S, Schaumberg, T, Scholand, Mb, Smith, C, Sussman, R, Yung, G, Saggar, R, Geffen, D, Zibrak, J, Alvarez, J, Chan, K, Ruzi, J, Mcconnell, J, Mehta, J, Verghese, G, Talwar, A, Haddad, T, Sood, N, Goldberg, H, Sundar, K, Ziedalski, T, Gibson, K, Chan, C, Lien, D, Fell, C, Fox, G, Poirier, C, Provencher, S, Wilcox, P, Vilayi Weiler, Z, Kramer, M, Yigla, M, Baloira, A, Parakova, Z, Kra, H, Schwarz, Y, Martinez, C, Ben Dov, I, Kahler, C, Xaubet, A, Skrickova, J, Kolek, V, Parfrey, H, Echave Sustaeta, J, Wuyts, W, Geiser, T, Muller Quernheim, J, Whyte, M, Pfeifer, M, Grohe, C, Bourdin, A, Olschewski, H, Sibille, Y, Snizek, T, Vytiska, J, Pesek, M, Crestani, B, Wallaert, B, Chanez, P, Biet, Di, Pompidou, G, Dromer, C, Gläser, S, Wagner, U, Witt, C, Herth, F, Hoeffken, G, Coswig, F, Egan, J, Breuer, R, Kerem, E, Adir, Y, Agostini, Carlo, Cremona, G, Vitulo, P, Poletti, V, Rottoli, P, Rybacki, C, Piotrowski, W, Morera, J, Hattotuwa, K, Warburton, C, Corris, P, Leonard, C, Booth, H, Britton, M, Marchand Adam, S, Marquette, Ch, Tamm, M, Lazor, R, Chalmers, Gw, Hirani, N, De Vuyst, P, Saltini, C, Harari, Sa, Maher, T, Campos, F, Ramirez, A, Wehbe, L, Altieri, H, Fuchigami, Am, Salinas, Cc, Mattos, W, Posadas, R, Fiss, E, Diaz Castanon, J, Munoz, S, Ramirez, Ln, Chercoff, J, Fritscher, Cc, Cardoso, A, Moreira, Ma, Steidle, L, Arakaki, J, Florenzano, M, Leon, Lp, Bernardini, Su, Gilberto, A, Duque, Ca, Awad, C, Severiche, D, Lucro, De, Grimaldos, Fb, Rubin, A, Barrera, Ci, Ore, Dj, Heredia, C, Mazzei, J, Matiz, C, Glanville, A, Hopkins, P, Smallwood, D, Veitch, E, Musk, M, Glaspole, I, Wood Baker, R, and Veale, A.

Published
2013

41. [Computed tomography screening for lung cancer]

Author

Lazor R, Cornuz J, Lovis A, and Laurent P Nicod

Subjects
Lung Neoplasms, Risk Factors, Practice Guidelines as Topic, Humans, Mass Screening, False Positive Reactions, Tomography, X-Ray Computed, Physicians, Primary Care
Abstract

Lung cancer screening has been the focus of intense interest since the publication in 2011 of the NLST trial (National Lung Screening Trial) showing a mortality reduction in smokers undergoing 3-year screening by chest computed tomography. Although these data appear promising, many issues remain to be resolved, such as high rate of false positive cases, risk of overdiagnosis, optimal intervals between screens, duration of the screening process, feasibility, and cost. Structured screening programs appear crucial to guarantee patient information, technical quality, and multidisciplinary management. Despite these uncertainties, several guidelines already state that screening should be performed in patients at risk, whereas investigators stress that more data are needed. How should the primary care physician deal with individual patients requests? This review provides some clues on this complex issue.

Published
2012

43. Primary ciliary dyskinesia

Author

Lucas, J S A, Kuehni, C E, Lazor, R, and Walker, W T

Subjects
otorhinolaryngologic diseases
Abstract

Primary ciliary dyskinesia (PCD) is an autosomal recessive disease with an incidence estimated between 1:2,000 and 1:40,000. Ciliated epithelia line the airways, nasal and sinus cavities, Eustachian tube and fallopian tubes. Congenital abnormalities of ciliary structure and function impair mucociliary clearance. As a consequence, patients present with chronic sinopulmonary infections, recurrent glue ear and female subfertility. Similarities in the ultrastructure of respiratory cilia, nodal cilia and sperm result in patients with PCD also presenting with male infertility, abnormalities of left-right asymmetry (most commonly situs inversus totalis) and congenital heart disease. Early diagnosis is essential to ensure specialist management of the respiratory and otological complications of PCD. Diagnostic tests focus on analysis of ciliary function and electron microscopy structure. Analysis is technically difficult and labour intensive. It requires expertise for interpretation, restricting diagnosis to specialist centres. Management is currently based on the consensus of experts, and there is a pressing need for randomised clinical trials to inform treatment.

Published
2011
Full Text
View/download PDF

44. Maladies rares et médicaments orphelins: un défi de santé publique

Author

Lazor, R and D'Amato-Sizonenko, L.

Abstract

Définies par une prévalence inférieure à 1⁄2000, lesmaladies rares toucheraient 5 à 6 % de la population,soit environ 500 000 personnes en Suisse. Une vasteenquête européenne a révélé pour la première foisavec précision les difficultés et les besoins despatients atteints, et montré que leur prise en chargen'est pas optimale. Des plans nationaux pour lesmaladies rares ont été développés ou vont l'être aucours de prochaines années dans la plupart des payseuropéens. La Suisse a un retard de plusieurs annéesdans ce domaine, mais des initiatives récentespourraient permettre de le combler si elles sontlargement soutenues. Les médicaments orphelins,destinés à un petit nombre de patients mais souventextrêmement coûteux, sont une source potentiellede tensions entre intérêt individuel et intérêt collectif.Entité éthique, sociale, économique, mais aussiscientifique et clinique, les maladies rares et leurstraitements confrontent aux limites des connaissanceset des ressources. Elles constituent un enjeu de santépublique et un défi que la Suisse est invitée à relever.

Published
2011

45. Seltene Krankheiten und Orphan-Medikamente: eine Herausforderung für das Gesundheitswesen

Author

Lazor, R. and D'Amato-Sizonenko, L.

Abstract

Von seltenen Erkrankungen, die definitionsgemässeine Prävalenz von unter 1/2000 aufweisen, sindschätzungsweise 5 bis 6 % der Bevölkerung betroffen,d. h. in der Schweiz rund 500 000 Menschen.Eine breit angelegte Umfrage hat erstmals dieSchwierigkeiten und Bedürfnisse der betroffenenPatientinnen und Patienten genau erfasst und aufgezeigt,dass diese Menschen nicht optimal versorgtwerden. In den meisten europäischen Ländernbestehen bereits nationale Pläne für seltene Erkrankungenoder werden in den nächsten Jahren entwickelt.Die Schweiz weist in diesem Bereich einenRückstand von mehreren Jahren auf, der jedoch aufgeholtwerden könnte, falls die kürzlich eingeleitetenInitiativen breite Unterstützung erhalten. BeiOrphan-Medikamenten, die für eine kleine Zahl vonPatientinnen und Patienten bestimmt, aber oft sehrteuer sind, kann das Einzelinteresse dem Allgemeininteresseentgegenstehen. Die seltenen Erkrankungenund ihre Behandlung werfen ethische, gesellschaftliche,ökonomische, aber auch wissenschaftlicheund klinische Fragen auf und zeigen die Grenzendes Wissens und der Mittel auf. Sie sind ein Problemfür das Gesundheitswesen, und eine Herausforderung,der sich die Schweiz stellen sollte.

Published
2011

48. European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis

Author

Johnson, Sr1, Cordier, Jf, Lazor, R, Cottin, V, Costabel, U, Harari, S, Reynaud Gaubert, M, Boehler, A, Brauner, M, Popper, H, Bonetti, F, Kingswood, C, Review Panel of the ERS LAM Task Force including Johnson SR, Albera, Carlo, Bissler, J, Bouros, D, Corris, P, Donnelly, S, Durand, C, Egan, J, Grutters, Jc, Hodgson, U, Hollis, G, Korzeniewska Kosela, M, Kus, J, Lacronique, J, Lammers, Jw, Mccormack, F, Mendes, Ac, Moss, J, Naalsund, A, Pohl, W, Radzikowska, E, Robalo Cordeiro, C, Rouvière, O, Ryu, J, Schiavina, M, Tattersfield, Ae, Travis, W, Travis, P, Urban, T, Valeyre, D, Verleden, Gm, University of Nottingham, UK (UON), Rétrovirus et Pathologie Comparée, Institut National de la Recherche Agronomique (INRA)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Ecole Nationale Vétérinaire de Lyon (ENVL)-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon, Centre Hospitalier Universitaire Vaudois (CHUV), Universitätsklinikum Essen, Ospedale San Giuseppe, Hôpital Sainte Marguerite, University Hospital Zurich, Université Paris Nord (Paris 13), Karl-Franzens-Universität [Graz, Autriche], University of Verona (UNIVR), Bath, and University of Zurich

Subjects
Pulmonary and Respiratory Medicine, Pathology, medicine.medical_specialty, Angiomyolipoma, pneumothorax, chylous effusions, cystic lung disease, lymphangioleiomyomatosis, tuberous sclerosis, Medizin, 610 Medicine & health, Lung biopsy, Cell morphology, [SDV.MHEP.PSR]Life Sciences [q-bio]/Human health and pathology/Pulmonology and respiratory tract, 03 medical and health sciences, Tuberous sclerosis, 0302 clinical medicine, hemic and lymphatic diseases, medicine, Humans, 030212 general & internal medicine, ComputingMilieux_MISCELLANEOUS, business.industry, Respiratory disease, Chylothorax, medicine.disease, 3. Good health, 030228 respiratory system, Pneumothorax, 2740 Pulmonary and Respiratory Medicine, Lymphangioleiomyomatosis, lipids (amino acids, peptides, and proteins), 10178 Clinic for Pneumology, business, Lung Transplantation
Abstract

Lymphangioleiomyomatosis (LAM) is a rare lung disease, which occurs sporadically or in association with the genetic disease tuberous sclerosis complex (TSC) 1, 2. Sporadic LAM affects ∼1 in 400,000 adult females; in TSC, LAM occurs in 30–40% of adult females 3, 4 and exceptionally in males and children 5, 6. Patients with LAM usually develop progressive dyspnoea and recurrent pneumothorax, chylous collections and occasional haemoptysis 1. Extra pulmonary lymphadenopathy and cystic masses of the axial lymphatics termed lymphangioleiomyomas can result in abdominal and pelvic lymphatic obstruction 7. LAM is often associated with angiomyolipoma in the kidneys 8, and an increased frequency of meningioma 9. LAM varies in clinical features and rate of progression: this together with an absence of clear prognostic factors results in patients being given conflicting information about prognosis. Diagnosis is made by tissue biopsy (generally from the lung but occasionally from lymph nodes or lymphangioleiomyomas) and/or a combination of history and high-resolution computed tomography scanning (HRCT). Pathological diagnosis relies on characteristic LAM cell morphology and positive immunoreactivity to smooth muscle actin and HMB-45 antibodies. Increasingly HRCT is used to diagnose LAM without resorting to lung biopsy; however a number of conditions with multiple pulmonary cysts can mimic LAM. As LAM is rare, there have been no controlled trials of its management. Supportive treatment includes management of airflow obstruction and hypoxaemia with bronchodilators and oxygen respectively, specific treatment for surgical or pleural complications including pneumo- and chylothorax, and interventional treatment of renal lesions 10, 11. As LAM is a disease of females and is thought to be accelerated by oestrogen, oophorectomy, tamoxifen, progesterone and gonadotropin-releasing hormone (GnRH) analogues have been used without evidence that they are effective. The recent finding of abnormalities in the TSC1/2 genes resulting …

Published
2010

50. Early detection of lung cancer: A statement from an expert panel of the Swiss university hospitals on lung cancer screening

Author

Frauenfelder, T, Puhan, Milo A, Lazor, R, von Garnier, C, Bremerich, J, Niemann, T, Christe, A, Montet, X, Gautschi, O, Weder, W, Kohler, M, Frauenfelder, T, Puhan, Milo A, Lazor, R, von Garnier, C, Bremerich, J, Niemann, T, Christe, A, Montet, X, Gautschi, O, Weder, W, and Kohler, M

Abstract

The discussion about setting up a program for lung cancer screening was launched with the publication of the results of the National Lung Screening Trial, which suggested reduced mortality in high-risk subjects undergoing CT screening. However, important questions about the benefit-harm balance and the details of a screening program and its cost-effectiveness remain unanswered. A panel of specialists in chest radiology, respiratory medicine, epidemiology, and thoracic surgery representing all Swiss university hospitals prepared this joint statement following several meetings. The panel argues that premature and uncontrolled introduction of a lung cancer screening program may cause substantial harm that may remain undetected without rigorous quality control. This position paper focuses on the requirements of running such a program with the objective of harmonizing efforts across the involved specialties and institutions and defining quality standards. The underlying statement includes information on current evidence for a reduction in mortality with lung cancer screening and the potential epidemiologic implications of such a program in Switzerland. Furthermore, requirements for lung cancer screening centers are defined, and recommendations for both the CT technique and the algorithm for lung nodule assessment are provided. In addition, related issues such as patient management, registry, and funding are addressed. Based on the current state of the knowledge, the panel concludes that lung cancer screening in Switzerland should be undertaken exclusively within a national observational study in order to provide answers to several critical questions before considering broad population-based screening for lung cancer.

Published
2014

Catalog

Books, media, physical & digital resources
See catalog results