3,395 results match your criteria Mondor Disease


Origin, HDV genotype and persistent viremia determine outcome and treatment response in patients with chronic hepatitis Delta.

J Hepatol 2020 Jul 4. Epub 2020 Jul 4.

AP-HP, Hôpital Avicenne, Laboratoire de microbiologie Clinique, Université Paris 13, Centre national de référence des hépatites B, C et Delta, Bobigny, Inserm U955, équipe 18, Université Paris-Est, Créteil.

Background/aims: Hepatitis delta virus (HDV) infection causes severe chronic liver disease in Hepatitis B virus (HBV) infected individuals. Factors associated with poor prognosis are largely unknown.

Methods: The French National Reference Centre for HDV performed a nationwide retrospective study on 1112 HDV-infected patients, collecting epidemiological, clinical, virological and histological data from the initial referral to the last recorded follow-up. Read More

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http://dx.doi.org/10.1016/j.jhep.2020.06.038DOI Listing

Cancer, immune suppression and Coronavirus Disease-19 (COVID-19): Need to manage drug safety (French Society for Oncology Pharmacy [SFPO] guidelines).

Cancer Treat Rev 2020 Jun 23;88:102063. Epub 2020 Jun 23.

Department of Pharmacy, Groupement Hospitalier Sud - Hospices Civils de Lyon, Lyon, France; EA 3738 CICLY, UCBL1 Université de Lyon, Lyon, France. Electronic address:

The Coronavirus disease (COVID-19) pandemic is disrupting our health environment. As expected, studies highlighted the great susceptibility of cancer patients to COVID-19 and more severe complications, leading oncologists to deeply rethink patient cancer care. This review is dedicated to the optimization of care pathways and therapeutics in cancer patients during the pandemic and aims to discuss successive issues. Read More

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http://dx.doi.org/10.1016/j.ctrv.2020.102063DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7308737PMC

Clinical recurrences of COVID-19 symptoms after recovery: viral relapse, reinfection or inflammatory rebound?

J Infect 2020 Jun 30. Epub 2020 Jun 30.

Service des Maladies infectieuses et tropicales, Assistance publique- hôpitaux de Paris, Centre hospitalier universitaire Bicêtre, 78 rue du général Leclerc, 94270 Le Kremlin-Bicêtre. Electronic address:

For the first 3 months of COVID-19 pandemic, COVID-19 was expected to be an immunizing non-relapsing disease. We report a national case series of 11 virologically-confirmed COVID-19 patients having experienced a second clinically- and virologically-confirmed acute COVID-19 episode. According to the clinical history, we discuss either re-infection or reactivation hypothesis. Read More

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http://dx.doi.org/10.1016/j.jinf.2020.06.073DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7326402PMC

Personalized surveillance for hepatocellular carcinoma in cirrhosis - using machine learning adapted to HCV status.

J Hepatol 2020 Jun 29. Epub 2020 Jun 29.

AP-HP, Hôpital Jean Verdier, Service d'Hépatologie, Bondy; Université Paris 13, Sorbonne Paris Cité, "Equipe labellisée Ligue Contre le Cancer", F-93206 Saint-Denis; Inserm, UMR-1162, "Génomique fonctionnelle des tumeur solides", F-75000, Paris, FRANCE. Electronic address:

Background And Aims: To develop algorithms based on machine learning predictive approaches to refine individualized predictions of hepatocellular carcinoma (HCC) risk according to HCV eradication in patients with cirrhosis included in the French ANRS CO12 CirVir cohort.

Methods: Patients with compensated biopsy-proven HCV-cirrhosis were included in 35 centers and followed a semi-annual HCC surveillance program. Three prognostic models for HCC occurrence were built, using (1) Fine-Gray regression as a benchmark, (2) single decision tree (DT), and (3) random survival forest for competing risks survival (RSF). Read More

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http://dx.doi.org/10.1016/j.jhep.2020.05.052DOI Listing

Robotic Versus Laparoscopic Partial Mesorectal Excision for Cancer of the High Rectum: A Single-Center Study with Propensity Score Matching Analysis.

World J Surg 2020 Jul 1. Epub 2020 Jul 1.

Unit of Digestive, Hepato-Pancreato-Biliary Surgery and Liver Transplantation, Henri Mondor Hospital, AP-HP, University of Paris Est, UPEC, 51 Avenue du Maréchal de Lattre de Tassigny, 94010, Créteil, France.

Background: The role of robotic surgery for partial mesorectal excision (PME) in patients with high rectal cancer (RC) remains unexplored. This study aimed to compare the operative and postoperative outcomes of robotic (R-PME) versus laparoscopic (L-PME) PME for high RC.

Methods: This was a single-center propensity score cohort study of consecutive patients diagnosed with RC in the high rectum (>10 to 15 cm from the anal verge) who underwent surgery between September 2012 and May 2019. Read More

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http://dx.doi.org/10.1007/s00268-020-05666-0DOI Listing

Optimized Protocol to Generate Spinal Motor Neuron Cells from Induced Pluripotent Stem Cells from Charcot Marie Tooth Patients.

Brain Sci 2020 Jun 27;10(7). Epub 2020 Jun 27.

CHU de Limoges, Service de Biochimie et Génétique Moléculaire, F-87000 Limoges, France.

Modelling rare neurogenetic diseases to develop new therapeutic strategies is highly challenging. The use of human-induced pluripotent stem cells (hiPSCs) is a powerful approach to obtain specialized cells from patients. For hereditary peripheral neuropathies, such as Charcot-Marie-Tooth disease (CMT) Type II, spinal motor neurons (MNs) are impaired but are very difficult to study. Read More

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http://dx.doi.org/10.3390/brainsci10070407DOI Listing

Striatal connectivity in premanifest Huntington disease is differentially affected by disease burden.

Eur J Neurol 2020 Jun 30. Epub 2020 Jun 30.

Département d'Études Cognitives, École normale supérieure, PSL University, 75005, Paris, France.

Background: Different amount of cumulative exposure to the toxic mutant form of the huntingtin protein might underlie distinctive pattern of striatal connectivity in premanifest Huntington's disease (pre-HD). Aim of this study is to investigate disease burden-dependent cortical- and subcortical-striatal loops in different pre-HD stages.

Methods: Sixteen pre-HD participants and 25 controls underwent magnetic resonance exam to investigate striatal structural and functional connectivity. Read More

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http://dx.doi.org/10.1111/ene.14423DOI Listing

Safety and efficacy of temsirolimus in combination with three different immuno-chemotherapy regimens in relapse and refractory mantle cell lymphoma, final results of the T phase IB trial of the LYSA.

Ann Hematol 2020 Aug 29;99(8):1771-1778. Epub 2020 Jun 29.

Department of Hematology, CHU de Nantes, University Hospital of Nantes, Nantes, France.

Mantle cell lymphoma has a dismal prognosis at relapse or in the refractory setting. Among therapies, mTor pathway targeting by temsirolimus has been the first strategy approved for relapse in Europe. While its efficacy in monotherapy has long been demonstrated, its use remains limited. Read More

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http://dx.doi.org/10.1007/s00277-020-04159-3DOI Listing

Phosphorus magnetic resonance spectroscopy and fatigue in multiple sclerosis.

J Neural Transm (Vienna) 2020 Jun 28. Epub 2020 Jun 28.

Service de Neuroradiologie, Hôpital Henri-Mondor, 51 avenue du Maréchal de Lattre de Tassigny, 94010, Créteil Cedex, France.

Fatigue stands among the most debilitating multiple sclerosis (MS) manifestations. Several pathophysiological mechanisms have been proposed at its origin. However, unmet needs still exist, and further investigations are required to better understand and manage this complaint. Read More

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http://dx.doi.org/10.1007/s00702-020-02221-yDOI Listing
June 2020
2.402 Impact Factor

Optimal priming of poxvirus vector (NYVAC)-based HIV vaccine regimens for T cell responses requires three DNA injections. Results of the randomized multicentre EV03/ANRS VAC20 Phase I/II Trial.

PLoS Pathog 2020 Jun 26;16(6):e1008522. Epub 2020 Jun 26.

Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland.

DNA vectors have been widely used as a priming of poxvirus vaccine in prime/boost regimens. Whether the number of DNA impacts qualitatively or quantitatively the immune response is not fully explored. With the aim to reinforce T-cell responses by optimizing the prime-boost regimen, the multicentric EV03/ANRS VAC20 phase I/II trial, randomized 147 HIV-negative volunteers to either 3xDNA plus 1xNYVAC (weeks 0, 4, 8 plus 24; n = 74) or to 2xDNA plus 2xNYVAC (weeks 0, 4 plus 20, 24; n = 73) groups. Read More

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http://dx.doi.org/10.1371/journal.ppat.1008522DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7319597PMC

Cutaneous manifestations in SARS-CoV-2 infection (COVID-19): a French experience and a systematic review of the literature.

J Eur Acad Dermatol Venereol 2020 Jun 26. Epub 2020 Jun 26.

Department of Dermatology, Hôpital Cochin, AP-HP, Groupe Hospitalier Paris Centre Cochin-Hôtel Dieu-Broca, 89 rue d'Assas, 75006, Paris, France.

Skin manifestations have been increasingly reported in the setting of COVID-19. However, their incidence and presentation are debated, and the role, direct or undirect, of SARS-CoV-2 in their pathogenesis has yet to be determined. In this work, we aimed to analyze our experience in a French referral center and to perform a systematic review of the literature to evaluate the incidence and prognosis of cutaneous lesions observed in COVID-19 patients. Read More

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http://dx.doi.org/10.1111/jdv.16775DOI Listing

Clinical Characteristics and Outcomes in Patients With Coronavirus Disease 2019 and Multiple Sclerosis.

JAMA Neurol 2020 Jun 26. Epub 2020 Jun 26.

Service de Neurologie, Clinical Investigation Center Institut National de la Santé et de la Recherche Médicale 1434, Centre Hospitalier Universitaire de Strasbourg, Strasbourg, France.

Importance: Risk factors associated with the severity of coronavirus disease 2019 (COVID-19) in patients with multiple sclerosis (MS) are unknown. Disease-modifying therapies (DMTs) may modify the risk of developing a severe COVID-19 infection, beside identified risk factors such as age and comorbidities.

Objective: To describe the clinical characteristics and outcomes in patients with MS and COVID-19 and identify factors associated with COVID-19 severity. Read More

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http://dx.doi.org/10.1001/jamaneurol.2020.2581DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7320356PMC

A review of the international early recommendations for departments organization and cancer management priorities during the global COVID-19 pandemic: applicability in low- and middle-income countries.

Eur J Cancer 2020 Jun 8;135:130-146. Epub 2020 Jun 8.

Department of Medical Oncology, Tenon Hospital, Institut Universitaire de Cancérologie AP-HP. Sorbonne University, Paris, France.

Coronavirus disease 2019 (COVID-19) is an infectious disease caused by a new virus that has never been identified in humans before. COVID-19 caused at the time of writing of this article, 2.5 million cases of infections in 193 countries with 165,000 deaths, including two-third in Europe. Read More

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http://dx.doi.org/10.1016/j.ejca.2020.05.015DOI Listing

High fetal hemoglobin level is associated with increased risk of cerebral vasculopathy in children with sickle cell disease in Mayotte.

BMC Pediatr 2020 Jun 20;20(1):302. Epub 2020 Jun 20.

Pediatric Medicine and Surgery, Cayenne General Hospital, Cayenne, French Guiana, France.

Background: Understanding the genetics underlying the heritable subphenotypes of sickle cell anemia, specific to each population, would be prognostically useful and could inform personalized therapeutics.The objective of this study was to describe the genetic modulators of sickle cell disease in a cohort of pediatric patients followed up in Mayotte.

Methods: This retrospective cohort study analyzed clinical and biological data, collected between January12007 and December 312017, in children younger than 18 years. Read More

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http://dx.doi.org/10.1186/s12887-020-02187-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7305627PMC

Mutltifaceted biological roles of adiponectin.

Ann Biol Clin (Paris) 2020 Jun;78(3):243-252

Sorbonne Université, Inserm UMR_S 938, Centre de recherche Saint-Antoine, Institut hospitalo-universitaire de cardio-métabolisme et nutrition (ICAN), Paris, France, Hôpital Tenon, AP-HP, Service de biochimie et hormonologie, UF Bio-marqueurs inflammatoires et métaboliques, Paris, France, Hôpitaux universitaires Henri Mondor, AP-HP, Département de biochimie-pharmacologie-biologie moléculaire-génétique médicale, Créteil, France.

Adiponectin is a major adipokine involved in energy homeostasis that exerts insulin-sensitizing properties. The level of adiponectin is reduced in situations of insulin resistance and is negatively associated with several pathophysiological situations including abdominal obesity, metabolic syndrome, steatosis and non-alcoholic steatohepatitis, type 2 diabetes, some cancers and cognitive diseases. These aspects are discussed in this review. Read More

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http://dx.doi.org/10.1684/abc.2020.1562DOI Listing

Mutltifaceted biological roles of leptin.

Ann Biol Clin (Paris) 2020 Jun;78(3):231-242

Hôpitaux universitaires Henri Mondor, AP-HP, Département de biochimie-pharmacologie-biologie moléculaire-génétique médicale, Créteil, France, Sorbonne Université, Inserm UMR_S 938, Centre de recherche Saint-Antoine, Institut hospitalo-universitaire de cardio-métabolisme et nutrition (ICAN), Paris, France, Hôpital Tenon, AP-HP, Service de biochimie et hormonologie, UF Bio-marqueurs inflammatoires et métaboliques, Paris, France.

The identification of leptin allowed the discovery of a new endocrine system. This major adipokine controlling energy homeostasis is also involved in the regulation of neuroendocrine function and fertility. Unfortunately, leptin is not able to treat common obesity, which associates hyperleptinemia and resistance to the hormone. Read More

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http://dx.doi.org/10.1684/abc.2020.1560DOI Listing

Efficacy of minimal residual disease driven immune-intervention after allogeneic hematopoietic stem cell transplantation for high-risk chronic lymphocytic leukemia: results of a prospective multicentric trial.

Haematologica 2020 Jun 11. Epub 2020 Jun 11.

Unité Adolescents et Jeunes Adultes, Hopital St Louis, Hopitaux de Paris, France.

Allogeneic hematopoietic stem cell transplantation (HSCT) remains a potentially curative and useful strategy in high-risk relapsing CLL. Minimal Residual Disease (MRD) assessment at 12 months post-HSCT is predictive of relapse. This phase 2 study aimed to achieve M12 MRD negativity (MRDneg) using MRD-driven immune-intervention (Md-PII) algorithm based on serial flow-cytometry blood MRD, involving cyclosporine tapering followed if failure by donor lymphocytes infusions. Read More

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http://dx.doi.org/10.3324/haematol.2019.239566DOI Listing
June 2020
5.868 Impact Factor

Allosteric control of hemoglobin S fiber formation by oxygen and its relation to the pathophysiology of sickle cell disease.

Proc Natl Acad Sci U S A 2020 Jun 11;117(26):15018-15027. Epub 2020 Jun 11.

Laboratory of Chemical Physics, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD 20892-0520;

The pathology of sickle cell disease is caused by polymerization of the abnormal hemoglobin S upon deoxygenation in the tissues to form fibers in red cells, causing them to deform and occlude the circulation. Drugs that allosterically shift the quaternary equilibrium from the polymerizing T quaternary structure to the nonpolymerizing R quaternary structure are now being developed. Here we update our understanding on the allosteric control of fiber formation at equilibrium by showing how the simplest extension of the classic quaternary two-state allosteric model of Monod, Wyman, and Changeux to include tertiary conformational changes provides a better quantitative description. Read More

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http://dx.doi.org/10.1073/pnas.1922004117DOI Listing

Evaluation of the impact of a nurse-led program of systematic screening of comorbidities in patients with axial spondyloarthritis: The results of the COMEDSPA prospective, controlled, one year randomized trial.

Semin Arthritis Rheum 2020 May 27;50(4):701-708. Epub 2020 May 27.

Rheumatology Department, Cochin Hospital, Assistance Publique Hôpitaux de Paris, Paris, France; Université de Paris, INSERM U-1153, CRESS, Paris, France.

Objective: To evaluate the impact of a nurse-led program of systematic screening for the management (detection/prevention) of comorbidities.

Methods: Prospective, randomized, controlled, open, 12-month trial (NCT02374749).

Participants: consecutive patients with axial Spondyloarthritis (axSpA) (according to the rheumatologist) THE PROGRAM: A nurse collected data on comorbidities during a specific outpatient visit. Read More

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http://dx.doi.org/10.1016/j.semarthrit.2020.05.012DOI Listing

Association Between Adult Acne and Dietary Behaviors: Findings From the NutriNet-Santé Prospective Cohort Study.

JAMA Dermatol 2020 Jun 10. Epub 2020 Jun 10.

Epidemiology in Dermatology and Evaluation of Therapeutics, EA7379, Paris-Est University, Paris Est Créteil University, Département Infectieux/Immuno/Vaccin, F-94000 Créteil, France.

Importance: Acne is a chronic, multifactorial inflammatory disease. The association between consumption of dairy products and fatty and sugary foods and occurrence and progression of acne remains unclear.

Objective: To assess the association between dietary behavior and current acne in adults. Read More

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http://dx.doi.org/10.1001/jamadermatol.2020.1602DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7287950PMC

Spondyloarthritis-Associated IgA Nephropathy.

Kidney Int Rep 2020 Jun 16;5(6):813-820. Epub 2020 Mar 16.

Department of Nephrology, Hôpital Européen Georges-Pompidou, Assistance Publique-Hopitaux de Paris, Paris, France.

Introduction: IgA nephropathy (IgAN) can be associated with spondyloarthritis (SpA). The course of SpA-associated IgAN remains largely unknown due to the absence of large cohorts.

Methods: This retrospective study included patients with biopsy-proven IgAN and definite SpA. Read More

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http://dx.doi.org/10.1016/j.ekir.2020.03.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7271945PMC

Altered keratinization and vitamin D metabolism may be key pathogenetic pathways in syndromic hidradenitis suppurativa: a novel whole exome sequencing approach.

J Dermatol Sci 2020 May 22. Epub 2020 May 22.

Dermatology Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Department of Physiopathology and Transplantation, Università degli Studi di Milano Via Pace 9, Milan, Italy; Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Milan, Italy. Electronic address:

Background: Diagnosis of pyoderma gangrenosum, acne and hidradenitis suppurativa (PASH) and pyogenic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PAPASH) patients, in spite of recently identified genetic variations, is just clinical, since most patients do not share the same mutations, and the mutations themselves are not informative of the biological pathways commonly disrupted in these patients.

Objective: To reveal genetic changes more closely related to PASH and PAPASH etiopathogenesis, identifying novel common pathways involved in these diseases.

Methods: Cohort study on PASH (n = 4) and PAPASH (n = 1) patients conducted using whole exome sequencing (WES) approach and a novel bioinformatic pipeline aimed at discovering potentially candidate genes selected from density mutations and involved in pathways relevant to the disease. Read More

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http://dx.doi.org/10.1016/j.jdermsci.2020.05.004DOI Listing

Transient antibody targeting of CD45RC inhibits the development of graft-versus-host disease.

Blood Adv 2020 Jun;4(11):2501-2515

Centre de Recherche en Transplantation et Immunologie, Institut de Transplantation Urologie-Néphrologie (ITUN), Unité Mixte de Recherche (UMR) 1064, INSERM/Université de Nantes, Nantes, France.

Allogeneic bone marrow transplantation (BMT) is a widely spread treatment of many hematological diseases, but its most important side effect is graft-versus-host disease (GVHD). Despite the development of new therapies, acute GVHD (aGVHD) occurs in 30% to 50% of allogeneic BMT and is characterized by the generation of effector T (Teff) cells with production of inflammatory cytokines. We previously demonstrated that a short anti-CD45RC monoclonal antibody (mAb) treatment in a heart allograft rat model transiently decreased CD45RChigh Teff cells and increased regulatory T cell (Treg) number and function allowing long-term donor-specific tolerance. Read More

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http://dx.doi.org/10.1182/bloodadvances.2020001688DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7284095PMC

Liver transplantation in patients with sickle cell disease: possible but challenging-a cohort study.

Transpl Int 2020 Jun 7. Epub 2020 Jun 7.

Centre Hepato-Biliaire, AP-HP Paul Brousse Hospital, Villejuif, France.

The liver is frequently affected in patients with sickle cell disease (SCD), but few reports have described liver transplantation (LT) in patients with SCD. We present a thorough analysis of the largest single-center series of LT in patients with SCD and the first systematic review. There were 21 patients with a median age of 37. Read More

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http://dx.doi.org/10.1111/tri.13669DOI Listing

Thoracic computerised tomography scans in one hundred eighteen orthopaedic patients during the COVID-19 pandemic: identification of chest lesions; added values; help in managing patients; burden on the computerised tomography scan department.

Int Orthop 2020 Jun 7. Epub 2020 Jun 7.

Orthopedic Department, Clinique Geoffroy Saint Hilaire, 75005, Paris, France.

Purpose: Based on the recent literature, chest computed tomography (CT) examination could aid for management of patients during COVID-19 pandemic. However, the role of chest CT in management of COVID-19 patients is not exactly the same for medical or surgical specialties. In orthopaedic or trauma emergency, abdomen, pelvis, cervical, dorsal, and lumbar spine CT are performed to investigate patients; the result is a thoracic CT scan incorporating usually the thorax; however, information about lung parenchyma can be obtained on this thorax CT, and manifestations of COVID-19 can be diagnosed. Read More

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http://dx.doi.org/10.1007/s00264-020-04651-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7275929PMC

Assessment of acute treatment of hypertension in ischemic stroke patients.

Clin Neurol Neurosurg 2020 May 22;195:105949. Epub 2020 May 22.

Life Sciences and Health Department, Paris-Est University, Paris, France; Henri Mondor Hospital, Paris, France. Electronic address:

Objectives: Stroke, the most common neurologic disorder and the major cause of disability and death after heart disease causing 11.8% of the total deaths worldwide, is defined as a rapidly developing clinical signs of focal disturbance of cerebral function lasting more than 24 h. This study aims to assess appropriateness of hypertension treatment in acute stroke and its adherence with the choice of medications tailored according to blood pressure readings. Read More

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http://dx.doi.org/10.1016/j.clineuro.2020.105949DOI Listing

Chronic obstructive pulmonary disease and the COVID-19 pandemic: Reciprocal challenges.

Respir Med Res 2020 May 11;78:100764. Epub 2020 May 11.

Service de Pneumologie, AP-HP Paris, Institut Cochin, inserm U1016, Université de Paris, Paris, France.

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http://dx.doi.org/10.1016/j.resmer.2020.100764DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7212957PMC

A Scope of the Social Brain in Multiple Sclerosis: Insights From Neuroimaging Studies.

Cogn Behav Neurol 2020 Jun;33(2):90-102

Excitabilité Nerveuse et Thérapeutique, Université Paris-Est-Créteil, Créteil, France.

Multiple sclerosis (MS) is the most common inflammatory neurologic disease in young adults. Its pathological mechanisms include demyelination, neurodegeneration, and synaptopathy. Cognitive deficits occur in up to 65% of individuals with MS and affect both nonsocial (eg, information processing speed, memory, and executive functions) and social (ie, emotion recognition, theory of mind, and empathy) cognitive domains. Read More

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http://dx.doi.org/10.1097/WNN.0000000000000227DOI Listing
June 2020
1.138 Impact Factor

Different phenotypes in dermatomyositis associated with anti-MDA5 antibody: Study of 121 cases.

Neurology 2020 Jul 2;95(1):e70-e78. Epub 2020 Jun 2.

From the Departments of Clinical Immunology and Internal Medicine (Y.A., S.T., G.L., B.H., N.C., K.M., O.B.), Immunobiology (M.M.), and Biostatistics, Public Health, and Medical Information (B.G.), Pitié-Salpêtrière Hospital, APHP, Sorbonne Universities; Myology Research Centre (Y.A., O.B.), INSERM, UMRS 974, Paris; Department of Pneumology (Y.U., H.N.), APHP, Avicenne Hospital, INSERM U1272, Université Sorbonne Paris Nord, Bobigny; Department of Internal Medicine (L.G., A. Marquet), Lyon Sud Hospital, Lyon Sud University, Paris; Reference Centre for Rare Autoimmune Diseases (A. Meyer) and Medical Intensive Care Unit, Hautepierre Hospital (V. Castelain), Hôpitaux Universitaires de Strasbourg, Strasbourg University; Division of Pneumology (R.B.), Bichat Hospital, AP-HP, Paris-Diderot University, Paris; Department of Pneumology (F.G., P.P.), Centre Hospitalier Universitaire, Angers University; Department of Internal Medicine Infectieuses (C.D.), CHU de Martinique, Fort de France; Departments of Internal Medicine and Clinical Immunology (S.A.) and Internal Medicine and Systemic Diseases (H.D.), François-Mitterrand University Hospital, University of Bourgogne-Franche-Comté, Dijon; Internal Medicine Department (B.T., A.B.), Cochin Hospital, AP-HP, René Descartes University, Paris; Department of Internal Medicine (C.G., N.L.), Henri Mondor Hospital, Paris Est Créteil University, Créteil, France; Department of Neurology (N.V.), Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Centre, Nijmegen, the Netherlands; Department of Internal Medicine (E.D.), Hôpital Bretonneau Tours, Tours University; Internal Medicine Department (A.S.), Hôpital Robert-Debré, Reims University; Departments of Rheumatology (T.M.) and Internal Medicine (L.D.S.M.), Bretagne Occidentale University, Centre Hospitalo-Universitaire de Brest; Department of Internal Medicine (S.H.), CHU de Besançon; Department of Internal Medicine (C.B.-D.), Centre Hospitalier Général de Niort; Department of Internal Medicine (N.T.), Nice University, CHU Nice; Department of Internal Medicine (P.C.), Institut Mutualiste Montsouris, Paris; Department of Internal Medicine (M.G.), Jean Verdier Hospital, Paris Seine-Saint-Denis University, Bondy; Department of Internal Medicine (A. Mekinian), Saint Antoine Hospital, APHP, Sorbonne Universities, Paris; Department of Pneumology CHU (J.C.M.), Poitiers University; Department of Pneumology (A.T.), Hôpital Saint-Louis, APHP, Sorbonne Paris Cité, University Paris-Diderot, Paris; Department of Respiratory Medicine (V. Cottin), National Reference Center for Rare Pulmonary Diseases, Hospices Civils de Lyon, Claude Bernard University Lyon 1, Lyon University; Department of Pneumology (D.I.-B.), European George Pompidou Hospital, APHP, Paris V University; Department of Pneumology (S.P.-H.), Sainte-Camille Hospital, Bry-sur-Marne; Department of Pneumology (C.B.), Foch Hospital, Saint Quentin en Yveline University, Suresnes; and Department of Immunology (N.F.), Lyon-Sud Hospital, Hospices Civils-de-Lyon, Pierre-Bénite, France.

Objectives: The predominance of extramuscular manifestations (e.g., skin rash, arthralgia, interstitial lung disease [ILD]) as well as the low frequency of muscle signs in anti-melanoma differentiation-associated gene 5 antibody-positive (anti-MDA5+) dermatomyositis caused us to question the term myositis-specific antibody for the anti-MDA5 antibody, as well as the homogeneity of the disease. Read More

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http://dx.doi.org/10.1212/WNL.0000000000009727DOI Listing

Case report of a familial triple: a syndrome and review of the literature.

Medicine (Baltimore) 2020 May;99(22):e20474

Pediatric Unit, Maternal and Child Department, Azienda USL of Reggio Emilia, Sant'Anna Hospital, Castelnovo ne' Monti, Italy.

Rationale: Triple-A syndrome, or Allgrove syndrome (AS), is a rare autosomal recessive disorder characterized by the alacrimia, achalasia, and adrenal insufficiency triad. Alacrimia usually starts at early infancy, while achalasia and adrenal insufficiency appear later during childhood or adulthood. Some patients may also present with the so-called Double-A syndrome (i. Read More

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http://dx.doi.org/10.1097/MD.0000000000020474DOI Listing

Managing patients with rheumatic diseases during the COVID-19 pandemic: The French Society of Rheumatology answers to most frequently asked questions up to May 2020.

Joint Bone Spine 2020 May 27. Epub 2020 May 27.

Department of Rheumatology, Hôpital Nord, CHU Saint-Étienne, Inserm U1059, Université de Lyon-Université Jean-Monnet, Saint-Étienne, France. Electronic address:

Background: Rheumatologists must contend with COVID-19 pandemic in the management of their patients and many questions have been raised on the use of both anti-inflammatory drugs and disease-modifying anti-rheumatic drugs (DMARD). The French Society of Rheumatology (SFR) selected the most critical ones to the daily practice of a rheumatologist and a group of 10 experts from SFR and Club Rheumatism and Inflammation (CRI) boards proposed responses based on the current knowledge of May 2020.

Methods: Following the availability of the first 18 questions and statements, 1400 individuals consulted the frequently asked questions between the March 31, 2020 and April 12, 2020. Read More

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http://dx.doi.org/10.1016/j.jbspin.2020.05.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7255274PMC

Whole genome copy number analysis in search of new prognostic biomarkers in first line treatment of mantle cell lymphoma. A study by the LYSA group.

Hematol Oncol 2020 May 29. Epub 2020 May 29.

CRCINA, INSERM, CNRS, Université d'Angers, Université de Nantes, Nantes, France.

Mantle cell lymphoma (MCL) is a lymphoproliferative disorder characterized by the t(11;14)(q13;q32) CCND1/IGH translocation. This lymphoma is however extremely heterogeneous in terms of molecular alterations. Moreover, the course of the disease can vary greatly between indolent forms with slow progression and aggressive conditions rapidly pejorative. Read More

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http://dx.doi.org/10.1002/hon.2750DOI Listing

Long before Huntington's disease: what matters most?

Lancet Neurol 2020 Jun 26;19(6):473-475. Epub 2020 May 26.

Assistance Publique-Hôpitaux de Paris, National Reference Center for Huntington's Disease, Neurology Department, Henri Mondor-Albert Chenevier Hospital, 94000 Créteil, France; Equipe Neuropsychologie Interventionnelle, Département d'Etudes Cognitives, École Normale Supérieure, PSL Research University, Institut Mondor de Recherche Biomédicale, Université Paris-Est, INSERM, Paris and Créteil, France; Faculté de Santé, Université Paris Est, Créteil, France. Electronic address:

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http://dx.doi.org/10.1016/S1474-4422(20)30156-3DOI Listing

Incorporating flow cytometry and next-generation sequencing in the diagnosis of CMML. Are we ready for prime?

Best Pract Res Clin Haematol 2020 Jun 7;33(2):101134. Epub 2019 Dec 7.

INSERM U1170, Gustave Roussy Cancer Center, Villejuif, France; Faculté de Médicine, Université Paris-Saclay, Le Kremlin-Bicêtre, France.

In the last version of the WHO classification of myeloid malignancies, flow cytometry and molecular investigation are listed as potentially useful, yet non-essential diagnostic tools in hard-to-recognize chronic myelomonocytic leukemias (CMML). Flow recognition of CMML was initially based on an increase in the fraction of peripheral blood, CD14,CD16 classical monocytes ≥94% of total monocytes. An associated inflammatory disease can preclude the detection of classical monocyte fraction increase by inducing accumulation of CD14,CD16 intermediate monocytes. Read More

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http://dx.doi.org/10.1016/j.beha.2019.101134DOI Listing

DISCOVERY: prevalence of transthyretin () mutations in a US-centric patient population suspected of having cardiac amyloidosis.

Amyloid 2020 May 26:1-8. Epub 2020 May 26.

Cardiovascular Research Lab for the Elderly at New York-Presbyterian/Columbia Allen Hospital, Columbia University Medical Center, New York, NY, USA.

Hereditary transthyretin-mediated amyloidosis (hATTR amyloidosis) is a multisystem disease that presents with polyneuropathy and/or cardiomyopathy. DISCOVERY, a multicenter screening study, enrolled patients with clinically suspected cardiac amyloidosis to determine the frequency of transthyretin () mutations and assess disease characteristics. Of 1007 patients, the majority were from the US (84%), Black/African American (56%), male (63%), and with a mean (standard deviation) age of 65 (13) years. Read More

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http://dx.doi.org/10.1080/13506129.2020.1764928DOI Listing

Parkinson's disease polygenic risk score is not associated with impulse control disorders: A longitudinal study.

Parkinsonism Relat Disord 2020 May 1;75:30-33. Epub 2020 May 1.

CHU, Toulouse, France.

Objective: To examine the relationship between a Parkinson's disease (PD) polygenic risk score (PRS) and impulse control disorders (ICDs) in PD.

Background: Genome wide association studies (GWAS) have brought forth a PRS associated with increased risk of PD and younger disease onset. ICDs are frequent adverse effects of dopaminergic drugs and are also more frequent in patients with younger disease onset. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2020.03.017DOI Listing

Meta-analysis results do not reflect the real safety of biologics in psoriasis.

Br J Dermatol 2020 May 23. Epub 2020 May 23.

Université Paris-Est Créteil, UPEC, EpiDermE EA 7379, Créteil, F-94010, France.

Background: In systematic reviews (SR) and meta-analyses (MA) of randomized controlled trials (RCTs) assessing treatments of psoriasis, the proportion of serious adverse events (SAEs) did not differ between treatments and placebo. Including cases of psoriasis worsening as SAEs may explain the lack of difference. This SR and MA aimed to explore this possibility. Read More

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http://dx.doi.org/10.1111/bjd.19244DOI Listing

Malaria, Collapsing Glomerulopathy, and Focal and Segmental Glomerulosclerosis.

Clin J Am Soc Nephrol 2020 May 22. Epub 2020 May 22.

Assistance Publique des Hôpitaux de Paris, Hôpitaux Universitaires Henri Mondor, Service de Néphrologie et Transplantation, Centre de Référence Maladie Rare Syndrome Néphrotique Idiopathique, Créteil, France

Background And Objectives: Malaria, a potentially life-threatening disease, is the most prevalent endemic infectious disease worldwide. In the modern era, the spectrum of glomerular involvement observed in patients after malarial infections remains poorly described.

Design, Setting, Participants, & Measurements: We therefore performed a retrospective multicenter study to assess the clinical, biologic, pathologic, and therapeutic characteristics of patients with glomerular disease demonstrated by kidney biopsy in France within 3 months of an acute malaria episode. Read More

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http://dx.doi.org/10.2215/CJN.00590120DOI Listing

[Impact of an intervention on the practice of venous thromboprophylaxis recommendations in a geriatric setting.]

Soins Gerontol 2020 May - Jun;25(143):39-43. Epub 2020 Mar 19.

Service de gériatrie B2, Hôpital Dupuytren, AP-HP, 1 rue Eugène-Delacroix, 91210 Draveil, France; Inserm U955, Université Paris Est Créteil, Institut Mondor de recherche biomédicale, équipe Cepia (Clinical Epidemiology and Ageing), 94000 Créteil, France. Electronic address:

The practice of prevention of venous thromboembolic disease in hospitalized elderly patients does not comply with published recommendations, in 30% of cases. The objective of this study was to evaluate the impact of recalling the recommendations on the venous thromboprophylaxis. Read More

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http://dx.doi.org/10.1016/j.sger.2020.03.014DOI Listing

Endurant Stent Graft Demonstrates Promising Outcomes in Challenging Abdominal Aortic Aneurysm Anatomy.

J Vasc Surg 2020 May 19. Epub 2020 May 19.

Division of Vascular and Endovascular Surgery, Erasmus University Medical Centre, Rotterdam, Netherlands.

Objective: We aimed to assess the five-year safety and effectiveness outcomes of patients enrolled in the Endurant Stent Graft Natural Selection Global Post Market Registry (ENGAGE) who were treated outside the approved indications for use (IFU) of the Endurant stent graft.

Methods: Our primary outcome measure was 12-month treatment success, defined as successful endograft delivery and deployment and the absence of type I or III endoleak, stent migration or limb occlusion, late conversion, and AAA diameter increase or rupture. Secondary outcome measures included 30-day all-cause mortality, major adverse events, secondary procedures, technical observations, aneurysm-related mortality, and all-cause mortality within 12 months. Read More

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http://dx.doi.org/10.1016/j.jvs.2020.04.508DOI Listing

Cornea verticillata and acroparesthesia efficiently discriminate clusters of severity in Fabry disease.

PLoS One 2020 22;15(5):e0233460. Epub 2020 May 22.

Internal Medicine Department, Reference Center for Lysosomal Storage Disorders, Groupe Hospitalier Diaconesses Croix Saint-Simon, Paris, France.

Backgroud: Fabry disease (OMIM #301 500), the most prevalent lysosomal storage disease, is caused by enzymatic defects in alpha-galactosidase A (GLA gene; Xq22.1). Fabry disease has historically been characterized by progressive renal failure, early stroke and hypertrophic cardiomyopathy, with a diminished life expectancy. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0233460PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7244174PMC

Phenotype and Outcomes of Pulmonary Hypertension Associated with Neurofibromatosis Type 1.

Am J Respir Crit Care Med 2020 May 21. Epub 2020 May 21.

CHU de Bicetre, Service de Pneumologie, Le Kremlin Bicetre, France.

Rationale: Pulmonary hypertension associated with neurofibromatosis type 1 (PH-NF1) is a rare and largely unknown complication of NF1.

Objectives: To describe characteristics and outcomes of PH-NF1.

Methods: We reported the clinical, functional, radiologic, histologic and hemodynamic characteristics, response to pulmonary arterial hypertension (PAH)-approved drugs and transplant-free survival of patients with PH-NF1 from the French PH registry. Read More

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http://dx.doi.org/10.1164/rccm.202001-0105OCDOI Listing

Analysis of Midterm Outcomes of Endovascular Aneurysm Repair in Octogenarians From the ENGAGE Registry.

J Endovasc Ther 2020 May 21:1526602820923827. Epub 2020 May 21.

Division of Vascular and Endovascular Surgery, Erasmus University Medical Center, Rotterdam, the Netherlands.

Purpose: To assess periprocedural results and secondary endovascular procedure outcomes over 5 years in patients aged ≥80 vs <80 years undergoing endovascular aneurysm repair (EVAR).

Materials And Methods: Data from the Endurant Stent Graft Natural Selection Global post-market registry (ENGAGE) were used for the analyses. A total of 1263 consecutive patients were enrolled in the prospective, observational, single-arm registry and divided into 2 groups according to age: ≥80 years (290, 22. Read More

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http://dx.doi.org/10.1177/1526602820923827DOI Listing

Cost-effectiveness analysis of pretreatment screening for NUDT15 defective alleles.

Pharmacogenet Genomics 2020 May 19. Epub 2020 May 19.

Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Service de Biochimie.

Background: Nucleotide triphosphate diphosphatase (NUDT15) genetic testing in addition to thiopurine methyl transferase (TPMT) is recommended to reduce the incidence of adverse severe myelotoxicity episodes induced by thiopurines.

Objective: We assessed the cost-effectiveness ratio of combined screening for TMPT and NUDT15 defective alleles by genotyping or next-generation sequencing (NGS) using TPMT genotyping as the reference. Because of the genetic differences in thiopurine toxicity, we tested the screening strategies on individuals of Caucasian and Asian descent. Read More

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http://dx.doi.org/10.1097/FPC.0000000000000410DOI Listing

Severe diffuse alveolar hemorrhage related to autoimmune disease: a multicenter study.

Crit Care 2020 05 18;24(1):231. Epub 2020 May 18.

Département Hospitalo-Universitaire Inflammation-Immunopathologie-Biotherapie (DHU i2B), UPMC Université Paris 06, UMR 7211, Sorbonne Universités, 75005, Paris, France.

Background: Diffuse alveolar hemorrhage (DAH) occurs during the course of autoimmune disease and may be life threatening. The objective was to assess characteristics and prognosis factors of DAH who required intensive care unit (ICU) admission in patients with autoimmune diseases.

Methods: French multicenter retrospective study including patients presenting DAH related to autoimmune diseases requiring ICU admission from 2000 to 2016. Read More

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http://dx.doi.org/10.1186/s13054-020-02936-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7236262PMC

The adiponectin to leptin ratio, a still unrecognized biomarker of insulin resistance and cardiometabolic risk.

Ann Biol Clin (Paris) 2020 06;78(3):265-268

Sorbonne Université, Inserm UMR_S 938, Centre de recherche Saint-Antoine, Institut hospitalo-universitaire de cardio-métabolisme et nutrition (ICAN), Paris, France, Hôpital Tenon, AP-HP, Service de biochimie et hormonologie, UF Bio-marqueurs inflammatoires et métaboliques, Paris, France, Hôpitaux universitaires Henri Mondor, AP-HP, Département de biochimie-pharmacologie-biologie moléculaire-génétique médicale, Créteil, France.

Leptin and adiponectin are two adipokines. Their circulating concentrations, high for leptin and low for adiponectin, are predictive of insulin resistance and of an unfavorable cardiometabolic evolution in patients with obesity, metabolic syndrome or type 2 diabetes. In addition, recently, the adiponectin/leptin ratio has been proposed as an index of adipose tissue dysfunction together with threshold values for cardiometabolic risk for this index. Read More

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http://dx.doi.org/10.1684/abc.2020.1559DOI Listing

Severe Acute Flaccid Myelitis Associated With Enterovirus in Children: Two Phenotypes for Two Evolution Profiles?

Front Neurol 2020 28;11:343. Epub 2020 Apr 28.

Department of Paediatric Neurology, Necker-Enfants malades Hospital, University of Paris, AP-HP, Paris, France.

Acute flaccid myelitis (AFM) is an acute paralysis syndrome defined by a specific inflammation of the anterior horn cells of the spinal cord. From 2014, worrying waves of life-threatening AFM consecutive to enterovirus infection (EV-D68 and EV-A71) have been reported. We describe 10 children displaying an AFM with an EV infection, the treatments performed and the 1 to 3-years follow-up. Read More

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http://dx.doi.org/10.3389/fneur.2020.00343DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7198806PMC