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1. Chronic Hepatitis C Virus Infection After Kidney Transplantation With or Without Direct-Acting Antivirals in a Real-Life Setting: A French Multicenter Experience

Author

Nicolas Bouvier, Yannick Le Meur, Cyril Garrouste, Dominique Bertrand, Johnny Sayegh, Eloi Chevallier, Matthias Büchler, Sophie Caillard, Joseph Rivalan, Antoine Thierry, Lionel Rostaing, Philippe Gatault, Charlotte Colosio, Jean-Philippe Rerolle, Service de néphrologie, dialyse, aphérèses et transplantation, CHU Grenoble-Université Joseph Fourier - Grenoble 1 (UJF), Centre Hospitalier Universitaire [Grenoble] (CHU), Laboratory of Immunology, CHRU de Tours, Tours, France, Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service de Néphrologie-Dialyse-Transplantation rénale [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Department of Nephrology, Reims University Hospital, Reims, France, Centre Hospitalier Universitaire de Rennes (CHU Rennes), Département de Néphrologie-Dialyse-Transplantation [CHU Angers], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Service de Néphrologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), CHRU Brest - Service de Nephrologie (CHU - BREST - Nephrologie), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Lymphocyte B et Auto-immunité (LBAI), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO), Département de Néphrologie [CHU Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de Néphrologie [Clermont-Ferrand], CHU Clermont-Ferrand-Université d'Auvergne - Clermont-Ferrand I (UdA), Service de néphrologie et immunologie clinique [CHRU Tours] (EA4245 UT), Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau-Université de Tours, Université de Brest (UBO)-Institut Brestois Santé Agro Matière (IBSAM), Université de Brest (UBO)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de néphrologie et immunologie clinique [CHRU Tours], Centre Hospitalier Régional Universitaire de Tours (CHRU Tours)-Hôpital Bretonneau-Université de Tours (UT), and Université Joseph Fourier - Grenoble 1 (UJF)-CHU Grenoble

Subjects
Adult, Male, Ledipasvir, medicine.medical_specialty, Pyrrolidines, Daclatasvir, Sustained Virologic Response, Sofosbuvir, 030232 urology & nephrology, Antiviral Agents, Gastroenterology, Group A, Virus, Group B, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Internal medicine, Humans, Medicine, Kidney transplantation, Retrospective Studies, Fluorenes, Transplantation, business.industry, Imidazoles, virus diseases, Valine, Hepatitis C, Chronic, Middle Aged, medicine.disease, Kidney Transplantation, 3. Good health, Treatment Outcome, chemistry, [SDV.IMM]Life Sciences [q-bio]/Immunology, Benzimidazoles, Female, 030211 gastroenterology & hepatology, Surgery, Carbamates, France, business, Viral hepatitis, medicine.drug
Abstract

Kidney transplant recipients (KTRs) are frequently infected with chronic hepatitis C virus (HCV), which can increase the risk of graft loss. Active HCV infections among KTRs are associated with shorter survival times. The emergence of very efficient interferon-free treatments (direct-acting antivirals [DAAs]) has revolutionized prognoses for chronic viral hepatitis. We performed a multicenter study where HCV (+)/RNA (+) KTRs were followed up and either received DAAs (group A) or not (group B) according to the transplant center. The aim was to assess, in a real-life setting, the impact of DAA therapy and to compare these results with those from HCV RNA (+) KTRs where HCV infection was not treated during the same period.This study included 66 patients from 11 centers: 44 patients (66.7%; group A) received DAAs, whereas 22 patients did not (group B); the 2 groups were comparable according to baseline data. Most patients (88.6%) received sofosbuvir, 50% received ledipasvir, and 34.7% received daclatasvir. The duration of treatments ranged from 8 to 24 weeks.HCV RNA clearance (ie, a sustained virologic response) was observed in 95.4% of treated patients. Eradication of HCV led to a significant decrease in liver enzymes (50% reduction for alanine aminotransferase [P ≤ .001] and 41% for gamma glutamyl transpeptidase [P .001]). Conversely, liver enzymes did not decrease in group B. Death occurred significantly more frequently in nontreated than treated patients (3 in group B vs none in group A, P = .003). Of the 10 treated patients with severe renal impairment before DAA therapy, 6 experienced graft loss.DAAs are very effective at treating chronic HCV and have an excellent tolerance profile.

Published
2020
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2. Qui sont ces patients en dialyse non inscrits sur liste d’attente de greffe rénale ?

Author

Elisabeth Monnet, Sahar Bayat, Christian Jacquelinet, Cécile Vigneau, Thierry Hannedouche, Elsa Vabret, Luc Frimat, CHU Pontchaillou [Rennes], 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 ), Recherche en Pharmaco-épidémiologie et Recours aux Soins (REPERES), Université de Rennes (UR)-École des Hautes Études en Santé Publique [EHESP] (EHESP), Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Agence de la biomédecine [Saint-Denis la Plaine], 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 de Rennes 1 (UR1), and Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-École des Hautes Études en Santé Publique [EHESP] (EHESP)

Subjects
03 medical and health sciences, Dialyse, 0302 clinical medicine, Contre-indication médicale, Nephrology, Liste d’attente de greffe, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Refus, Greffe rénale
Abstract

Resume L’acces a la greffe renale pour les patients insuffisants renaux chroniques terminaux est un enjeu majeur en France. L’inscription sur liste d’attente de greffe est une etape obligatoire pour pouvoir acceder a cette technique. La Haute Autorite de sante a mis a jour, en 2015, les contre-indications a l’acces a cette liste. Dans ce contexte, nous avons voulu realiser un etat des lieux des motifs de non-inscription sur la liste d’attente de greffe renale chez les patients dialyses depuis au moins un an, âges de moins de 80 ans, en France. Tous les patients inclus dans le registre d’epidemiologie et d’information en nephrologie (REIN), devant avoir leur point annuel de suivi du 1er aout au 30 novembre 2016 repondant aux criteres, ont ete inclus et un questionnaire dedie a ete rempli par leur centre de dialyse. Ainsi, 3172 patients ont ete analyses : 2302 (73 %) avaient une contre-indication medicale a la greffe, le plus souvent (33 %) vasculaire, 458 (14 %) refusaient d’etre inscrits, avec proportionnellement plus de femmes dans cette categorie et enfin, chez 412 patients (13 %), on ne retrouvait pas de motif renseigne dans notre questionnaire. Neanmoins, pour 65 % des patients de cette derniere categorie, un bilan en vue d’une inscription etait debute. Il y avait donc, dans notre cohorte, seulement 144 patients (4,5 %) sans explication a la non-inscription. Cette etude nationale est la premiere a donner a un etat des lieux des motifs de non-inscription sur liste de greffe renale en France. Sans surprise, la contre-indication medicale est le premier motif de non-inscription dans cette population de patients chroniques. Le fort taux de refus doit etre souligne et analyse plus precisement, notamment avec le ressenti du patient. Et enfin, tres peu de patients n’ont pas de motif renseigne ou de bilan en cours.

Published
2020
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3. Eculizumab discontinuation in children and adults with atypical hemolytic-uremic syndrome: a prospective multicenter study

Author

Véronique Frémeaux-Bacchi, Christiane Mousson, Emma Allain Launay, David Ribes, François Provôt, Simon Ville, Aurélie Le Thuaut, Claire Presne, Anne-Laure Sellier-Leclerc, Aurélie Hummel, Ferielle Louillet, Leila Tricot, Marc Fila, Quentin Raimbourg, Stéphane Bally, Fadi Fakhouri, Eric Rondeau, Ariane Zaloszyc, Moglie Le Quintrec, Djamal Djeddi, William Hanf, Guillaume Favre, Annie Lahoche, Valérie Châtelet, Sophie Caillard, Chantal Loirat, Jean-Philippe Coindre, Claire Pouteil-Noble, Yahsou Delmas, Olivia Boyer, Centre hospitalier universitaire de Nantes (CHU Nantes), Centre Hospitalier Universitaire Vaudois [Lausanne] (CHUV), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Service Néphrologie et transplantation rénale Adultes [CHU Necker], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie - Immunologie Clinique [Toulouse], CHU Toulouse [Toulouse]-Hôpital de Rangueil, CHU Toulouse [Toulouse]-PRES Université de Toulouse, Hospices Civils de Lyon (HCL), Centre Hospitalier Le Mans (CH Le Mans), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Urgences néphrologiques et transplantation rénale [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Centre de référence des microangiopathies thrombotiques [CHU Saint-Antoine] (Cnr-mat), Service d'hématologie clinique et de thérapie cellulaire [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Imagine - Institut des maladies génétiques (IHU) (Imagine - U1163), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Paris (UP), Service de néphrologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie et Transplantation rénale [CHRU-lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHU Amiens-Picardie, Centre Hospitalier Alpes Léman (CHAL), Service de Néphrologie-transplantation-dialyse [Bordeaux], CHU Bordeaux [Bordeaux], Service de Néphrologie [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU), Centre Hospitalier Universitaire de Nice (CHU Nice), Service de Néphrologie-Dialyse-Transplantation rénale [CHU Caen], Université de Caen Normandie (UNICAEN), Normandie Université (NU)-Normandie Université (NU)-CHU Caen, Normandie Université (NU)-Tumorothèque de Caen Basse-Normandie (TCBN)-Tumorothèque de Caen Basse-Normandie (TCBN), Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service de néphrologie - dialyse [Centre hospitalier Métropole Savoie - Chambéry], Centre Hospitalier Métropole Savoie [Chambéry], AP-HP - Hôpital Bichat - Claude Bernard [Paris], Hôpital Foch [Suresnes], Service de néphrologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), AP-HP Hôpital universitaire Robert-Debré [Paris], Service d'immunologie [HEGP, Paris], Hôpital Européen Georges Pompidou [APHP] (HEGP), and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)

Subjects
Adult, Male, medicine.medical_specialty, Adolescent, medicine.medical_treatment, Immunology, 030232 urology & nephrology, Renal function, Disease, 030204 cardiovascular system & hematology, Antibodies, Monoclonal, Humanized, urologic and male genital diseases, Biochemistry, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Quality of life, Internal medicine, Atypical hemolytic uremic syndrome, medicine, Humans, Prospective Studies, Child, Dialysis, Atypical Hemolytic Uremic Syndrome, business.industry, Infant, Newborn, Infant, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Cell Biology, Hematology, Eculizumab, Prognosis, medicine.disease, 3. Good health, Discontinuation, Survival Rate, Complement Inactivating Agents, Withholding Treatment, Child, Preschool, Female, business, BLOOD Commentary, Follow-Up Studies, Kidney disease, medicine.drug
Abstract

The optimal duration of eculizumab treatment in patients with atypical hemolytic uremic syndrome (aHUS) remains poorly defined. We conducted a prospective national multicenter open-label study to assess eculizumab discontinuation in children and adults with aHUS. Fifty-five patients (including 19 children) discontinued eculizumab (mean treatment duration, 16.5 months). Twenty-eight patients (51%) had rare variants in complement genes, mostly in MCP (n = 12; 22%), CFH (n = 6; 11%), and CFI (n = 6; 10%). At eculizumab discontinuation, 17 (30%) and 4 patients (7%) had stage 3 and 4 chronic kidney disease, respectively. During follow-up, 13 patients (23%; 6 children and 7 adults) experienced aHUS relapse. In multivariable analysis, female sex and presence of a rare variant in a complement gene were associated with an increased risk of aHUS relapse, whereas requirement for dialysis during a previous episode of acute aHUS was not. In addition, increased sC5b-9 plasma level at eculizumab discontinuation was associated with a higher risk of aHUS relapse in all patients and in the subset of carriers with a complement gene rare variant, both by log-rank test and in multivariable analysis. Of the 13 relapsing patients, all of whom restarted eculizumab, 11 regained their baseline renal function and 2 had a worsening of their preexisting chronic kidney disease, including 1 patient who progressed to end-stage renal disease. A strategy of eculizumab discontinuation in aHUS patients based on complement genetics is reasonable and safe. It improves the management and quality of life of a sizeable proportion of aHUS patients while reducing the cost of treatment. This trial was registered at www.clinicaltrials.gov as #NCT02574403.

Published
2021
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4. A regimen with caplacizumab, immunosuppression, and plasma exchange prevents unfavorable outcomes in immune-mediated TTP

Author

Jean-François Augusto, Naïke Bigé, Lionel Galicier, Alain Wynckel, Nihal Martis, Michael Bubenheim, Ygal Benhamou, Antoine Dossier, Claire Presne, Elie Azoulay, Virginie Rieu, Agnès Veyradier, Sandrine Malot, François Provôt, Christelle Barbet, Miguel Hie, Amélie Seguin, Sten de Witte, Pascale Poullin, M. Ulrich, Paul Coppo, Thierry Krummel, François Lhote, Yahsou Delmas, Pierre Perez, Anne Charvet Rumpler, Ruben Benainous, Olivier Moranne, Salvy-Córdoba, Nathalie, Centre de référence des microangiopathies thrombotiques [CHU Saint-Antoine] (Cnr-mat), Service d'hématologie clinique et de thérapie cellulaire [CHU Saint-Antoine], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Sorbonne Université (SU), Laboratoire d'Hématologie et d'Immunologie [CHU Saint-Antoine], Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité), Centre d'Investigation Clinique [CHU Rouen] (CIC Rouen), Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Hopital Saint-Louis [AP-HP] (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Université Paris Cité - UFR Médecine [Santé] (UPCité UFR Médecine), Université Paris Cité (UPCité), Service d'Immunopathologie [Hôpital Saint-Louis, Paris], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris Cité (UPCité), Service d'Anesthésie réanimation [CHU Saint-Antoine], CHU Saint-Antoine [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital de la Timone [CHU - APHM] (TIMONE), Service de Néphrologie [Hôpital Albert Calmette], Hôpital Albert Calmette [Lille], Centre Hospitalier Universitaire de Nice (CHU Nice), CHU Amiens-Picardie, Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Service de médecine interne [Avicenne], Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), AP-HP - Hôpital Bichat - Claude Bernard [Paris], Centre hospitalier universitaire de Nantes (CHU Nantes), Service de Médecine Interne 2, maladies auto-immunes et systémiques [CHU Pitié-Salpêtrière], Institut E3M [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Pitié-Salpêtrière [AP-HP], Hôpital Maison Blanche, Centre Hospitalier Universitaire de Reims (CHU Reims), CHU Bordeaux [Bordeaux], Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM), Hôpital Brabois, CHU, Partenaires INRAE, Service de Médecine Interne [CHU Clermont-Ferrand], CHU Gabriel Montpied [Clermont-Ferrand], CHU Clermont-Ferrand-CHU Clermont-Ferrand, Centre Hospitalier Régional Universitaire de Tours (CHRU Tours), Hôpital Delafontaine, Centre Hospitalier de Saint-Denis [Ile-de-France], Centre hospitalier [Valenciennes, Nord], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Centre Hospitalier Libourne, Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service d’Hématologie Biologique [CHU Lariboisière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Lariboisière-Fernand-Widal [APHP], Institut de Recherche Saint-Louis - Hématologie Immunologie Oncologie (Département de recherche de l’UFR de médecine, ex- Institut Universitaire Hématologie-IUH) (IRSL), Normandie Université (NU)-Normandie Université (NU), Endothélium, valvulopathies et insuffisance cardiaque (EnVI), Université de Rouen Normandie (UNIROUEN), and Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM)

Subjects
Compassionate Use Trials, Male, [SDV.MHEP.HEM] Life Sciences [q-bio]/Human health and pathology/Hematology, MESH: Combined Modality Therapy, MESH: Von Willebrand Factor, medicine.medical_treatment, Plenary Paper, Salvage therapy, 030204 cardiovascular system & hematology, Severity of Illness Index, Biochemistry, MESH: Historically Controlled Study, 0302 clinical medicine, Adrenal Cortex Hormones, Prospective Studies, Thiamine, Prospective cohort study, MESH: Treatment Outcome, MESH: Middle Aged, Plasma Exchange, MESH: Compassionate Use Trials, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Immunosuppression, Hematology, Middle Aged, MESH: Plasma Exchange, [SDV.SP]Life Sciences [q-bio]/Pharmaceutical sciences, Combined Modality Therapy, [SDV.SP] Life Sciences [q-bio]/Pharmaceutical sciences, Treatment Outcome, MESH: Thromboembolism, 030220 oncology & carcinogenesis, Disease Progression, Drug Therapy, Combination, Female, MESH: Disease Progression, Rituximab, MESH: Rituximab, MESH: Hemorrhage, medicine.drug, Adult, medicine.medical_specialty, MESH: Purpura, Thrombotic Thrombocytopenic, Immunology, Thrombotic thrombocytopenic purpura, ADAMTS13 Protein, Hemorrhage, MESH: Single-Domain Antibodies, MESH: Adrenal Cortex Hormones, 03 medical and health sciences, Thromboembolism, MESH: Severity of Illness Index, Internal medicine, von Willebrand Factor, medicine, Humans, MESH: Platelet Count, MESH: ADAMTS13 Protein, Adverse effect, Immunosuppression Therapy, MESH: Humans, Purpura, Thrombotic Thrombocytopenic, Platelet Count, business.industry, Historically Controlled Study, MESH: Adult, Cell Biology, Single-Domain Antibodies, medicine.disease, MESH: Male, MESH: Prospective Studies, MESH: Drug Therapy, Combination, Regimen, Caplacizumab, business, MESH: Female
Abstract

The anti–von Willebrand factor nanobody caplacizumab was licensed for adults with immune-mediated thrombotic thrombocytopenic purpura (iTTP) based on prospective controlled trials. However, few data are available on postmarketing surveillance. We treated 90 iTTP patients with a compassionate frontline triplet regimen associating therapeutic plasma exchange (TPE), immunosuppression with corticosteroids and rituximab, and caplacizumab. Outcomes were compared with 180 historical patients treated with the standard frontline treatment (TPE and corticosteroids, with rituximab as salvage therapy). The primary outcome was a composite of refractoriness and death within 30 days since diagnosis. Key secondary outcomes were exacerbations, time to platelet count recovery, the number of TPE, and the volume of plasma required to achieve durable remission. The percentage of patients in the triplet regimen with the composite primary outcome was 2.2% vs 12.2% in historical patients (P = .01). One elderly patient in the triplet regimen died of pulmonary embolism. Patients from this cohort experienced less exacerbations (3.4% vs 44%, P < .01); they recovered durable platelet count 1.8 times faster than historical patients (95% confidence interval, 1.41-2.36; P < .01), with fewer TPE sessions and lower plasma volumes (P < .01 both). The number of days in hospital was 41% lower in the triplet regimen than in the historical cohort (13 vs 22 days; P < .01). Caplacizumab-related adverse events occurred in 46 patients (51%), including 13 major or clinically relevant nonmajor hemorrhagic events. Associating caplacizumab to TPE and immunosuppression, by addressing the 3 processes of iTTP pathophysiology, prevents unfavorable outcomes and alleviates the burden of care.

Published
2021
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5. Vascular access cannulation and haemostasis: a national observational study of French practices

Author

Thierry Hannedouche, Marion Sallée, Jan-Marc Charrel, Bruno Guéry, Didier Borniche, Guillaume Jean, Lucile Mercadal, Philippe Brunet, Frank Le Roy, HAL-SU, Gestionnaire, Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), Service de Néphrologie [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Clinique Charcot, Service de néphrologie adultes [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), French Association of Nurses for Dialysis, Transplantation and Nephrology , (AFIDTN), Coordination Nationale Rein [Paris, France], Agence de la biomédecine [Saint-Denis la Plaine], Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Centre d'Investigation Clinique [CHU Rouen] (CIC Rouen), Hôpital Charles Nicolle [Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de néphrologie [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Hôpital Charles Nicolle [Rouen]-CHU Rouen, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), CHU Necker - Enfants Malades [AP-HP], and Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)

Subjects
medicine.medical_specialty, cannulation, medicine.medical_treatment, 030232 urology & nephrology, Vascular access, Arteriovenous fistula, 030204 cardiovascular system & hematology, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 03 medical and health sciences, 0302 clinical medicine, medicine, AcademicSubjects/MED00340, Hemostatic function, arteriovenous fistula, Dialysis, Transplantation, Local anaesthetic, business.industry, vascular access, Original Articles, catheter, medicine.disease, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 3. Good health, Surgery, haemodialysis, Catheter, Nephrology, haemostasis, dialysis, Observational study, Hemodialysis, business
Abstract

Background We report the results of an observational study of arteriovenous fistula (AVF) cannulation and haemostasis practices in France. Methods The study (sponsored by Brothier Pharmaceutical Inc.) was conducted in 150 dialysis units. Data obtained from 150 supervisory nurses, 1538 nurses and 3588 patients with an AVF were analysed. Results The nurses reported using rope-ladder, area or buttonhole cannulation techniques in 68, 26 and 6% of cases, respectively. Metal needles were used most frequently (64%), with mainly a diameter of 15 G or 16 G. The needle was introduced with the bevel up in 56% of cases. Compression applied using dressings (in particular, pure calcium alginate dressings) was the method of choice for haemostasis of the puncture sites and was assessed as being strong by most of the nurses and very strong in cases of prolonged bleeding. Most (82%) of the patients reported the use of local anaesthetic before cannulation and 23% reported an allergic skin reaction to the anaesthetic. Bleeding of the puncture sites lasted for >10 min for 48% of the patients and it reappeared between two sessions for 29% of the patients. Whereas the nurses appeared to have a good understanding of AVF, more than half of the patients did not know how to care for it, with 55% requiring more information. Conclusions This study underlines the lack of national consensus concerning AVF cannulation practices. It suggests that haemostasis methods of the puncture sites can be improved and it highlights the need to improve patient knowledge.

Published
2021
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6. IMPact of the COVID-19 epidemic on the moRTAlity of kidney transplant recipients and candidates in a French Nationwide registry sTudy (IMPORTANT)

Author

Olivier Thaunat, Camille Legeai, Dany Anglicheau, Lionel Couzi, Gilles Blancho, Marc Hazzan, Myriam Pastural, Emilie Savoye, Florian Bayer, Emmanuel Morelon, Yann Le Meur, Olivier Bastien, Sophie Caillard, Charlene Levi, Fanny Buron, Alice Koenig, Thomas Barba, Bruno Moulin, Samira Fafi-Kremer, Anglicheau Dany, Alexandre Hertig, Jérôme Tourret, Benoit Barrou, Pierre Merville, Anna Kaminski, Valérie Moal, Tristan Legris, Pierre-François Westeel, Maïté Jaureguy, Luc Frimat, Didier Ducloux, Jamal Bamoulid, Dominique Bertrand, Michel Tsimaratos, Florentine Garaix-Gilardo, Jérôme Dumortier, Sacha Mussot, Antoine Roux, Laurent Sebbag, Yannick Le Meur, Christophe Masset, Nassim Kamar, Hélène Francois, Eric Rondeau, Nicolas Bouvier, Christiane Mousson, Matthias Buchler, Philippe Gatault, Jean-François Augusto, Agnès Duveau, Cécile Vigneau, Marie-Christine Morin, Jonathan Chemouny, Leonard Golbin, Philippe Grimbert, Marie Matignon, Antoine Durrbach, Clarisse Greze, Renaud Snanoudj, Charlotte Colosio, Betoul Schvartz, Paolo Malvezzi, Christophe Mariat, Antoine Thierry, Moglie Le Quintrec, Antoine Sicard, Jean Philippe Rerolle, Anne-Élisabeth Heng, Cyril Garrouste, Henri Vacher Coponat, Éric Epailly, Olivier Brugiere, Sébastien Dharancy, Éphrem Salame, Faouzi Saliba, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL), Cellules B normales et pathogéniques - Normal and pathogenic B cell responses (NOPAB), Centre International de Recherche en Infectiologie - UMR (CIRI), Institut National de la Santé et de la Recherche Médicale (INSERM)-École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS)-Institut National de la Santé et de la Recherche Médicale (INSERM)-École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Centre National de la Recherche Scientifique (CNRS), Agence de la biomédecine [Saint-Denis la Plaine], Service Néphrologie et transplantation rénale Adultes [CHU Necker], CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie-transplantation-dialyse [Bordeaux], CHU Bordeaux [Bordeaux], Centre hospitalier universitaire de Nantes (CHU Nantes), Centre de Recherche en Transplantation et Immunologie (U1064 Inserm - CRTI), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Nantes - UFR de Médecine et des Techniques Médicales (UFR MEDECINE), Université de Nantes (UN)-Université de Nantes (UN), Institute for Translational Research in Inflammation - U 1286 (INFINITE (Ex-Liric)), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lille-Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), CHRU Brest - Service de Nephrologie (CHU - BREST - Nephrologie), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Université de Montpellier (UM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Centre International de Recherche en Infectiologie (CIRI), École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), CHU Lille, French nationwide Registry of Solid Organ Transplant Recipients with COVID-19: Olivier Thaunat, Emmanuel Morelon, Charlene Levi, Fanny Buron, Alice Koenig, Thomas Barba, Sophie Caillard, Bruno Moulin, Samira Fafi-Kremer, Marc Hazzan, Anglicheau Dany, Alexandre Hertig, Jérôme Tourret, Benoit Barrou, Lionel Couzi, Pierre Merville, Anna Kaminski, Valérie Moal, Tristan Legris, Pierre-François Westeel, Maïté Jaureguy, Luc Frimat, Didier Ducloux, Jamal Bamoulid, Dominique Bertrand, Michel Tsimaratos, Florentine Garaix-Gilardo, Jérôme Dumortier, Sacha Mussot, Antoine Roux, Laurent Sebbag, Yannick Le Meur, Gilles Blancho, Christophe Masset, Nassim Kamar, Hélène Francois, Eric Rondeau, Nicolas Bouvier, Christiane Mousson, Matthias Buchler, Philippe Gatault, Jean-François Augusto, Agnès Duveau, Cécile Vigneau, Marie-Christine Morin, Jonathan Chemouny, Leonard Golbin, Philippe Grimbert, Marie Matignon, Antoine Durrbach, Clarisse Greze, Renaud Snanoudj, Charlotte Colosio, Betoul Schvartz, Paolo Malvezzi, Christophe Mariat, Antoine Thierry, Moglie Le Quintrec, Antoine Sicard, Jean Philippe Rerolle, Anne-Élisabeth Heng, Cyril Garrouste, Henri Vacher Coponat, Éric Epailly, Olivier Brugiere, Sébastien Dharancy, Éphrem Salame, Faouzi Saliba, CCSD, Accord Elsevier, École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), and Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Male, 0301 basic medicine, Pediatrics, MESH: Registries, Registry study, 030232 urology & nephrology, Kidney transplant, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, MESH: Kidney Transplantation, Postoperative Complications, 0302 clinical medicine, MESH: Aged, 80 and over, MESH: Postoperative Complications, Pandemic, MESH: COVID-19, Registries, Wasting, Kidney transplantation, Aged, 80 and over, MESH: Aged, MESH: Middle Aged, Middle Aged, renal transplantation, 3. Good health, MESH: Waiting Lists, Nephrology, Female, France, medicine.symptom, Adult, medicine.medical_specialty, Waiting Lists, Coronavirus disease 2019 (COVID-19), 03 medical and health sciences, Intensive care, medicine, Humans, ESRD, Epidemics, MESH: Epidemics, Aged, Retrospective Studies, MESH: Humans, business.industry, SARS-CoV-2, COVID-19, Retrospective cohort study, MESH: Adult, MESH: Retrospective Studies, medicine.disease, Kidney Transplantation, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, mortality, MESH: Male, MESH: France, 030104 developmental biology, business, MESH: Female
Abstract

International audience; End stage kidney disease increase the risk of COVID-19 related death but how the kidney replacement strategy should be adapted during the pandemic is unknown. Chronic hemodialysis makes social distancing difficult to achieve. Alternatively, kidney transplantation could increase the severity of COVID-19 due to therapeutic immunosuppression and contribute to saturation of intensive care units. For these reasons, kidney transplantation was suspended in France during the first epidemic wave. Here, we retrospectively evaluated this strategy by comparing the overall and COVID-19 related mortality in kidney transplant recipients and candidates over the last three years. Cross-interrogation of two national registries for the period 1 March and 1 June 2020, identified 275 deaths among the 42812 kidney transplant recipients and 144 deaths among the 16210 candidates. This represents an excess of deaths for both populations, as compared with the same period the two previous years (mean of two previous years: 253 in recipients and 112 in candidates). This difference was integrally explained by COVID-19, which accounted for 44% (122) and 42% (60) of the deaths in recipients and candidates, respectively. Taking into account the size of the two populations and the geographical heterogeneity of virus circulation, we found that the excess of risk of death due to COVID-19 was similar for recipients and candidates in high viral risk area but four-fold higher for candidates in the low viral risk area. Thus, in case of a second epidemic wave, kidney transplantation should be suspended in high viral risk areas but maintained outside those areas, both to reduce the excess of deaths of candidates and avoid wasting precious resources.

Published
2020
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7. Short- and long-term renal outcomes following severe rhabdomyolysis: a French multicenter retrospective study of 387 patients

Author

Nelly Candela, Stein Silva, Bernard Georges, Claire Cartery, Thomas Robert, Julie Moussi-Frances, Eric Rondeau, Jean-Michel Rebibou, Laurence Lavayssiere, Julie Belliere, Thierry Krummel, Céline Lebas, Olivier Cointault, Marion Sallee, Stanislas Faguer, on behalf of the French Intensive Care Renal Network (F.I.R.N), Service de Néphrologie - Hypertension Artérielle Dialyse - Transplantation, CHU Toulouse [Toulouse]-Hôpital de Rangueil, CHU Toulouse [Toulouse], Hôpital Purpan [Toulouse], Service d'Anesthésie - Réanimation, Université Toulouse III - Paul Sabatier (UT3), Université Fédérale Toulouse Midi-Pyrénées-Université Fédérale Toulouse Midi-Pyrénées-CHU Toulouse [Toulouse]-Hôpital de Rangueil, Centre hospitalier [Valenciennes, Nord], Centre de néphrologie et transplantation rénale [Hôpital de la Conception - APHM], Hôpital de la Conception [CHU - APHM] (LA CONCEPTION)-Assistance Publique - Hôpitaux de Marseille (APHM), Urgences néphrologiques et transplantation rénale [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de néphrologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service de Néphrologie et Transplantation rénale [CHRU-lille], Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre recherche en CardioVasculaire et Nutrition = Center for CardioVascular and Nutrition research (C2VN), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National de Recherche pour l’Agriculture, l’Alimentation et l’Environnement (INRAE), Département de Néphrologie et Transplantation d'organes, Hôpital de Rangueil, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Université de Toulouse (UT)-Université de Toulouse (UT)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Assistance Publique - Hôpitaux de Marseille (APHM)-Hôpital de la Conception [CHU - APHM] (LA CONCEPTION), CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Département de Néphrologie et Transplantation d'organes [CHU Toulouse], Pôle Urologie - Néphrologie - Dialyse - Transplantations - Brûlés - Chirurgie plastique - Explorations fonctionnelles et physiologiques [CHU Toulouse], and Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)

Subjects
medicine.medical_specialty, Hyperkalemia, medicine.medical_treatment, CKD progression, 030232 urology & nephrology, Outcomes, Critical Care and Intensive Care Medicine, urologic and male genital diseases, Rhabdomyolysis, 03 medical and health sciences, 0302 clinical medicine, Anesthesiology, Internal medicine, medicine, Mechanical ventilation, business.industry, Myoglobin, Incidence (epidemiology), Research, Acute kidney injury, lcsh:Medical emergencies. Critical care. Intensive care. First aid, 030208 emergency & critical care medicine, Retrospective cohort study, lcsh:RC86-88.9, medicine.disease, female genital diseases and pregnancy complications, 3. Good health, medicine.symptom, business, [SDV.MHEP]Life Sciences [q-bio]/Human health and pathology, Kidney disease
Abstract

BackgroundRhabdomyolysis is a life-threatening disease that can lead to severe hyperkalemia, acute kidney injury (AKI) and hypovolemic shock. The predictive factors of AKI and acute to chronic kidney disease (CKD) transition remain poorly described.MethodsThis multicenter retrospective study enrolled 387 patients with severe rhabdomyolysis (CPK > 5000 U/L). Primary end-point was the development of severe AKI, defined as stage 2 or 3 of KDIGO classification. Secondary end-points included the incidence of AKI to CKD transition.ResultsAmong the 387 patients, 315 (81.4%) developed AKI, including 171 (44.1%) with stage 3 AKI and 103 (26.6%) requiring RRT. Stage 2–3 AKI was strongly correlated with serum phosphate, potassium and bicarbonate at admission, as well as myoglobin over 8000 U/L and the need for mechanical ventilation. 42 patients (10.8%) died before day 28. In the 80 patients with available eGFR values both before and 3 months after the rhabdomyolysis, the decrease in eGFR (greater than 20 mL/min/1.73 m2in 23 patients; 28.8%) was correlated to the severity of the AKI and serum myoglobin levels > 8000 U/L at admission.ConclusionsSevere rhabdomyolysis leads to AKI in most patients admitted to an ICU. Mechanical ventilation and severity of the rhabdomyolysis, including myoglobin level, are associated with the risk of stage 2–3 AKI. The long-term renal decline is correlated to serum myoglobin at admission.

Published
2020
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8. Deleterious effects of dialysis emergency start, insights from the French REIN registry

Author

Michel, Alain, Pladys, Adélaide, Bayat, Sahar, Couchoud, Cecile, Hannedouche, Thierry, Vigneau, Cécile, CHU Pontchaillou [Rennes], École des Hautes Études en Santé Publique [EHESP] (EHESP), Institut de Génétique et Développement de Rennes (IGDR), Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique )-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES), Registre REIN, Agence de la biomédecine [Saint-Denis la Plaine], Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service de néphrologie [Rennes], Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], Institut de recherche en 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 ), Troccaz, Olivier, Université de Rennes (UR)-Centre National de la Recherche Scientifique (CNRS)-Structure Fédérative de Recherche en Biologie et Santé de Rennes ( Biosit : Biologie - Santé - Innovation Technologique ), Université de Rennes (UR)-Hôpital Pontchaillou-CHU Pontchaillou [Rennes], 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
Male, Emergency Medical Services, Survival, Emergency start, lcsh:RC870-923, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Renal Dialysis, Humans, Registries, ESRD, Aged, Outcome, Aged, 80 and over, Correction, Middle Aged, lcsh:Diseases of the genitourinary system. Urology, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Survival Rate, Treatment Outcome, [SDV.SPEE] Life Sciences [q-bio]/Santé publique et épidémiologie, Kidney Failure, Chronic, [SDV.SPEE]Life Sciences [q-bio]/Santé publique et épidémiologie, Female, France, Dialysis, Follow-Up Studies
Abstract

International audience; Background: Emergency start (ES) of dialysis has been associated with worse outcome, but remains poorly documented. This study aims to compare the profile and outcome of a large cohort of patients starting dialysis as an emergency or as a planned step in France.Methods: Data on all patients aged 18 years or older who started dialysis in mainland France in 2012 or in 2006 were collected from the Renal Epidemiology and Information Network and compared, depending on the dialysis initiation condition: ES or Planned Start (PS). ES was defined as a first dialysis within 24 h after a nephrology visit due to a life-threatening event. Three-year survival were compared, and a multivariate model was performed after multiple imputation of missing data, to determine the parameters independently associated with three-year survival.Results: In 2012, 30.3% of all included patients (n = 8839) had ES. Comorbidities were more frequent in the ES than PS group (≥ 2 cardiovascular diseases: 39.2% vs 28.8%, p < 0.001). ES was independently associated with worse three-year survival (57% vs. 68.2%, p = 0.029, HR 1.10, 95% CI 1.01–1.19) in multivariate analysis. Among ES group, a large part had a consistent previous follow-up: 36.4% of them had ≥3 nephrology consultations in the previous year. This subgroup of patients had a particularly high comorbidity burden. ES rate was stable between 2006 and 2012, but some proactive regions succeeded in reducing markedly the ES rate.Conclusion: ES remains frequent and is independently associated with worse three-year survival, demonstrating that ES deleterious impact is never overcome. This study shows that a large part of patients with ES had a previous follow-up, but high comorbidity burden that could favor acute decompensation with life-threatening conditions before uremic symptoms appearance. This suggests the need of closer end-stage renal disease follow-up or early dialysis initiation in these high-risk patients.

Published
2018
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9. EPURE Transplant (Eplerenone in Patients Undergoing Renal Transplant) study: study protocol for a randomized controlled trial

Author

Philippe Rieu, Yannick Le Meur, Frederic Jaisser, Luc Frimat, Christophe Mariat, Sophie Girerd, Ludovic Merckle, Nicolas Girerd, Xavier Lepage, Didier Ducloux, Christiane Mousson, Nassim Dali-Youcef, Bruno Moulin, Patrick Rossignol, Service de Néphrologie [CHRU Nancy], Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy), Défaillance Cardiovasculaire Aiguë et Chronique (DCAC), Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL), Cardiovascular and Renal Clinical Trialists [Vandoeuvre-les-Nancy] (INI-CRCT), Institut Lorrain du Coeur et des Vaisseaux Louis Mathieu [Nancy], Université de Lorraine (UL), Service de Néphrologie [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHRU Brest - Service de Nephrologie (CHU - BREST - Nephrologie), Centre Hospitalier Régional Universitaire de Brest (CHRU Brest), Université de Brest (UBO), CHU Saint-Etienne, Université Jean Monnet - Saint-Étienne (UJM), Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Université de Strasbourg (UNISTRA), Service de néphrologie (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Université Bourgogne Franche-Comté [COMUE] (UBFC), Centre Hospitalier Universitaire de Reims (CHU Reims), Université de Reims Champagne-Ardenne (URCA), Institut de Génétique et de Biologie Moléculaire et Cellulaire (IGBMC), Université de Strasbourg (UNISTRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre d'investigation clinique [Nancy] (CIC), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU)-Université Paris Cité (UPCité), This work was supported by INI-CRCT. The EPURE Transplant study was supported by a grant from the French Ministry of Health (Programme Hospitalier de Recherche Clinique Inter Régional 2014). The authors are supported by a public grant overseen by the French National Research Agency (ANR) as part of the second 'Investissements d’Avenir' program (reference: ANR-15-RHU-0004), and GEENAGE Impact Lorraine Université d’Excellence- reference ANR-15-IDEX-04-LUE)., IMPACT GEENAGE, ANR-15-IDEX-0004,LUE,Isite LUE(2015), ANR-15-RHUS-0004,FIGHT-HF,Combattre l'insuffisance cardiaque(2015), DALI-YOUCEF, Nassim, ISITE - Isite LUE - - LUE2015 - ANR-15-IDEX-0004 - IDEX - VALID, Combattre l'insuffisance cardiaque - - FIGHT-HF2015 - ANR-15-RHUS-0004 - RHUS - VALID, Service de néphrologie-hémodialyse-transplantation [CHRU Nancy], Service d'urologie, andrologie et transplantation rénale, Hôpital Saint-Jacques-Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), CHRU - Service de néphrologie, dialyse et transplantation rénale, Service de néphrologie et tranplantation, CHU Saint-Etienne-Hôpital nord, Service de Néphrologie et Transplantation, Centre d'investigation clinique plurithématique Pierre Drouin [Nancy] (CIC-P), Université de Lorraine (UL)-Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)-Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Sorbonne Université - Faculté de Médecine (SU FM), Sorbonne Université (SU), Université Paris Descartes - Paris 5 (UPD5), Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon)-Hôpital Saint-Jacques, and Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)-Centre Hospitalier Régional Universitaire de Nancy (CHRU Nancy)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Lorraine (UL)

Subjects
medicine.medical_specialty, Hyperkalemia, medicine.medical_treatment, [SDV]Life Sciences [q-bio], Population, 030232 urology & nephrology, Urology, Medicine (miscellaneous), Renal function, 030204 cardiovascular system & hematology, Expanded Criteria Donor, law.invention, Kidney transplantation, 03 medical and health sciences, Study Protocol, 0302 clinical medicine, Randomized controlled trial, Double-Blind Method, law, medicine, Humans, Multicenter Studies as Topic, Pharmacology (medical), education, Dialysis, ComputingMilieux_MISCELLANEOUS, Mineralocorticoid Receptor Antagonists, Randomized Controlled Trials as Topic, education.field_of_study, lcsh:R5-920, business.industry, Mineralocorticoid receptor antagonist, Expanded criteria donor, medicine.disease, Tissue Donors, 3. Good health, Eplerenone, [SDV] Life Sciences [q-bio], Data Interpretation, Statistical, Reperfusion Injury, Hemodialysis, medicine.symptom, business, lcsh:Medicine (General), Ischemia/reperfusion lesions, Glomerular Filtration Rate
Abstract

Background Despite advances in immunosuppressive therapy, kidney graft survival has failed to improve during the last decades. Ischemia/reperfusion injury (IRI) is one of the main pathophysiological mechanisms underlying delayed graft function, which is associated with poor long-term graft survival. Due to organ shortage, the proportion of grafts from expanded criteria donors (ECDs) is ever growing. These grafts may particularly benefit from IRI prevention. In preclinical models, mineralocorticoid receptor antagonists (MRAs) have been shown to efficiently prevent IRI. This study aims to assess the effect of MRA administration in the early phase of kidney transplantation (KT) among recipients of ECD grafts on mid-term graft function. Methods/design This is a multicenter, double-blind, placebo-controlled, randomized clinical trial. Patients on hemodialysis and undergoing a single or a dual KT from an ECD will be eligible for inclusion. We plan to randomize 132 patients. Included patients will be randomized (1:1) to receive either eplerenone 25 mg every 12 h during 4 days (the first dose being administered just prior to KT) or placebo. The primary outcome is graft function at 3 months, assessed by glomerular filtration rate (GFR, in mL/min/1.73m2) measured using iohexol clearance. Secondary outcomes include (1) proportion of patients with either dialysis dependency or a GFR

Published
2018
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10. Pregnancy outcomes in simultaneous pancreas and kidney transplant recipients: a national French survey study

Author

Marielle Catton, Georges Karam, Flora Brunner, Olivier Thaunat, Diego Cantarovich, Gabrielle Normand, Laure Esposito, Sophie Caillard, Georges Mourad, Emmanuel Morelon, Fanny Buron, Jean E. Serre, Lionel Badet, Jérôme Massardier, Philippe Grimbert, Service de Néphrologie Hospices Civils - hta - dialyse, Service d'Urologie et Chirurgie de la Transplantation, Hôpital Edouard Herriot [CHU - HCL], Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Hospices Civils de Lyon (HCL), Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Service de Néphrologie - Hypertension Artérielle Dialyse - Transplantation, Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier), Cellules Souches, Plasticité Cellulaire, Médecine Régénératrice et Immunothérapies (IRMB), Centre Hospitalier Régional Universitaire [Montpellier] (CHRU Montpellier)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Montpellier (UM), Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Institut de transplantation urologie-néphrologie (ITUN), Université de Nantes (UN)-Centre hospitalier universitaire de Nantes (CHU Nantes), Lymphocytes B effecteurs et à mémoire – Effector and memory B cells, Centre International de Recherche en Infectiologie (CIRI), École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)-École normale supérieure de Lyon (ENS de Lyon)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Université Jean Monnet - Saint-Étienne (UJM)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Service de Transplantation, Néphrologie et Immunologie Clinique [Hôpital Edouard Herriot, HCL], Hospices Civils de Lyon (HCL)-Hôpital Edouard Herriot [CHU - HCL], CHU Toulouse [Toulouse]-Hôpital de Rangueil, CHU Toulouse [Toulouse], Service de Néphrologie Transplantation, Institut de Transplantation Urologie-Néphrologie (ITUN), Centre International de Recherche en Infectiologie - UMR (CIRI), École normale supérieure - Lyon (ENS 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)-École normale supérieure - Lyon (ENS Lyon)-Université Claude Bernard Lyon 1 (UCBL), and Université de Lyon-Université de Lyon-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS)

Subjects
Gestational hypertension, Adult, medicine.medical_specialty, Birth weight, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, kidney transplantation, 030230 surgery, simultaneous pancreas and kidney transplantation, 03 medical and health sciences, 0302 clinical medicine, Postoperative Complications, medicine, Humans, Kidney transplantation, Retrospective Studies, Transplantation, Type 1 diabetes, Pregnancy, Fetus, Obstetrics, business.industry, Graft Survival, Pregnancy Outcome, Gestational age, medicine.disease, 3. Good health, Surgery, Pregnancy Complications, Diabetes Mellitus, Type 1, Treatment Outcome, Kidney Failure, Chronic, Female, France, Pancreas Transplantation, pregnancy, business, Follow-Up Studies
Abstract

International audience; Simultaneous pancreas and kidney transplantation (SPK) is currently the best therapeutic option for patients with type 1 diabetes and terminal renal failure. Renal transplantation restores fertility enabling women to pursue pregnancies. However, scarcity of available data on pregnancy outcomes in SPK impedes fair medical counseling. Medical files of all pregnancies that lasted >=3 months among recipients of functional SPK performed between 1990 and 2015 in France were retrospectively analyzed. Twenty-six pregnancies in 22 SPK recipients were identified. Main maternal complications included gestational hypertension (53.8%) and infections (50%). Cesarean section was performed in 73% of cases. Overall fetal survival was 92.6% with a mean gestational age of 34.2 \textpm 3 weeks. Four children (16.7% of live births) had a birth weight \textless10th percentile. Endocrine pancreas graft function remained stable during pregnancy. An acute kidney rejection occurred in two patients, one of which resulting in graft loss. Kidney and pancreas graft survival was, respectively, 96% and 100% at 1 year postconception and did not differ from controls. Pregnancy in SPK is feasible, but patients should be informed of the risks for the fetus, the mother, and the grafts. Planning of pregnancy in SPK women is key to allow a personalized multidisciplinary monitoring, which represents the most straightforward approach to optimize outcomes.

Published
2017
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11. Predialysis blood pressure on survival in hemodialysis patients Reply

Author

Hannedouche, Thierry, Roth, Hubert, Krummel, Thierry, Drueke, Tilman, Fouque, Denis, Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Centre de recherche en épidémiologie et santé des populations (CESP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Néphrologie [Lyon], Hospices Civils de Lyon (HCL)-Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Université Joseph Fourier - Grenoble 1 (UJF)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), and Université Grenoble Alpes (UGA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Joseph Fourier - Grenoble 1 (UJF)

Subjects
[SDV]Life Sciences [q-bio], ComputingMilieux_MISCELLANEOUS
Abstract

International audience

Published
2017
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12. Epitope Spreading of Autoantibody Response to PLA2R Associates with Poor Prognosis in Membranous Nephropathy

Author

Vincent L.M. Esnault, Guillaume Dolla, Barbara Seitz-Polski, Thierry Krummel, Joel Polidori, Cécile Courivaud, Ghislaine Bernard, Eléonore Birgy-Barelli, Perrine Jullien, Gérard Lambeau, Christophe Girard, Sylvia Benzaken, Stéphane Burtey, Christophe Mariat, Kévin Zorzi, Christine Payré, Institut de pharmacologie moléculaire et cellulaire (IPMC), Centre National de la Recherche Scientifique (CNRS)-Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Université Côte d'Azur (UCA), Institut Laue-Langevin (ILL), ILL, Thales Electron Devices, Génétique et Ecophysiologie de la qualité des agrumes (GEQA), Institut National de la Recherche Agronomique (INRA), Régulations des réactions immunitaires et inflammatoires, Université Nice Sophia Antipolis (... - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-IFR50-Institut National de la Santé et de la Recherche Médicale (INSERM), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (RIGHT), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS [Bourgogne-Franche-Comté]), Service d'urologie, andrologie et transplantation rénale, Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon)-Hôpital Saint-Jacques, Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Vascular research center of Marseille (VRCM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), Service de néphrologie et tranplantation, CHU Saint-Etienne-Hôpital nord, Centre Hospitalier Universitaire de Nice (CHU Nice), DIGNAT-GEORGE, Françoise, Université Nice Sophia Antipolis (1965 - 2019) (UNS), COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-COMUE Université Côte d'Azur (2015-2019) (COMUE UCA)-Centre National de la Recherche Scientifique (CNRS)-Université Côte d'Azur (UCA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-Franche-Comté] (EFS BFC)-Université de Franche-Comté (UFC), Université Bourgogne Franche-Comté [COMUE] (UBFC)-Université Bourgogne Franche-Comté [COMUE] (UBFC), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre Hospitalier Universitaire de Saint-Etienne [CHU Saint-Etienne] (CHU ST-E), Université Côte d'Azur (UCA)-Université Côte d'Azur (UCA)-Centre National de la Recherche Scientifique (CNRS), Université Côte d'Azur (UCA)-Université Côte d'Azur (UCA)-IFR50-Institut National de la Santé et de la Recherche Médicale (INSERM), Interactions hôte-greffon-tumeur, ingénierie cellulaire et génique - UFC (UMR INSERM 1098) (HOTE GREFFON), Université de Franche-Comté (UFC)-Etablissement français du sang [Bourgogne-France-Comté] (EFS [Bourgogne-France-Comté])-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de Néphrologie et Urologie, Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon)-Hôpital Saint-Jacques, and Institut National de la Santé et de la Recherche Médicale (INSERM)-Etablissement français du sang [Bourgogne-France-Comté] (EFS [Bourgogne-France-Comté])-Université de Franche-Comté (UFC)

Subjects
Male, 0301 basic medicine, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Spontaneous remission, Glomerulonephritis, Membranous, Epitope, Epitopes, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Membranous nephropathy, Antigen, Up Front Matters, Humans, Medicine, Autoantibodies, Creatinine, business.industry, Receptors, Phospholipase A2, Autoantibody, Antibody titer, membranous nephropathy, General Medicine, Middle Aged, Prognosis, medicine.disease, 3. Good health, [SDV] Life Sciences [q-bio], Titer, 030104 developmental biology, chemistry, Nephrology, Immunology, Female, business, epidemiology and outcomes, chronic kidney disease
Abstract

International audience; The phospholipase A2 receptor (PLA2R1) is the major autoantigen in idiopathic membranous nephropathy. However, the value of anti-PLA2R1 antibody titers in predicting patient outcomes is unknown. Here, we screened serum samples from 50 patients positive for PLA2R1 for immunoreactivity against a series of PLA2R1 deletion mutants covering the extracellular domains. We identified reactive epitopes in the cysteine-rich (CysR), C-type lectin domain 1 (CTLD1), and C-type lectin domain 7 (CTLD7) domains and confirmed the reactivity with soluble forms of each domain. We then used ELISAs to stratify 69 patients positive for PLA2R1 by serum reactivity to one or more of these domains: CysR (n=23), CysRC1 (n=14), and CysRC1C7 (n=32). Median ELISA titers measured using the full-length PLA2R1 antigens were not statistically different between subgroups. Patients with anti-CysR-restricted activity were younger (P=0.008), had less nephrotic range proteinuria (P=0.02), and exhibited a higher rate of spontaneous remission (P=0.03) and lower rates of renal failure progression (P=0.002) and ESRD (P=0.01) during follow-up. Overall, 31 of 69 patients had poor renal prognosis (urinary protein/creatinine ratio >4 g/g or eGFR

Published
2016

13. Outcomes of acute kidney injury depend on initial clinical features: A national French cohort study

Author

Olivier Moranne, Alexandre Hertig, Cécile Couchoud, Thierry Hannedouche, Natacha Riffaut, Centre Hospitalier Universitaire de Nîmes (CHU Nîmes), Aide à la Décision pour une Médecine Personnalisé - Laboratoire de Biostatistique, Epidémiologie et Recherche Clinique - EA 2415 (AIDMP), Université Montpellier 1 (UM1)-Université de Montpellier (UM), Urgences néphrologiques et transplantation rénale [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, and Agence de la biomédecine [Saint-Denis la Plaine]

Subjects
Male, Time Factors, Databases, Factual, Epidemiology, medicine.medical_treatment, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, 030204 cardiovascular system & hematology, urologic and male genital diseases, 0302 clinical medicine, Cumulative incidence, Registries, 030212 general & internal medicine, Incidence (epidemiology), Incidence, Acute kidney injury, Acute Kidney Injury, female genital diseases and pregnancy complications, 3. Good health, Survival Rate, Hospitalization, Renal Replacement Therapy, Nephrology, Cohort, Female, France, 0305 other medical science, Cohort study, medicine.medical_specialty, Subgroup analysis, Lower risk, Databases, 03 medical and health sciences, Internal medicine, medicine, Humans, Rifle, Renal replacement therapy, Survival rate, Dialysis, Factual, Aged, Transplantation, 030505 public health, business.industry, Public Health, Environmental and Occupational Health, medicine.disease, business, Follow-Up Studies, Kidney disease
Abstract

Background Acute kidney injury (AKI) is a common condition that is associated with poor short- and long-term outcomes. The aim of this nationwide cohort study was to profile the long-term outcome of patients admitted for AKI in France. Methods Based on the comprehensive French hospital discharge database, all hospitalizations for an AKI episode were categorized in four groups according to the presence of at least one dialysis session (RRT) and according to the coding of AKI as the principal or associated diagnosis (PRINC_DIAG or ASS_DIAG). The cumulative incidences of death and ESRD in each group were analyzed with a subdistribution hazard (Fine and Gray) model to take into account the competing risks between those two outcomes. A subgroup analysis was done for patients who were alive and not on RRT at hospital discharge. The effect of the initial clinical feature on death or ESRD was analyzed with an adjusted cause-specific Cox proportional hazard regression censored at other outcomes. All the models were adjusted for age, gender and comorbidities. Results In this nationwide cohort of 989,974 patients (median age 77 years) hospitalized with AKI during the 2009–2016 period, 422,739 (43%) patients died (235,572 during the first hospitalization) and 40,015 (4%) patients reached ESRD (5962 during first hospitalization) up to 31 December 2016. Former cardiovascular disease and CKD were diagnosed in 40% and 16% of patients, respectively. Patients without RRT and discharged from hospital had a cumulative incidence of ESRD which ranged from 5.3% [5.2–5.4] in the ASS_DIAG group to 28.7% [27.9–29.5] in the RRT-PRINC_DIAG group at 60 months. The cumulative incidence of death ranged from 31.0% [30.2–32.2] in the RRT-ASS_DIAG group to 45.5% [45.3–45.7] in the ASS_DIAG group. Initial clinical features were associated with outcome independent of comorbidities and age. Compared to RRT-PRINC_DIAG, PRINC_DIAG (HR: 0.4, 95% CI: 0.4–0.4), ASS_DIAG patients (HR: 0.1, 95% CI: 0.1–0.2) and RRT-ASS_DIAG (HR: 0.4, 95% CI: 0.4–0.5) patients were at lower risk of reaching ESRD. Results were similar in each age group and whether or not patients had a previous diagnosis related to urinary tract or kidney disease. RRT-ASS_DIAG patients had a higher risk of death (HR: 1.9, 95% CI: 1.9–2.0) as compared to RRT-PRINC_DIAG, while patients with AKI as principal diagnosis (PRINC_DIAG) or associated diagnosis (ASS_DIAG) not requiring dialysis had a lower risk (HR: 0.7, 95% CI: 0.6–0.7 and HR: 0.9, 95% CI: 0.9–0.9, respectively). Conclusions The major strength of our observational study was the national coverage obtained from the comprehensive French hospital discharge database and a long follow-up over seven years. Our study strengthens the current recommendations for long-term follow-up of patients with AKI. The novelty of this study is to propose a clinical classification of AKI episodes that is easy to detect in administrative medical databases and that is strongly associated with immediate and long-term outcomes. This novel classification is based on the perceived primacy of AKI as a driver of the illness that brought the patient to the hospital and the need of RRT. Further studies are now warranted to compare the performance of our classification to predict long-term outcome to other classifications, including the KDIGO, AKIN or RIFLE.

Published
2018
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14. Multiphasic effects of blood pressure on survival in hemodialysis patients

Author

Thierry Hannedouche, Hubert Roth, Thierry Krummel, Gérard M. London, Guillaume Jean, Jean-Louis Bouchet, Tilman B. Drüeke, Denis Fouque, Lahcene Attroun, Raymond Azar, Pierre Bories, Agnès Caillette-Beaudouin, Bernard Canaud, Gabriel Choukroun, Vincent Esnault, Mohamed Hammadi, Patrick Henri, Philippe Honoré, Belkacem Issad, Dominique Joly, Eric Laruelle, Gildas Le Mao, Sylvain Marchais, Benoît Vendrely, Philippe Zaoui, Larbi Aazib, Abdelhamid Abbassi, Elias Abdullah, Habib Abou-Bekr, Carine Achard-Hottelart, Geneviève Achin, Salima Ahriz-Saksi, Mahen Albadawy, Catherine Albert, Samir Albitar, Farideh Alenabi, Mahmoud Allouache, Amar Amaouche, Brahim Amara, Mounia Ammor, Kim Seng Ang, Ubald Assogba, Lynda Azzouz, Chérif Badid, Juliette Baleynaud, Evelyne Bargas, Emmanuel Baron, François Basse, Jean-Marie Batho, Marc Bauwens, Dorothée Bazin, Abdelmajid Ben Aicha, Seddik Benarbia, Larbi Bencheikh, Jean-Christophe Bendini, Djeleddine Benyakoub, Dominique Bergua, Catherine Bessin, Luc Billaux, Stéphane Billion, Haïat Bittar, Jean-Paul Bocquet, Hervé Bonarek, Claude Bonniol, Jean-Sébastien Borde, Samir Boubenider, Rémi Boudet, Waël Boudi, Loreley Boudier, Djema Bouguern, M Boukelmoune, Fatha Zohra Boukhalfa, Henri Boulanger, Philippe Bouvier, Mouloud Bouzernidj, Mohamed Brahim Bounab, José Brasseur, Laura Braun, Marie Briet, Doan Bui-Quang, Sebastien Canet, Eric Canivet, Jorge Cardozo, Carlos Cardozo, Baher Chaghouri, Mokhtar Chawki, Charles Chazot, Philippe Choulet, Pierre Clavel, Jean-Philippe Coindre, Olivier Coldefy, M.A. Colomina, François Combarnous, Christian Dabot, Djamal Dahmane, Ahmed Dahmani, Daniel Daubresse, Jean-François De Fremont, Valérie De Precigout, Françoise Dehais, M. Dehina, Caroline Delclaux, Yashou Delmas, Coralia Denicola, Jean-Philippe Devaux, Raji Diab, Zineddine Diddaoui, null Didelot, Yves Dimitrov, Assia Djema, Patrick Donnadieu, Valérie Drouillat, Olivier Drouineau, Geneviève Dumont, Philippe Dupuy, Pierre-Yves Durand, Stéphane Edet, Hamid El Ali, Khuzama El Nasser, Christian Emond, Baya Fadel, Mohamed Fakir, Jean-Paul Faucon, André Faure, Assia Ferhat-Carre, Hafedh Fessi, Rocsana Fickl, Mahammed Fodil-Cherif, Jacques Fourcade, Philippe Fournier, Rabah Fraoui, Olivier Fritz, Elke Gaboriau, Alexandre Ganea, Roula Galland, Jacqes Gaultier, Eric Gauthier, Sylvie Geffroy Guiberteau, Sandrine Genestier, Patrick Giraud, Françoise Glowacki, Christophe Goupy, Pierre Grimal, Mounir Guergour, Jean Gugliotta, Marie-Paule Guillodo, Marie-Claude Guimont, Toufic Hachache, Sabria Hacini, Imad Haddad, Mohamed Hadj-Abdelkader, Pascale Halin, Patrick Hallonet, Nasser Hamdini, Didier Hamel, Françoise Heibel, Alain Hermelin, Alim Heyani, Philippe Hiernaux, Maxime Hoffmann, Valérie Hugot, Richard Ibos, Dominque Jacq, Jean-Paul Jaulin, Philippe Jousset, Benoît Jonon, Véronique Joyeux, Laurent Juillard, Amer Kamal, Mimi Kareche Chibout, Rateb Khayat, Franklin Khazine, Karim Khellaf, Arnaud Klisnick, Yannick Knefati, A. Kolko-Labadens, Amir Kolta, Niloufar Kossari, Olivier Kourilsky, Nicolas Krayem, Marc Kribs, François Kuentz, Kristian Kunz, Christian Lamotte, Jean-Marc Lanau, Isabelle Landru, Achour Laradi, Nicole Larroumet, Olivier Lavelle, Frank Le Roy, Alejandra Lenz, Denis Lerda, Fanny Leroy, Marc Leteif, Martial Levannier, Thierry Lobbedez, Hassan Lockmane, Nathalie Longlune, Christie Lorriaux Mortuaire, Alain Lyon, Ghassan Maakaroun, Mehadji Maaz, Eric Magnant, Ghandour Majdalani, Jean-Luc Mahe, Edward Maksour, Stéphane Martin, Catherine Martinat-Calvo, Valérie Masson, Delia May, Claire Maynard, Brice Mayor, Omar Mazouz, Hocine Mehama, Dominique Mercier, Gilles Messier, Robert Milongo, Nicole Monnier, Karine Moreau, Xavier Moreau-Gaudry, Bertrand Morel, Luc Moulonguet Doleris, Alexandre Mouneimne, Catherine Mourey-Epron, Françoise Moussion, Blanca Muniz, J. Mustel, Rachida Nebbad, Fazia Nemmar, Sylvie Neuville, Tien Nguyen-Quang, Patrice Nolen, Michel Normand, Emerson N’Sembani, Jacques Ollier, Jean-Paul Ortiz, Messaoud Ouziala, Bernard Painchart, Pedro Palacin, Josette Pengloan, Franck Perrin, Bruno Perrone, Philippe Petitjean, Dominique Petregne, Jean-Baptiste Philit, Vincent Planquois, Marc Pocheville, Jacky Potier, Jean-Michel Poux, Olivier Puyoo, Catherine Quere-Maurouard, Ahmed Rachi, Anderson Ratsimbazafy, Matthieu Reberolle, Henri Renaud, Bernard Richalet, Sarah Richter, Philippe Rieu, Michel Rince, Odile Rivault, Alain Robert, Jacques Rottembourg, Philippe Rousseau, Sophie Rubens Duval, Christa Roubicek, Piotr Seniuta, Pascal Seris, Irina Shahapuni, Reda Sharobeem, Milad Shenouda, Hélène Sichez Com, Danlèle Simonin, Nadia Soltani, Marc Souid, Hadia Sow, Jean-Christophe Szelag, Catherine Taddei, Zafer Takla, Dominque Teboulle, Jean-Claude Terrat, Patrick Thomas, Adam Tifoura, Jacques Toulon, Dominique Touzard, Pablo Urena Torres, Hans Van der Pijl, Thierry Vanel, Carlos Vela, Isabelle Vernier, Cathy Verove, François Pascal Wambergue, Bassem Wehbe, Maeva Wong-Fat, Fatima Yazbeck, Djamal Yousfi, Maan Youssef, Abdelaziz Ziane, Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Centre de Recherche en Nutrition Humaine Rhône-Alpes (CRNH-RA), Université Grenoble Alpes [2016-2019] (UGA [2016-2019])-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Recherche Agronomique (INRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-CHU Saint-Etienne-Hospices Civils de Lyon (HCL)-CHU Grenoble, Laboratory of Fundamental and Applied Bioenergetics = Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes [2016-2019] (UGA [2016-2019]), Centre Hospitalier Manhès, Centre de Rein Artificiel, Centre de traitement des maladies rénales, CTMR Saint-Augustin, Centre de recherche en épidémiologie et santé des populations (CESP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Université Paris-Sud - Paris 11 (UP11)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université de Versailles Saint-Quentin-en-Yvelines (UVSQ), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), Hospices Civils de Lyon (HCL), Centre Européen de Nutrition pour la Santé, Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Centre de Recherche en Nutrition Humaine Rhône-Alpes (CRNH-RH), Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National de la Recherche Agronomique (INRA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Jean Monnet [Saint-Étienne] (UJM)-CHU Saint-Etienne-Hospices Civils de Lyon (HCL)-CHU Grenoble-Université Joseph Fourier - Grenoble 1 (UJF), Laboratoire de bioénergétique fondamentale et appliquée (LBFA), Université Joseph Fourier - Grenoble 1 (UJF)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Grenoble Alpes (UGA), Université de Versailles Saint-Quentin-en-Yvelines (UVSQ)-Université Paris-Sud - Paris 11 (UP11)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Paul Brousse-Institut National de la Santé et de la Recherche Médicale (INSERM), and Université Grenoble Alpes (UGA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Joseph Fourier - Grenoble 1 (UJF)

Subjects
Male, medicine.medical_specialty, hypertension, Systole, medicine.medical_treatment, Population, 030232 urology & nephrology, Diastole, Blood Pressure, 030204 cardiovascular system & hematology, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Prehypertension, 03 medical and health sciences, 0302 clinical medicine, cardiovascular disease, Renal Dialysis, Risk Factors, Internal medicine, Medicine, Humans, Prospective Studies, Renal Insufficiency, Chronic, education, Aged, Aged, 80 and over, education.field_of_study, hemodialysis, business.industry, Hazard ratio, Blood Pressure Determination, Middle Aged, 3. Good health, Pulse pressure, Blood pressure, Nephrology, Cardiovascular Diseases, Aortic pressure, Cardiology, Female, Hemodialysis, France, business, Follow-Up Studies
Abstract

International audience; Dialysis patients exhibit an inverse, L- or U-shaped association between blood pressure and mortality risk, in contrast to the linear association in the general population. We prospectively studied 9333 hemodialysis patients in France, aiming to analyze associations between predialysis systolic, diastolic, and pulse pressure with all-cause mortality, cardiovascular mortality, and nonfatal cardiovascular endpoints for a median follow-up of 548 days. Blood pressure components were tested against outcomes in time-varying covariate linear and fractional polynomial Cox models. Changes throughout follow-up were analyzed with a joint model including both the time-varying covariate of sequential blood pressure and its slope over time. A U-shaped association of systolic blood pressure was found with all-cause mortality and of both systolic and diastolic blood pressure with cardiovascular mortality. There was an L-shaped association of diastolic blood pressure with all-cause mortality. The lowest hazard ratio of all-cause mortality was observed for a systolic blood pressure of 165 mm Hg, and of cardiovascular mortality for systolic/diastolic pressures of 157/90 mm Hg, substantially higher than currently recommended values for the general population. The 95% lower confidence interval was approximately 135/70 mm Hg. We found no significant correlation for either systolic, diastolic, or pulse pressure with myocardial infarction or nontraumatic amputations, but there were significant positive associations between systolic and pulse pressure with stroke (per 10-mm Hg increase: hazard ratios 1.15, 95% confidence interval 1.07 and 1.23; and 1.20, 1.11 and 1.31, respectively). Thus, whereas high pre-dialysis blood pressure is associated with stroke risk, low pre-dialysis blood pressure may be both harmful and a proxy for comorbid conditions leading to premature death.

Published
2015
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15. Mutation Update of the CLCN5 Gene Responsible for Dent Disease 1

Author

Patrick Niaudet, François Nobili, Guylhène Bourdat-Michel, Estelle Colin, Karin Dahan, Brigitte Gilbert-Dussardier, Claire Rigothier, Robert Novo, Dominique Martin-Coignard, Justine Bacchetta, Karim Bouchireb, Rémi Salomon, Thierry Hannedouche, Ariela Vergara-Jaque, Chantal Loirat, Dominique Chauveau, Pascal Houillier, Stéphane Burtey, Nicole Van Regemorter, Lamisse Mansour-Hendili, Sandrine Lemoine, Xavier Jeunemaitre, Muriel Holder-Espinasse, Michel Fischbach, Denis Morin, François-Guillaume Debray, S. Benoit, Valérie Leroy, Gilles Morin, Marie Alice Macher, Andreas Schleich, Mathilde Cailliez, Hassan Izzedine, Isabelle Roncelin, Gwenaëlle Roussey-Kesler, Cyrielle Treard, Claire Cartery, Hubert Nivet, Stéphane Lourdel, Véronique Baudouin, Pierre Cochat, Bertrand Knebelmann, Anne Blanchard, Wendy González, Estelle Desport, Tim Ulinski, Stella Dieguez, Marco Janner, Rosa Vargas-Poussou, Laurence Faivre, Alexandre Karras, Nelly Le Pottier, Francesco Emma, Philippe Vanhille, Renaud de la Faille, Anne Laure Sellier-Leclerc, J. Fourcade, Olivier Devuyst, Denis Fouque, Kenza Soulami, Aurélien Tiple, Etienne Bérard, Laurenne Dehoux, Marie Pierre Lavocat, Hélène François, Gérard Champion, Gerard Cardon, Georges Deschênes, Université Paris Descartes - Faculté de Médecine (UPD5 Médecine), Université Paris Descartes - Paris 5 (UPD5), Centre de Recherche des Cordeliers (CRC (UMR_S 872)), Université Paris Descartes - Paris 5 (UPD5)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Paris-Centre de Recherche Cardiovasculaire (PARCC - UMR-S U970), 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)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centro de Bioinformática y Simulación Molecular, Universidad de Talca, Univ Talca, Escuela Ingn Bioinfomat, Talca, Chile, Institut d'histoire du temps présent (IHTP), Centre National de la Recherche Scientifique (CNRS), Service de génétique [Angers], Université d'Angers (UA)-Centre Hospitalier Universitaire d'Angers (CHU Angers), PRES Université Nantes Angers Le Mans (UNAM)-PRES Université Nantes Angers Le Mans (UNAM), Centre Hospitalier Régional Universitaire [Lille] (CHRU Lille), Centre de référence des maladies rénales rares Néphrogones [CHU-HCL, Lyon], Hôpital Femme Mère Enfant [CHU - HCL] (HFME), Hospices Civils de Lyon (HCL)-Hospices Civils de Lyon (HCL), Service de neuropédiatrie et maladies métaboliques [CHU Robert-Debré], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré, Centre de Référence des Maladies Rénales Héréditaires de l'Enfant et de l'Adulte (MARHEA), CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie Pédiatrique, Centre Hospitalier Universitaire de Nice (CHU Nice)-Fondation LENVAL-Hopital pour Enfants, Vascular research center of Marseille (VRCM), Institut National de la Santé et de la Recherche Médicale (INSERM)-Aix Marseille Université (AMU), Hôpital de la Timone [CHU - APHM] (TIMONE), Département de Néphrologie et Transplantation d'organes, Hôpital de Rangueil, CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse], Service de Néphrologie et Immunopathologie Clinique, Centre Hospitalier Universitaire de Toulouse, PRES Université de Toulouse, Département de Pédiatrie, Hôpital Edouard Herriot [CHU - HCL], Service de Néphrologie-Transplantation-Dialyse, CHU Bordeaux [Bordeaux]-Groupe hospitalier Pellegrin, Centre Hospitalier Universitaire de Liège (CHU-Liège), Unité de Néphrologie Pédiatrique, Centre hospitalier universitaire de Poitiers (CHU Poitiers), Division of Nephrology, Université catholique de Louvain Medical school, Université Catholique de Louvain = Catholic University of Louvain (UCL), Department of Nephrology and Urology, IRCCS Ospedale Pediatrico Bambino Gesù [Roma]-IRCCS, Service de Néphrologie pédiatrique [Hôpital de Hautepierre, Strasbourg], CHU Strasbourg-Hôpital de Hautepierre [Strasbourg], Cardiovasculaire, métabolisme, diabétologie et nutrition (CarMeN), Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Institut National de la Recherche Agronomique (INRA), Human-machine spoken dialogue (CORDIAL), Institut de Recherche en Informatique et Systèmes Aléatoires (IRISA), Université de Rennes 1 (UR1), Université de Rennes (UNIV-RENNES)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes 1 (UR1), Institut National des Sciences Appliquées (INSA)-Université de Rennes (UNIV-RENNES)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-INRIA Rennes, Institut National de Recherche en Informatique et en Automatique (Inria)-École Nationale Supérieure des Sciences Appliquées et de Technologie (ENSSAT), Génétique Médicale, Centre hospitalier universitaire de Poitiers (CHU Poitiers)-Centre de Référence Anomalies du Développement Ouest, Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service de Physiologie [Georges-Pompidou], 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)-Université Paris Descartes - Paris 5 (UPD5), Service de Néphrologie [CHU Pitié-Salpêtrière], CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Service de néphrologie adultes [CHU Necker], Sciences pour l'environnement (SPE), Centre National de la Recherche Scientifique (CNRS)-Université Pascal Paoli (UPP), Service de néphrologie pédiatrique, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), Laboratoire de génétique médicale et cytogénétique [Le Mans], Centre Hospitalier Le Mans (CH Le Mans), Métal, Travaux et recherches archéologiques sur les cultures, les espaces et les sociétés (TRACES), École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS)-École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS), Service de néphrologie pédiatrique [CHU Necker], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Service de Néphrologie, Hôpital de Clocheville, Service de néphrologie pédiatrique [CHRU Besançon], Centre Hospitalier Régional Universitaire de Besançon (CHRU Besançon), Unité Néphrologie Pédiatrique [CHRU Lille], Génétique des Anomalies du Développement (GAD), Université de Bourgogne (UB)-IFR100 - Structure fédérative de recherche Santé-STIC, Department of Nephrology, Transplantation and Dialysis, Bordeaux, Hospices Civils de Lyon (HCL), Service de Néphrologie Dialyse Transplantation, CHU Ibn Rochd [Casablanca], CHU Clermont-Ferrand, Service de néphrologie et pédiatrie générale [CHU Trousseau], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-CHU Trousseau [APHP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de médecine interne et néphrologie, CH Valenciennes, Centre de Génétique de Bruxelles, Université libre de Bruxelles (ULB), Service de Génétique [CHU HEGP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), French Ministry of Health (Plan Maladies Rares), European Community FP7EUNEFRON 201590 and EURenOmics 2012‐305608, The Chilean PIA‐Conycit program (grant ACT‐1104), The Swiss National Science Foundation project (grant 310030_146490), RADIZ, Rare Diseases Initiative Zürich, a KFSP of the University of Zurich., Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre de Référence des Maladies Rénales Héréditaires de l'Enfant et de l'Adulte [CHU-Necker] (MARHEA), Aix Marseille Université (AMU)-Institut National de la Santé et de la Recherche Médicale (INSERM), Département de Néphrologie et Transplantation d'organes [CHU Toulouse], Pôle Urologie - Néphrologie - Dialyse - Transplantations - Brûlés - Chirurgie plastique - Explorations fonctionnelles et physiologiques [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Service Médecine interne et immunopathologie clinique [CHU Toulouse], Pôle IUCT [CHU Toulouse], Institut National de la Recherche Agronomique (INRA)-Université Claude Bernard Lyon 1 (UCBL), Université de Lyon-Université de Lyon-Institut National des Sciences Appliquées de Lyon (INSA Lyon), Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Hospices Civils de Lyon (HCL)-Institut National de la Santé et de la Recherche Médicale (INSERM), Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-Université de Rennes (UR)-Institut National des Sciences Appliquées - Rennes (INSA Rennes), Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de Recherche en Informatique et en Automatique (Inria)-Centre National de la Recherche Scientifique (CNRS)-INRIA Rennes, Université Pascal Paoli (UPP)-Centre National de la Recherche Scientifique (CNRS), École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université de Toulouse (UT)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS)-École des hautes études en sciences sociales (EHESS)-Université Toulouse - Jean Jaurès (UT2J), Université de Toulouse (UT)-Université de Toulouse (UT)-Ministère de la Culture et de la Communication (MCC)-Centre National de la Recherche Scientifique (CNRS), CHU Trousseau [APHP], Centre de Recherche des Cordeliers (CRC), Université Paris Diderot - Paris 7 (UPD7)-École pratique des hautes études (EPHE)-Université Pierre et Marie Curie - Paris 6 (UPMC)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Centre de Recherche des Cordeliers (CRC (UMR_S_1138 / U1138)), École pratique des hautes études (EPHE)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Max Planck Institute for Biophysical Chemistry (MPI-BPC), Max-Planck-Gesellschaft, Service de Génétique [AP-HP Hôpital Européen Georges Pompidou, Paris], Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré, Université Catholique de Louvain (UCL), IRCCS-IRCCS Ospedale Pediatrico Bambino Gesù [Roma], Université de Lyon-Institut National des Sciences Appliquées (INSA)-Institut National des Sciences Appliquées (INSA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hospices Civils de Lyon (HCL), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), Service de néphrologie [CHU Pitié-Salpêtrière], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Pitié-Salpêtrière [APHP], Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Necker - Enfants Malades [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpital Robert Debré-Université Paris Diderot - Paris 7 (UPD7), CHU Necker - Enfants Malades [AP-HP]-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP), Service de nephrologie pédiatrique, Centre Hospitalier Régional Universitaire [Besançon] (CHRU Besançon), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-CHU Trousseau [APHP]-Sorbonne Université (SU), Free University of Brussels (BELGIUM), Université Paris Descartes - Paris 5 (UPD5)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Institut National de la Santé et de la Recherche Médicale (INSERM), Physiopathologie Cardiovasculaire et Mitochondriale (MITOVASC), Université d'Angers (UA)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Centre National de la Recherche Scientifique (CNRS), Centre Hospitalier Lyon Sud [CHU - HCL] (CHLS), IFR100 - Structure fédérative de recherche Santé-STIC-Université de Bourgogne (UB), Centre de génétique - Centre de référence des maladies rares, anomalies du développement et syndromes malformatifs (CHU de Dijon), Centre Hospitalier Universitaire de Dijon - Hôpital François Mitterrand (CHU Dijon), Néphrologie, Centre Hospitalier Universitaire (CHU), Institut National des Sciences Appliquées (INSA)-Université de Lyon-Institut National des Sciences Appliquées (INSA)-Université de Lyon-Université Claude Bernard Lyon 1 (UCBL), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Pitié-Salpêtrière [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Université Pierre et Marie Curie - Paris 6 (UPMC)-École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM), École pratique des hautes études (EPHE), Université Paris sciences et lettres (PSL)-Université Paris sciences et lettres (PSL)-Université Paris Diderot - Paris 7 (UPD7)-Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Sorbonne Université (SU), Université Paris Descartes - Paris 5 (UPD5)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO), Institut de biologie de Lille - IBL (IBLI), Université de Lille, Sciences et Technologies-Institut Pasteur de Lille, Réseau International des Instituts Pasteur (RIIP)-Réseau International des Instituts Pasteur (RIIP)-Centre National de la Recherche Scientifique (CNRS)-Université de Lille, Droit et Santé, Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Université Paris Descartes - Paris 5 (UPD5), and Assistance publique - Hôpitaux de Paris (AP-HP) (APHP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)

Subjects
Male, chloride channel, renal failure, [SDV]Life Sciences [q-bio], 030232 urology & nephrology, Dent Disease, ClC-5, medicine.disease_cause, Cohort Studies, Mice, 0302 clinical medicine, Missense mutation, MESH: Animals, MESH: DNA Mutational Analysis, low molecular weight proteinuria, Genetics (clinical), Genetics, Mice, Knockout, 0303 health sciences, Mutation, biology, MESH: Genetic Predisposition to Disease, MESH: Anion Transport Proteins, Phenotype, 3. Good health, Pedigree, Chloride Channels/chemistry/*genetics/metabolism, Dent Disease/*genetics/metabolism, Nephrocalcinosis, MESH: Bartter Syndrome, MESH: Mutation, Knockout, [SDV.BC]Life Sciences [q-bio]/Cellular Biology, 03 medical and health sciences, Tubulopathy, Chloride Channels, medicine, Animals, Humans, Genetic Association Studies, 030304 developmental biology, MESH: Humans, CLCN5, MESH: Chloride Channels, Dent disease 1, medicine.disease, Bartter syndrome, biology.protein, OCRL, CLC family of chloride transporters and channels
Abstract

International audience; Dent disease is a rare X-linked tubulopathy characterized by low molecular weight proteinuria, hypercalciuria, nephrocalcinosis and/or nephrolithiasis, progressive renal failure, and variable manifestations of other proximal tubule dysfunctions. It often progresses over a few decades to chronic renal insufficiency, and therefore molecular characterization is important to allow appropriate genetic counseling. Two genetic subtypes have been described to date: Dent disease 1 is caused by mutations of the CLCN5 gene, coding for the chloride/proton exchanger ClC-5; and Dent disease 2 by mutations of the OCRL gene, coding for the inositol polyphosphate 5-phosphatase OCRL-1. Herein, we review previously reported mutations (n = 192) and their associated phenotype in 377 male patients with Dent disease 1 and describe phenotype and novel (n = 42) and recurrent mutations (n = 24) in a large cohort of 117 Dent disease 1 patients belonging to 90 families. The novel missense and in-frame mutations described were mapped onto a three-dimensional homology model of the ClC-5 protein. This analysis suggests that these mutations affect the dimerization process, helix stability, or transport. The phenotype of our cohort patients supports and extends the phenotype that has been reported in smaller studies.

Published
2015
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16. Physical activity level and home blood pressure measurement: Pilot study 'Acti-HTA'

Author

S. Bally, M. Lopez-Sublet, Olivier Steichen, Vincent Gremeaux, Philippe Sosner, P. Llaty, M. Brucker, S. Le Coz, Benoit Lequeux, A. Miranne, C. Dourmap, Club des jeunes hypertensiologues, Thierry Krummel, J. Ott, S. Baguet, M. Labrunee, université de Bourgogne, CAPS, Laboratoire 'Mobilité, Vieillissement, Exercice' (MOVE) (MOVE), Université de Poitiers, Service de néphrologie [Centre hospitalier de Haguenau], Centre hospitalier de Haguenau, Service de Médecine Interne = Hôpital de jour de médecine [CHU Tenon], CHU Tenon [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), Service de néphrologie - dialyse [Centre hospitalier Métropole Savoie - Chambéry], Centre Hospitalier Métropole Savoie [Chambéry], Service de néphrologie et hémodialyse [CHU de Strasbourg], CHU Strasbourg, Service de néphrologie [Centre hospitalier de Valence], Centre hospitalier de Valence, Service de cardiologie [CHU de Poitiers], Centre hospitalier universitaire de Poitiers (CHU Poitiers), Service de cardiologie et maladies vasculaires [Rennes] = Cardiac, Thoracic, and Vascular Surgery [Rennes], CHU Pontchaillou [Rennes], Service de néphrologie - dialyse [Centre hospitalier de Saint-Nazaire], Centre hospitalier de Saint-Nazaire, Groupe Hospitalier Mutualiste [Grenoble] (GHM), Université de Poitiers - Faculté de Médecine et de Pharmacie, Service Médecine physique et de réadaptation [CHU Toulouse], Pôle Neurosciences [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Centre Hospitalier Universitaire de Toulouse (CHU Toulouse)-Pôle Santé publique et médecine publique [CHU Toulouse], Centre Hospitalier Universitaire de Toulouse (CHU Toulouse), Cognition, Action, et Plasticité Sensorimotrice [Dijon - U1093] (CAPS), Université de Bourgogne (UB)-Institut National de la Santé et de la Recherche Médicale (INSERM), Service de médecine interne [Avicenne], Hôpital Avicenne [AP-HP], Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP), Laboratoire 'Mobilité, Vieillissement, Exercice' (MOVE) ( MOVE ), Service de Médecine Interne [CHU Tenon], Assistance publique - Hôpitaux de Paris (AP-HP)-CHU Tenon [APHP], CHU de Poitiers, Service de cardiologie et maladies vasculaires [CHU de Rennes], groupe hospitalier Mutualiste, Faculté de Médecine et de Pharmacie - Université de Poitiers, Service de médecine physique et réadaptation [Hôpital Rangueil - Toulouse], Hôpital de Rangueil, Cognition, Action, et Plasticité Sensorimotrice [Dijon - U1093] ( CAPS ), Université de Bourgogne ( UB ) -Institut National de la Santé et de la Recherche Médicale ( INSERM ), Hôpital Avicenne, Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-CHU Tenon [AP-HP], Sorbonne Université (SU)-Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Sorbonne Université (SU), and CHU Toulouse [Toulouse]-CHU Toulouse [Toulouse]

Subjects
medicine.medical_specialty, Evening, Physical activity, Dépense énergétique, Hypertension artérielle, Caloric expenditure, High blood pressure, [SDV.MHEP.CSC]Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Diabetes mellitus, Medicine, Motor activity, Morning, Automesure tensionnelle, Gynecology, Blood pressure management, business.industry, Home blood pressure measurement, Objective method, [ SDV.MHEP.CSC ] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, medicine.disease, [SDV.MHEP.CSC] Life Sciences [q-bio]/Human health and pathology/Cardiology and cardiovascular system, Activité physique, Blood pressure, Hypertension, Physical therapy, Cardiology and Cardiovascular Medicine, business
Abstract

While physical activity (PA) is recommended for high blood pressure management, the level of PA practice of hypertensive patients remains unclear. We aimed to assess the association between the level of both PA and blood pressure of individuals consulting in 9 hypertension specialist centres. Eighty-five hypertensive patients were included (59 ± 14 years, 61% men, 12% smokers, 29% with diabetes). Following their consultation, they performed home blood pressure measurement (HBPM) over 7 days (2 in the morning+2 in the evening), they wrote in a dedicated form their daily activities to estimate the additional caloric expenditure using Acti-MET device (built from International physical Activity Questionnaire [IPAQ]). Thus, patients completed a self-administered questionnaire "score of Dijon" (distinguishing active subjects with a score>20/30, from sedentary, Alors que la pratique régulière d’une activité physique (AP) est recommandée en cas d’hypertension artérielle (HTA), le niveau d’AP des sujets hypertendus reste mal connu. Notre objectif était d’étudier l’association entre le niveau d’AP et le caractère contrôlé ou non de l’HTA de sujets vus en consultation dans 9 centres spécialisés en HTA. Quatre-vingt-cinq sujets hypertendus ont été inclus (59 ± 14 ans, 61 % d’hommes, 12 % fumeurs, 29 % diabétiques). Au décours de leur consultation, une automesure tensionnelle (AMT) sur 7 jours (2 mesures le matin + 2 le soir) était réalisée, ainsi qu’un compte rendu de leurs activités quotidiennes permettant d’estimer la dépense calorique additionnelle à l’aide d’une réglette Acti-MET extrapolée du questionnaire international d’activités physiques (IPAQ). Les sujets complétaient ensuite un auto-questionnaire « score de Dijon » sur 30 points (distinguant les sujets actifs, score > 20/30, des sédentaires < 10/30). Les hypertendus contrôlés en AMT (< 135/85 mm Hg) (55 % des sujets), en comparaison aux non contrôlés, étaient plus âgés, avaient une tendance non significative à une dépense calorique déclarée plus élevée (4959 ± 5045 kcal/semaine vs 4048 ± 4199 kcal/semaine, p = 0,3755) et leur score de Dijon (19,44 ± 5,81 vs 18,00 ± 4,32, p = 0,2094) montrait une proportion de sujets « actifs » plus importante (48,9 % vs 34,2 %, p = 0,1773). En conclusion, nos résultats ont objectivé une « tendance » à une AP déclarée plus importante pour les sujets dont l’HTA est contrôlée. Ceci nous encourage à poursuivre par une étude qui inclurait davantage de sujets et dont l’AP serait évaluée par le port pendant 7 jours d’un capteur de type accéléromètre (méthode objective).

Published
2015
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17. The spectrum of kidney pathology in B-cell chronic lymphocytic leukemia / small lymphocytic lymphoma: a 25-year multicenter experience

Author

Anne-Laure Poitou-Verkinder, B. Legallicier, Bruno Moulin, Michel Godin, Stéphane Leprêtre, Arnaud François, Fanny Drieux, Dominique Guerrot, Service de Néphrologie [Rouen], Université de Rouen Normandie (UNIROUEN), Normandie Université (NU)-Normandie Université (NU)-CHU Rouen, Normandie Université (NU), Service d'Anatomie et Cytologie Pathologique [CHU Rouen], CHU Rouen, Normandie Université (NU)-Normandie Université (NU)-Université de Rouen Normandie (UNIROUEN), Centre de Lutte Contre le Cancer Henri Becquerel Normandie Rouen (CLCC Henri Becquerel), Service de Néphrologie et Transplantation, CHU Strasbourg, Service de néphrologie et hémodialyse [CHU de Strasbourg], Endothélium, valvulopathies et insuffisance cardiaque (EnVI), Normandie Université (NU)-Normandie Université (NU)-Institut National de la Santé et de la Recherche Médicale (INSERM), and VILLIER, Venceslas

Subjects
[SDV.MHEP.HEM] Life Sciences [q-bio]/Human health and pathology/Hematology, Male, Pathology, medicine.medical_specialty, Nephrotic Syndrome, Science, Chronic lymphocytic leukemia, 030232 urology & nephrology, [SDV.CAN]Life Sciences [q-bio]/Cancer, Kidney, [SDV.MHEP.UN]Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, 03 medical and health sciences, 0302 clinical medicine, Glomerulonephritis, [SDV.CAN] Life Sciences [q-bio]/Cancer, immune system diseases, Recurrence, hemic and lymphatic diseases, Antineoplastic Combined Chemotherapy Protocols, medicine, Neoplasm, Humans, Lymphocyte Count, Aged, Demography, Retrospective Studies, Multidisciplinary, business.industry, Amyloidosis, Kidney metabolism, [SDV.MHEP.HEM]Life Sciences [q-bio]/Human health and pathology/Hematology, Middle Aged, medicine.disease, [SDV.MHEP.UN] Life Sciences [q-bio]/Human health and pathology/Urology and Nephrology, Leukemia, Lymphocytic, Chronic, B-Cell, 3. Good health, Radiography, Leukemia, 030220 oncology & carcinogenesis, Medicine, Female, business, Nephrotic syndrome, Kidney disease, Follow-Up Studies, Research Article
Abstract

BackgroundChronic lymphocytic leukemia and small lymphocytic lymphoma are 2 different presentations of the most common B-cell neoplasm in western countries (CLL/SLL). In this disease, kidney involvement is usually silent, and is rarely reported in the literature. This study provides a clinicopathological analysis of all-cause kidney disease in CLL/SLL patients.MethodsFifteen CLL/SLL patients with kidney biopsy were identified retrospectively. Demographic, clinical, pathological and laboratory data were assessed at biopsy, and during follow-up.ResultsAt biopsy 11 patients presented impaired renal function, 7 patients nephrotic syndrome, 6 patients dysproteinemia, and 3 patients cryoglobulinemia. Kidney pathology revealed CLL/SLL-specific monoclonal infiltrate in 10 biopsies, glomerulopathy in 9 biopsies (5 membranoproliferative glomerulonephritis, 2 minimal change disease, 1 glomerulonephritis with organized microtubular monoclonal immunoglobulin deposits, 1 AHL amyloidosis). Five patients presented interstitial granulomas attributed to CLL/SLL. After treatment of the hematological disease, improvement of renal function was observed in 7/11 patients, and remission of nephrotic syndrome in 5/7 patients. During follow-up, aggravation of the kidney disease systematically occurred in the absence of favorable response to hematological treatment.ConclusionsA broad spectrum of kidney diseases is associated with CLL/SLL. In this setting, kidney biopsy can provide important information for diagnosis and therapeutic guidance.

Published
2014
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