-
1
المؤلفون: Charles Dumontet, Delphine Demangel, Perrine Galia, Lionel Karlin, Laurent Roche, Mathieu Fauvernier, Camille Golfier, Marie‐Charlotte Laude, Xavier Leleu, Philippe Rodon, Murielle Roussel, Isabelle Azaïs, Chantal Doyen, Borhane Slama, Salomon Manier, Olivier Decaux, Maroulio Pertesi, Marie Beaumont, Denis Caillot, Eileen M. Boyle, Manuel Cliquennois, Pascale Cony‐Makhoul, Anne‐Violaine Doncker, Véronique Dorvaux, Marie Odile Petillon, Jean Fontan, Bénédicte Hivert, Isabelle Leduc, Cécile Leyronnas, Margaret Macro, Michel Maigre, Clara Mariette, Philippe Mineur, Sophie Rigaudeau, Bruno Royer, Laure Vincent, James Mckay, Emeline Perrial, Laurent Garderet
المساهمون: Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL), Centre Léon Bérard [Lyon]-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hospices Civils de Lyon (HCL), Microenvironment and B-cells: Immunopathology,Cell Differentiation, and Cancer (MOBIDIC), Université de Rennes (UR)-Etablissement français du sang [Rennes] (EFS Bretagne)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Pontchaillou [Rennes], Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Fédération Française pour la Recherche contre le Myélome et les Gammapathies (FFRMG) Institut National Du Cancer, UCL - SSS/IREC/MONT - Pôle Mont Godinne, UCL - (MGD) Service d'hématologie
المصدر: American Journal of Hematology
American Journal of Hematology, 2023, 98 (2), pp.264-271. ⟨10.1002/ajh.26785⟩
American journal of hematology, Vol. 98, no.2, p. 264-271 (2023)مصطلحات موضوعية: Chromosome Aberrations, MESH: Humans, Paraproteinemias, MESH: Multiple Myeloma, [SDV.CAN]Life Sciences [q-bio]/Cancer, Hematology, Prognosis, Monoclonal Gammopathy of Undetermined Significance, MESH: Prognosis, MESH: Paraproteinemias, MESH: Child, MESH: Monoclonal Gammopathy of Undetermined Significance, Humans, MESH: Chromosome Aberrations, Child, Multiple Myeloma
الوصف: International audience; Familial forms of monoclonal gammopathy, defined as multiple myeloma (MM) or Monoclonal Gammopathy of Undetermined Significance (MGUS), are relatively infrequent and most series reported in the literature describe a limited number of families. MM rarely occurs in a familial context. MGUS is observed much more commonly, which can in some cases evolve toward full-blown MM. Although recurrent cytogenetic abnormalities have been described in tumor cells of sporadic cases of MM, the pathogenesis of familial MM remains largely unexplained. In order to identify genetic factors predisposing to familial monoclonal gammopathy, the Intergroupe Francophone du Myélome identified 318 families with at least two confirmed cases of monoclonal gammopathy. There were 169 families with parent/child pairs and 164 families with cases in at least two siblings, compatible with an autosomal transmission. These familial cases were compared with sporadic cases who were matched for age at diagnosis, sex and immunoglobulin isotype, with 10 sporadic cases for each familial case. The gender distribution, age and immunoglobulin subtypes of familial cases were unremarkable in comparison to sporadic cases. With a median follow-up of 7.4 years after diagnosis, the percentage of MGUS cases having evolved to MM was 3%. The median overall survival of the 148 familial MM cases was longer than that of matched sporadic cases, with projected values of 7.6 and 16.1 years in patients older and younger than 65 years, respectively. These data suggest that familial cases of monoclonal gammopathy are similar to sporadic cases in terms of clinical presentation and carry a better prognosis.
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::2bb8f2444f93c53edcfc2b2d1ed87766Test
https://univ-rennes.hal.science/hal-03980698Test -
2دورية أكاديمية
المؤلفون: Dumontet, Charles, Demangel, Delphine, Galia, Perrine, Karlin, Lionel, Roche, Laurent, Fauvernier, Mathieu, Golfier, Camille, Laude, Marie-Charlotte, Leleu, Xavier, Rodon, Philippe, Roussel, Murielle, Azaïs, Isabelle, Doyen, Chantal, Slama, Borhane, Manier, Salomon, Decaux, Olivier, Pertesi, Maroulio, Beaumont, Marie, Caillot, Denis, Boyle, Eileen, Cliquennois, Manuel, Cony-Makhoul, Pascale, Doncker, Anne-Violaine, Dorvaux, Véronique, Petillon, Marie Odile, Fontan, Jean, Hivert, Bénédicte, Leduc, Isabelle, Leyronnas, Cécile, Macro, Margaret, Maigre, Michel, Mariette, Clara, Mineur, Philippe, Rigaudeau, Sophie, Royer, Bruno, Vincent, Laure, Mckay, James, Perrial, Emeline, Garderet, Laurent
المساهمون: Centre de Recherche en Cancérologie de Lyon (UNICANCER/CRCL), Centre Léon Bérard Lyon -Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Hospices Civils de Lyon (HCL), Microenvironment and B-cells: Immunopathology,Cell Differentiation, and Cancer (MOBIDIC), Université de Rennes (UR)-Etablissement français du sang Rennes (EFS Bretagne)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Rennes CHU Rennes = Rennes University Hospital Ponchaillou, Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Fédération Française pour la Recherche contre le Myélome et les Gammapathies (FFRMG) Institut National Du Cancer
المصدر: ISSN: 0361-8609.
مصطلحات موضوعية: MESH: Child, MESH: Humans, MESH: Monoclonal Gammopathy of Undetermined Significance, MESH: Paraproteinemias, MESH: Multiple Myeloma, MESH: Prognosis, MESH: Chromosome Aberrations, [SDV.CAN]Life Sciences [q-bio]/Cancer
الوصف: International audience ; Familial forms of monoclonal gammopathy, defined as multiple myeloma (MM) or Monoclonal Gammopathy of Undetermined Significance (MGUS), are relatively infrequent and most series reported in the literature describe a limited number of families. MM rarely occurs in a familial context. MGUS is observed much more commonly, which can in some cases evolve toward full-blown MM. Although recurrent cytogenetic abnormalities have been described in tumor cells of sporadic cases of MM, the pathogenesis of familial MM remains largely unexplained. In order to identify genetic factors predisposing to familial monoclonal gammopathy, the Intergroupe Francophone du Myélome identified 318 families with at least two confirmed cases of monoclonal gammopathy. There were 169 families with parent/child pairs and 164 families with cases in at least two siblings, compatible with an autosomal transmission. These familial cases were compared with sporadic cases who were matched for age at diagnosis, sex and immunoglobulin isotype, with 10 sporadic cases for each familial case. The gender distribution, age and immunoglobulin subtypes of familial cases were unremarkable in comparison to sporadic cases. With a median follow-up of 7.4 years after diagnosis, the percentage of MGUS cases having evolved to MM was 3%. The median overall survival of the 148 familial MM cases was longer than that of matched sporadic cases, with projected values of 7.6 and 16.1 years in patients older and younger than 65 years, respectively. These data suggest that familial cases of monoclonal gammopathy are similar to sporadic cases in terms of clinical presentation and carry a better prognosis.
العلاقة: info:eu-repo/semantics/altIdentifier/pmid/36588407; hal-03980698; https://univ-rennes.hal.science/hal-03980698Test; PUBMED: 36588407
-
3دورية أكاديمية
المؤلفون: Ronsin, Charles, Kandel-Aznar, Christine, Le Bris, Yannick, Deltombe, Clément, Blin, Nicolas, Ville, Simon
المساهمون: Service de Néphrologie et Immunologie Clinique CHU de Nantes, Centre Hospitalier Universitaire de Nantes = Nantes University Hospital (CHU Nantes), Institut de Transplantation et de Recherche en Transplantation CHU Nantes (ITERT), Hôtel-Dieu de Nantes, Centre de Recherche en Cancérologie et Immunologie Nantes-Angers (CRCINA), Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-Centre Hospitalier Universitaire de Nantes = Nantes University Hospital (CHU Nantes)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Service d'Hématologie Clinique Nantes (Unité d'Investigation Clinique), Centre de Recherche en Transplantation et Immunologie - Center for Research in Transplantation and Translational Immunology (U1064 Inserm - CR2TI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Nantes Université - UFR de Médecine et des Techniques Médicales (Nantes Univ - UFR MEDECINE), Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)-Nantes Université - pôle Santé, Nantes Université (Nantes Univ)-Nantes Université (Nantes Univ)
المصدر: ISSN: 1121-8428.
مصطلحات موضوعية: Light-chain cast nephropathy, Lymphoplasmocytic lymphoma, Myeloma, Waldenström macroglobulinemia, MESH: Acute Kidney Injury / diagnosis, MESH: Acute Kidney Injury / etiology, MESH: Acute Kidney Injury / therapy, MESH: Humans, MESH: Immunoglobulin A, MESH: Multiple Myeloma, MESH: Nephrologists, MESH: Paraproteinemias / complications, MESH: Paraproteinemias / diagnosis, MESH: Waldenstrom Macroglobulinemia, [SDV]Life Sciences [q-bio]
الوصف: International audience
العلاقة: hal-04261587; https://hal.science/hal-04261587Test
-
4رسالة جامعية
المؤلفون: Burns, Robert Edward
المساهمون: UFR Médecine Santé - Université Paris Cité (UFR Médecine UPCité), Université Paris Cité (UPCité), Sébastien Mule
المصدر: https://dumas.ccsd.cnrs.fr/dumas-03637708Test ; Médecine humaine et pathologie. 2020.
مصطلحات موضوعية: PET/MRI, Hybrid Imaging, Monoclonal Gammopathy, WholeBbody Diffusion, Whole-Body MRI, 18F-FDG-PET, TEP/IRM, Imagerie hybride, Myélome multiple, Gammapathie monoclonale, Diffusion corps entier, 18F-FDG-TEP, MESH: Multiple myeloma, MESH: Paraproteinemias, MESH: Multimodal imaging, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology
الوصف: Purpose: PET/MRI can provide simultaneous whole-body (WB) acquisition of T1,T2DIXON, Diffusion Weighted Images (DWI), Dynamic Contrast-Enhanced (DCE), and FDG-PET sequences. The objective of this study was to assess and compare the diagnostic performance of 4 combinations of these sequences for baseline staging of patients with monoclonal gammopathies (MG). Materials and methods: 104 patients with MG were included in this IRB approved study. Readers reviewed initial PET/MRIs for the presence of focal lesions (FL) and bone marrow infiltration (BMI) using 4 different image datasets: WB-MRI (including DWI), 3DT1-PET, WB-PET/MRI and WB-DCE-PET/MRI. A reference standard was established by a panel review of all baseline and follow-up imaging, biological and pathological data. The diagnostic performance for each image dataset to detect FL and BMI were evaluated and compared (Fisher’s exact test). Results: The sensitivity, specificity and accuracy for FL of WB-MRI was 87, 97 and 92%; of 3DT1-PET was 78, 97, and 95%; of WB-PET-MRI was 93, 97, and 95%; WB-DCE-PET-MRI was 93, 97, and 95%. The sensitivity of WB-PET-MRI and WB-DCE-PET-MRI were superior to 3DT1-PET (p=0,036) without decreasing specificity. The sensitivity, specificity and accuracy of WB-MRI for BMI was 91, 80 and 85% ; of 3DT1-PET was 53, 89, and 74% ; of WB-PET-MRI was 98, 66, and 79% ; of WB-DCE-PET-MRI was 98, 59, and 75%. 3DT1-PET lacked sensitivity compared to all other datasets (p<0.0001). WB-MRI had better sensitivity than 3DT1-PET (p<0.001) and specificity than DCE-PET/MRI (p<0.001). Conclusion: PET/MRI should combine both WB bone marrow dedicated MRI and FDG PET images with no added value of DCE for baseline staging. ; L’objectif de cette étude était d'évaluer et de comparer les performances diagnostiques de 4 combinaisons de séquences en TEP/IRM pour le bilan initial des patients atteints de gammapathies monoclonales (GM). Matériel et Méthodes :104 patients avec une GM ont été inclus dans cette étude approuvée par l'IRB. Les lecteurs ont ...
العلاقة: dumas-03637708; https://dumas.ccsd.cnrs.fr/dumas-03637708Test; https://dumas.ccsd.cnrs.fr/dumas-03637708/documentTest; https://dumas.ccsd.cnrs.fr/dumas-03637708/file/ThEXE_Robert_Edward_BURNS_DUMAS.pdfTest
-
5دورية أكاديمية
المؤلفون: Colombat, Magali, Mal, Hervé, Diebold, Jacques, Copie-Bergman, Christiane, Damotte, Diane, Callard, Patrice, Fournier, Michel, Farcet, Jean-Pierre, Stern, Marc, Delfau-Larue, Marie-Hélène
المساهمون: CHU Tenon AP-HP, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de pneumologie B, 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), Service d'anatomie pathologique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôtel-Dieu, Département de pathologie Mondor, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 (UPEC UP12), Service d'anatomo-pathologie CHU HEGP, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-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)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service d'immunologie biologique, Service de pneumologie Hôpital Foch, Hôpital Foch Suresnes
المصدر: ISSN: 0006-4971.
مصطلحات موضوعية: MESH: Adult, MESH: Amino Acid Sequence, MESH: Immunoglobulin Heavy Chains, MESH: Immunoglobulin Light Chains, MESH: Immunoglobulin Variable Region, MESH: Immunoglobulin kappa-Chains, MESH: Lung Diseases, MESH: Lung Transplantation, MESH: Molecular Sequence Data, MESH: Paraproteinemias, MESH: B-Lymphocytes, MESH: Base Sequence, MESH: Cysts, MESH: DNA, MESH: Female, MESH: Genes, Immunoglobulin Heavy Chain, Immunoglobulin Light Chain, MESH: Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology
الوصف: International audience ; We have recently described a new form of light chain deposition disease (LCDD) presenting as a severe cystic lung disorder requiring lung transplantation. There was no bone marrow plasma cell proliferation. Because of the absence of disease recurrence after bilateral lung transplantation and of serum-free light chain ratio normalization after the procedure, we hypothesized that monoclonal light chain synthesis occurred within the lung. The aim of this study was to look for the monoclonal B-cell component in 3 patients with cystic lung LCDD. Histologic examination of the explanted lungs showed diffuse nonamyloid kappa light chain deposits associated with a mild lymphoid infiltrate composed of aggregates of small CD20(+), CD5(-), CD10(-) B lymphocytes reminiscent of bronchus-associated lymphoid tissue. Using polymerase chain reaction (PCR), we identified a dominant B-cell clone in the lung in the 3 studied patients. The clonal expansion of each patient shared an unmutated antigen receptor variable region sequence characterized by the use of IGHV4-34 and IGKV1 subgroups with heavy and light chain CDR3 sequences of more than 80% amino acid identity, a feature evocative of an antigen-driven process. Combined with clinical and biologic data, our results strongly argue for a new antigen-driven primary pulmonary lymphoproliferative disorder.
العلاقة: info:eu-repo/semantics/altIdentifier/pmid/18483396; inserm-00326865; https://inserm.hal.science/inserm-00326865Test; https://inserm.hal.science/inserm-00326865/documentTest; https://inserm.hal.science/inserm-00326865/file/inserm-00326865_edited.pdfTest; https://inserm.hal.science/inserm-00326865/file/LCDD.pdfTest; PUBMED: 18483396
الإتاحة: https://doi.org/10.1182/blood-2007-11-123596Test
https://inserm.hal.science/inserm-00326865Test
https://inserm.hal.science/inserm-00326865/documentTest
https://inserm.hal.science/inserm-00326865/file/inserm-00326865_edited.pdfTest
https://inserm.hal.science/inserm-00326865/file/LCDD.pdfTest -
6دورية أكاديمية
المؤلفون: Lafarge, L., Bourguignon, L., Bernard, N., Vial, T., Dehan-Moya, M.-J., de la Gastine, B., Goutelle, Sylvain
المساهمون: Université Claude Bernard Lyon 1 - Institut des Sciences Pharmaceutiques et Biologiques (UCBL ISPB), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon, Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université de Lyon-Université de Lyon-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS), Hospices Civils de Lyon (HCL), Evaluation et modélisation des effets thérapeutiques LBBE, Département biostatistiques et modélisation pour la santé et l'environnement LBBE, Université de Lyon-Université de Lyon-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-VetAgro Sup - Institut national d'enseignement supérieur et de recherche en alimentation, santé animale, sciences agronomiques et de l'environnement (VAS)-Centre National de la Recherche Scientifique (CNRS)-Laboratoire de Biométrie et Biologie Evolutive - UMR 5558 (LBBE), Université de Lyon
المصدر: ISSN: 0003-3928.
مصطلحات موضوعية: Adrenergic beta-antagonists, Aged, Bisoprolol, Bêta-bloquants, Personne âgée, Pharmacocinétique, Pharmacokinetics, MESH: Adrenergic beta-Antagonists, MESH: Aged, 80 and over, MESH: Humans, MESH: Myocardial Ischemia, MESH: Paraproteinemias, MESH: Renal Insufficiency, Chronic, MESH: Risk Factors, MESH: Aging, MESH: Atrial Fibrillation, MESH: Bisoprolol, MESH: Bradycardia, MESH: Depression, MESH: Drug Interactions, MESH: Drug Overdose, MESH: Female, [SDV.SP.PHARMA]Life Sciences [q-bio]/Pharmaceutical sciences/Pharmacology
الوصف: International audience ; Beta-blockers are widely prescribed in elderly patients and may induce severe adverse drug reactions. We report a case of bisoprolol-induced bradycardia in an elderly patient with impaired renal function and use of cytochrome P450 inhibitors. A literature review has been performed in order to analyze pharmacokinetic risk factors of beta-blockers overdosing in geriatrics. Various mechanisms can result in decreased elimination of beta-blockers. These mechanisms vary according to the beta-blocker agent and may be combined in some individuals, especially elderly patients. This can lead to unexpected overexposure. Knowledge about drug interactions and pharmacokinetic elimination pathways is important for preventing overexposure and adverse drug reactions when using beta-blockers.
العلاقة: info:eu-repo/semantics/altIdentifier/pmid/29544975; hal-04627209; https://hal.science/hal-04627209Test; PUBMED: 29544975
-
7دورية أكاديمية
المؤلفون: Luciani, Alessandro, Sirac, Christophe, Terryn, Sara, Javaugue, Vincent, Prange, Jenny Ann, Bender, Sébastien, Bonaud, Amélie, Cogné, Michel, Aucouturier, Pierre, Ronco, Pierre, Bridoux, Frank, Devuyst, Olivier
المساهمون: Universität Zürich Zürich = University of Zurich (UZH), Contrôle de la Réponse Immune B et des Lymphoproliférations (CRIBL), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)-Centre National de la Recherche Scientifique (CNRS), Université Catholique de Louvain = Catholic University of Louvain (UCL), Centre de Recherche Saint-Antoine (CRSA), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Des Maladies Rénales Rares aux Maladies Fréquentes, Remodelage et Réparation, Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM), CHU Tenon AP-HP, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre hospitalier universitaire de Poitiers = Poitiers University Hospital (CHU de Poitiers La Milétrie )
المصدر: ISSN: 1046-6673.
مصطلحات موضوعية: cell & transport physiology, endocytosis, multiple myeloma, proximal tubule, renal proximal tubule cell, MESH: Animals, MESH: Fanconi Syndrome, MESH: Immunoglobulin Light Chains, MESH: Lysosomes, MESH: Mice, Transgenic, MESH: Paraproteinemias, [SDV.IMM]Life Sciences [q-bio]/Immunology
الوصف: International audience ; Monoclonal gammopathies are frequently complicated by kidney lesions that increase the disease morbidity and mortality. In particular, abnormal Ig free light chains (LCs) may accumulate within epithelial cells, causing proximal tubule (PT) dysfunction and renal Fanconi syndrome (RFS). To investigate the mechanisms linking LC accumulation and PT dysfunction, we used transgenic mice overexpressing human control or RFS-associated κ LCs (RFS- κ LCs) and primary cultures of mouse PT cells exposed to low doses of corresponding human κ LCs (25 μ g/ml). Before the onset of renal failure, mice overexpressing RFS- κ LCs showed PT dysfunction related to loss of apical transporters and receptors and increased PT cell proliferation rates associated with lysosomal accumulation of κ LCs. Exposure of PT cells to RFS- κ LCs resulted in κ LC accumulation within enlarged and dysfunctional lysosomes, alteration of cellular dynamics, defective proteolysis and hydrolase maturation, and impaired lysosomal acidification. These changes were specific to the RFS- κ LC variable (V) sequence, because they did not occur with control LCs or the same RFS- κ LC carrying a single substitution (Ala30→Ser) in the V domain. The lysosomal alterations induced by RFS- κ LCs were reflected in increased cell proliferation, decreased apical expression of endocytic receptors, and defective endocytosis. These results reveal that specific κ LCs accumulate within lysosomes, altering lysosome dynamics and proteolytic function through defective acidification, thereby causing dedifferentiation and loss of reabsorptive capacity of PT cells. The characterization of these early events, which are similar to those encountered in congenital lysosomal disorders, provides a basis for the reported differential LC toxicity and new perspectives on LC-induced RFS.
العلاقة: info:eu-repo/semantics/altIdentifier/pmid/26614382; hal-02364437; https://hal.science/hal-02364437Test; PUBMED: 26614382; PUBMEDCENTRAL: PMC4926980
-
8
المؤلفون: Jean-Michel Vallat, Stéphane Mathis, Laurent Magy
المساهمون: Service de Neurologie [CHU Limoges], CHU Limoges, Biomolécules Thérapies anti-tumorales (EA4021), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503), Centre de référence national neuropathies périphériques rares [CHU Limoges]
المصدر: Current Opinion in Critical Care
Current Opinion in Critical Care, Lippincott, Williams & Wilkins, 2011, 17 (2), pp.101-5. ⟨10.1097/MCC.0b013e328342aded⟩مصطلحات موضوعية: Male, medicine.medical_specialty, [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology, Treatment outcome, Paraproteinemias, Consensus criteria, Chronic inflammatory demyelinating polyneuropathy, Critical Care and Intensive Care Medicine, Polyneuropathies, 03 medical and health sciences, MESH: Paraproteinemias, 0302 clinical medicine, Immune system, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], medicine, Humans, [SDV.BBM]Life Sciences [q-bio]/Biochemistry, Molecular Biology, Intensive care medicine, MESH: Treatment Outcome, 030304 developmental biology, [SDV.GEN]Life Sciences [q-bio]/Genetics, 0303 health sciences, MESH: Humans, MESH: Polyneuropathies, business.industry, [SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology, Polyradiculoneuropathy, medicine.disease, MESH: Male, MESH: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, 3. Good health, Treatment Outcome, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, Physical therapy, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Female, business, MESH: Female, 030217 neurology & neurosurgery
الوصف: International audience; PURPOSE OF REVIEW: Immune-mediated neuropathies are increasingly recognized as potential causes of disability. Their diagnostic criteria and management options are still a matter of debate. This review focuses on recent findings in the diagnostic and therapeutic management of these disorders and on their prognostic factors. RECENT FINDINGS: The more widespread use of consensus criteria has improved diagnostic strategies for immune-mediated neuropathies. Studies of large prospective series have defined the spectrum of these disorders and their respective prognoses. Although treatment of these disorders mostly relies on corticosteroids and intravenous immunoglobulin, recent controlled trials have indicated the value of targeted immunotherapies. The further development of these therapies requires better understanding of the pathophysiology of these disorders. Immunological and immunopathological studies have improved our understanding of immune-mediated neuropathies over the last few years. SUMMARY: Identifying immune-mediated neuropathies is important, as these disorders are treatable. Diagnostic strategies have improved our capacity to recognize affected patients who can be offered effective treatments. Therapeutic options for immune-mediated neuropathies are still rather limited, although targeted immunotherapies may improve the prognosis of these disorders in the near future.
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::e3482afe670445085d2f696a6f2c0d5cTest
https://doi.org/10.1097/mcc.0b013e328342adedTest -
9
المؤلفون: Laurent Magy, Jean-Michel Vallat
المساهمون: Service de Neurologie [CHU Limoges], CHU Limoges, Biomolécules Thérapies anti-tumorales (EA4021), Université de Limoges (UNILIM)-Génomique, Environnement, Immunité, Santé, Thérapeutique (GEIST FR CNRS 3503)
المصدر: Expert Opinion on Pharmacotherapy
Expert Opinion on Pharmacotherapy, Taylor & Francis, 2009, 10 (11), pp.1741-54. ⟨10.1517/14656560903036095⟩مصطلحات موضوعية: Pathology, MESH: Immunotherapy, [SDV.NEU.NB]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC]/Neurobiology, medicine.medical_treatment, Paraproteinemias, MESH: Antibodies, Monoclonal, law.invention, MESH: Paraproteinemias, 0302 clinical medicine, Randomized controlled trial, law, MESH: Motor Neuron Disease, MESH: Animals, Pharmacology (medical), Randomized Controlled Trials as Topic, 0303 health sciences, Antibodies, Monoclonal, Peripheral Nervous System Diseases, General Medicine, [SDV.SP]Life Sciences [q-bio]/Pharmaceutical sciences, 3. Good health, [SDV.NEU]Life Sciences [q-bio]/Neurons and Cognition [q-bio.NC], Immunotherapy, MESH: Immunosuppressive Agents, MESH: Peripheral Nervous System Diseases, Immunosuppressive Agents, medicine.medical_specialty, Evidence-based practice, medicine.drug_class, Monoclonal antibody, 03 medical and health sciences, [SDV.BBM.GTP]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Genomics [q-bio.GN], medicine, Animals, Humans, Motor Neuron Disease, Adverse effect, 030304 developmental biology, Pharmacology, MESH: Humans, business.industry, MESH: Chronic Disease, [SDV.BBM.BM]Life Sciences [q-bio]/Biochemistry, Molecular Biology/Molecular biology, Polyradiculoneuropathy, medicine.disease, Dermatology, MESH: Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, MESH: Randomized Controlled Trials as Topic, Peripheral neuropathy, Polyradiculoneuropathy, Chronic Inflammatory Demyelinating, [SDV.GEN.GH]Life Sciences [q-bio]/Genetics/Human genetics, Chronic Disease, business, 030217 neurology & neurosurgery, Multifocal motor neuropathy
الوصف: International audience; Immune-mediated neuropathies are treatable disorders that can result in considerable disability in some cases. Chronic immune-mediated neuropathies include chronic inflammatory demyelinating polyradiculoneuropathy, multifocal motor neuropathy and neuropathy associated with monoclonal gammopathy. In recent years, therapeutic strategies for these disorders have become clearer as a result of large, randomized controlled trials. However, the treatments used can have serious adverse effects and at least 30% of patients do not respond to conventional therapies. The aim of this paper is to review available data on evidence-based immunotherapy of chronic immune-mediated neuropathies and to summarize recent findings and results obtained with new innovative therapies, such as new immunosuppressants and monoclonal antibodies.
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::5f03d94d339c24fe4f49269f12daa70bTest
https://doi.org/10.1517/14656560903036095Test -
10
المؤلفون: Marc Stern, Marie-Hélène Delfau-Larue, Hervé Mal, Diane Damotte, Magali Colombat, Michel Fournier, Patrice Callard, Jacques Diebold, Jean-Pierre Farcet, Christiane Copie-Bergman
المساهمون: Service d'anatomie pathologique [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], Service de pneumologie B, Assistance publique - Hôpitaux de Paris (AP-HP)-AP-HP - Hôpital Bichat - Claude Bernard [Paris]-Université Paris Diderot - Paris 7 ( UPD7 ), Service d'anatomie pathologique, Assistance publique - Hôpitaux de Paris (AP-HP)-Hôtel-Dieu, Département de pathologie [Mondor], Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Henri Mondor-Université Paris-Est Créteil Val-de-Marne - Paris 12 ( UPEC UP12 ), Service d'anatomo-pathologie [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP)-Hôpital Européen Georges Pompidou [APHP] ( HEGP ), Service d'immunologie biologique, Service de pneumologie, Hôpital Foch [Suresnes]
المصدر: Blood
Blood, American Society of Hematology, 2008, 112 (5), pp.2004-12. 〈10.1182/blood-2007-11-123596〉مصطلحات موضوعية: Lung Diseases, Pathology, MESH : Molecular Sequence Data, MESH : DNA, medicine.medical_treatment, Immunoglobulin Variable Region, Paraproteinemias, MESH : Immunoglobulin Heavy Chains, Plasma cell, Biochemistry, 0302 clinical medicine, MESH : Female, MESH : Immunoglobulin Variable Region, B-Lymphocytes, 0303 health sciences, MESH : Lung Diseases, Cysts, MESH : Amino Acid Sequence, Hematology, MESH : Adult, MESH : Immunoglobulin Light Chains, 3. Good health, medicine.anatomical_structure, MESH : Paraproteinemias, 030220 oncology & carcinogenesis, MESH : Lung Transplantation, Female, Genes, Immunoglobulin Light Chain, Immunoglobulin Heavy Chains, Lung Transplantation, Adult, medicine.medical_specialty, Genes, Immunoglobulin Heavy Chain, Molecular Sequence Data, Immunology, MESH : Genes, Immunoglobulin Heavy Chain, MESH : Cysts, Biology, Immunoglobulin light chain, Article, Light chain deposition disease, MESH : B-Lymphocytes, Lung Disorder, Immunoglobulin kappa-Chains, 03 medical and health sciences, MESH : Immunoglobulin kappa-Chains, medicine, Humans, Lung transplantation, Amino Acid Sequence, [ SDV.BBM ] Life Sciences [q-bio]/Biochemistry, Molecular Biology, MESH : Genes, Immunoglobulin Light Chain, 030304 developmental biology, Lung, Base Sequence, MESH : Humans, DNA, Cell Biology, medicine.disease, Immunoglobulin heavy chain, MESH : Base Sequence, Immunoglobulin Light Chains, CD5
الوصف: We have recently described a new form of light chain deposition disease (LCDD) presenting as a severe cystic lung disorder requiring lung transplantation. There was no bone marrow plasma cell proliferation. Because of the absence of disease recurrence after bilateral lung transplantation and of serum-free light chain ratio normalization after the procedure, we hypothesized that monoclonal light chain synthesis occurred within the lung. The aim of this study was to look for the monoclonal B-cell component in 3 patients with cystic lung LCDD. Histologic examination of the explanted lungs showed diffuse nonamyloid κ light chain deposits associated with a mild lymphoid infiltrate composed of aggregates of small CD20+, CD5−, CD10− B lymphocytes reminiscent of bronchus-associated lymphoid tissue. Using polymerase chain reaction (PCR), we identified a dominant B-cell clone in the lung in the 3 studied patients. The clonal expansion of each patient shared an unmutated antigen receptor variable region sequence characterized by the use of IGHV4-34 and IGKV1 subgroups with heavy and light chain CDR3 sequences of more than 80% amino acid identity, a feature evocative of an antigen-driven process. Combined with clinical and biologic data, our results strongly argue for a new antigen-driven primary pulmonary lymphoproliferative disorder.
الوصول الحر: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8b121f36a9399468ebcd762f01ba3763Test
https://doi.org/10.1182/blood-2007-11-123596Test
