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Transplant Direct 2022 Jul 17;8(7):e1323. Epub 2022 Jun 17.

French National Referral Centre for Cardiac Amyloidosis, Créteil, France.

Heart transplantation in cardiac amyloidosis (CA) patients is possible and generally considered for transplantation if other organs are not affected. In this study, we aimed to describe and assess outcome in patients following heart transplantations at our CA referral center.

Methods: We assessed all CA patients that had heart transplantations at our center between 2005 and 2018. Read More

Vaccines (Basel) 2022 May 31;10(6). Epub 2022 May 31.

Department of Infection Biology, London School of Hygiene and Tropical Medicine, London WC1E 7HT, UK.

Natural killer cells play an important role in the control of viral infections both by regulating acquired immune responses and as potent innate or antibody-mediated cytotoxic effector cells. NK cells have been implicated in control of Ebola virus infections and our previous studies in European trial participants have demonstrated durable activation, proliferation and antibody-dependent NK cell activation after heterologous two-dose Ebola vaccination with adenovirus type 26.ZEBOV followed by modified vaccinia Ankara-BN-Filo. Read More

Pathogens 2022 Jun 17;11(6). Epub 2022 Jun 17.

Centre de Recherche Saint-Antoine, CRSA, Inserm, AP-HP, Hôpital Saint-Antoine, Service de Parasitologie-Mycologie, Sorbonne Université, 75012 Paris, France.

Cryptococcosis is the third most common cause of invasive fungal infection in solid organ transplant recipients and cryptococcal meningitis (CM) its main clinical presentation. CM outcomes, as well as its clinical features and radiological characteristics, have not yet been considered on a large scale in the context of kidney transplantation (KT). We performed a nationwide retrospective study of adult patients diagnosed with cryptococcosis after KT between 2002 and 2020 across 30 clinical centers in France. Read More

Stem Cell Res 2022 Jun 6;63:102830. Epub 2022 Jun 6.

Harry Perkins Institute of Medical Research, QEII Medical Centre, Nedlands, WA, Australia; Centre for Medical Research, University of Western Australia, QEII Medical Centre, Nedlands, WA, Australia. Electronic address:

Nemaline myopathy (NM) is a congenital skeletal muscle disorder that typically results in muscle weakness and the presence of rod-like structures (nemaline bodies) in the sarcoplasma and/or in the nuclei of myofibres. Two induced pluripotent stem cell (iPSC) lines were generated from the lymphoblastoid cells of a 1-month-old male with severe NM caused by a homozygous recessive mutation in the ACTA1 gene (c.121C > T, p. Read More

Stem Cell Res 2022 Jun 6;63:102829. Epub 2022 Jun 6.

Harry Perkins Institute of Medical Research, QEII Medical Centre, Nedlands, WA, Australia; Centre for Medical Research, University of Western Australia, QEII Medical Centre, Nedlands, WA, Australia.

Variants in the ACTA1 gene are a common cause of nemaline myopathy (NM); a muscle disease that typically presents at birth or early childhood with hypotonia and muscle weakness. Here, we generated an induced pluripotent stem cell line (iPSC) from lymphoblastoid cells of a 3-month-old female patient with intermediate NM caused by a dominant ACTA1 variant (c.515C > A (p. Read More

Front Physiol 2022 2;13:908230. Epub 2022 Jun 2.

Univ Paris Est Creteil, INSERM, IMRB, Creteil, France.

Cystic fibrosis (CF) is a lethal and widespread autosomal recessive disorder affecting over 80,000 people worldwide. It is caused by mutations of the gene, which encodes an epithelial anion channel. CF is characterized by a great phenotypic variability which is currently not fully understood. Read More

Orphanet J Rare Dis 2022 06 18;17(1):236. Epub 2022 Jun 18.

Pfizer Inc, New York, NY, USA.

Background: Transthyretin amyloidosis (ATTR amyloidosis) is a rare, life-threatening disease caused by the accumulation of variant or wild-type (ATTRwt amyloidosis) transthyretin amyloid fibrils in the heart, peripheral nerves, and other tissues and organs.

Methods: Established in 2007, the Transthyretin Amyloidosis Outcomes Survey (THAOS) is the largest ongoing, global, longitudinal observational study of patients with ATTR amyloidosis, including both inherited and wild-type disease, and asymptomatic carriers of pathogenic TTR mutations. This descriptive analysis examines baseline characteristics of symptomatic patients and asymptomatic gene carriers enrolled in THAOS since its inception in 2007 (data cutoff: August 1, 2021). Read More

Eur Heart J Acute Cardiovasc Care 2022 Jun 17. Epub 2022 Jun 17.

European Georges Pompidou Hospital, Cardiology Department, Paris, France.

Aims: Guidelines recommend that in the absence of reversible cause for sudden cardiac arrest (SCA), implantable cardioverter defibrillator (ICD) should be performed to prevent further fatal event. We sought to describe the frequency and characteristics of patients discharged from the hospital without ICD after the SCA in the daily practice.

Methods And Results: From 2011 to 2018, all SCAs related to a cardiac cause admitted alive across the 48 hospitals of Great Paris Area were prospectively enrolled. Read More

Haematologica 2022 Jun 16. Epub 2022 Jun 16.

Department of Hematology, Centre Hospitalier Lyon Sud and Inserm U1111, Pierre Bénite.

Monomorphic epitheliotropic intestinal T-cell lymphoma (MEITL) is a rare aggressive T-cell lymphoma most reported in Asia. We performed a comprehensive clinical, pathological and genomic study of 71 European MEITL patients (36 males; 35 females, median age 67 years). The majority presented with gastrointestinal involvement and had emergency surgery, and 40% had stage IV disease. Read More

Blood 2022 Jun 10. Epub 2022 Jun 10.

Medstar Georgetown University Hospital, Washington, District of Columbia, United States.

Sutimlimab, a first-in-class humanized immunoglobulin G4 (IgG4) monoclonal antibody that selectively inhibits the classical complement pathway at C1s, rapidly halted hemolysis in the single-arm CARDINAL study in recently transfused patients with cold agglutinin disease (CAD). CADENZA was a 26-week randomized, placebo-controlled phase 3 study to assess safety and efficacy of sutimlimab in CAD patients without recent (within 6 months prior to enrollment) transfusion history. Forty-two patients with screening hemoglobin ≤10 g/dL, elevated bilirubin, and ≥1 CAD symptom received sutimlimab (n=22) or placebo (n=20) on Days 0 and 7, then biweekly. Read More

J Am Coll Surg 2022 Apr 8. Epub 2022 Apr 8.

Sorbonne Université, Paris, France; Department of Hepato-Pancreato-Biliary Surgery and Liver Transplantation, AP-HP Pitié-Salpêtrière Hospital, Paris, France.

Background: Liver surgeons need to know the expected outcomes of laparoscopic liver resection (LLR) in obese patients.

Objective: To assess morbidity, mortality and textbook outcomes (TO) after LLR in obese patients.

Methods: This is a French multicenter study of patients undergoing LLR between 1996 and 2018. Read More

Genet Med 2022 Jun 8. Epub 2022 Jun 8.

Departments of Neurology and Pediatrics, NYU Langone Health, New York, NY.

Purpose: Neurofibromatosis type 2 (NF2) and schwannomatosis (SWN) are genetically distinct tumor predisposition syndromes with overlapping phenotypes. We sought to update the diagnostic criteria for NF2 and SWN by incorporating recent advances in genetics, ophthalmology, neuropathology, and neuroimaging.

Methods: We used a multistep process, beginning with a Delphi method involving global disease experts and subsequently involving non-neurofibromatosis clinical experts, patients, and foundations/patient advocacy groups. Read More

Neuroimage 2022 Jun 2;258:119351. Epub 2022 Jun 2.

Univ Paris Est Creteil, EA4391, ENT, Créteil, France; Clinical Neurophysiology Unit, Henri Mondor Hospital, AP-HP, Créteil, France. Electronic address:

Diagnosis and management of chronic neuropathic pain are challenging, leading to current efforts to characterize 'objective' biomarkers of pain using imaging or neurophysiological techniques, such as electroencephalography (EEG). A systematic literature review was conducted in PubMed-Medline and Web-of-Science until October 2021 to identify EEG biomarkers of chronic neuropathic pain in humans. The risk of bias was assessed by the Newcastle-Ottawa-Scale. Read More

Lancet HIV 2022 06;9(6):e414-e427

The Kirby Institute, University of New South Wales Sydney, Sydney, NSW, Australia.

Background: The benefits of direct-acting antivirals towards the elimination of hepatitis C virus (HCV) in people living with HIV are decreased when individuals are reinfected with HCV following treatment. We aimed to systematically review the existing evidence of HCV reinfection risk after treatment among people living with HIV, including people who inject drugs and men who have sex with men (MSM), and to identify the factors that explain heterogeneity in the incidence of HCV reinfection.

Methods: For this systematic review and meta-analysis, we searched PubMed, Scopus, Web of Science, Cochrane, PsycINFO, and conference presentations from date of database inception to Jan 10, 2022, for clinical trials and cohort studies providing data that could be used to calculate the incidence of HCV reinfection following HCV treatment. Read More

Eur Urol 2022 May 30. Epub 2022 May 30.

Barts Cancer Institute, Experimental Cancer Medicine Centre, Queen Mary University of London, St Bartholomew's Hospital, London, UK.

Background: In JAVELIN Bladder 100, avelumab first-line maintenance plus best supportive care (BSC) significantly prolonged overall survival (OS; primary endpoint) versus BSC alone in patients with advanced urothelial carcinoma (aUC) without disease progression with first-line platinum-containing chemotherapy.

Objective: To evaluate patient-reported outcomes (PROs) with avelumab plus BSC versus BSC alone.

Design, Setting, And Participants: A randomized phase 3 trial (NCT02603432) was conducted in 700 patients with locally advanced or metastatic urothelial carcinoma that had not progressed with first-line gemcitabine plus cisplatin or carboplatin. Read More

Blood 2022 06 2. Epub 2022 Jun 2.

City of Hope National Medical Center, Duarte, California, United States.

Since the publication of the Revised European-American Classification of mature lymphoid neoplasms in 1994, subsequent updates of the classification of mature lymphoid neoplasms have been generated through iterative international efforts to achieve broad consensus among hematopathologists, geneticists, molecular scientists, and clinicians. Significant progress in the characterization of malignancies of the immune system in the last years, with many new insights provided by genomic studies, have led to the current proposal. We have followed the same process that was successfully used for the 3rd and 4th editions of the WHO classification of hematological neoplasms. Read More

Amyloid 2022 Jun 2:1-13. Epub 2022 Jun 2.

Amyloidosis Research and Treatment Center, Fondazione IRCCS Policlinico San Matteo and University of Pavia, Pavia, Italy.

The recent approval of three drugs for the treatment of amyloid transthyretin (ATTR) amyloidosis, both hereditary and wild-type, has opened a new era in the care of these diseases. ATTR amyloidosis is embedded in its pathophysiology, and the drugs target critical steps of the amyloid cascade. In addition to liver transplant, which removes the pathogenic variants, the introduction of gene silencers has allowed the suppression of both wild type and mutant transthyretin (TTR), thus extending the potential therapeutic range to wild-type cardiac amyloidosis. Read More

Ann Thorac Med 2022 Apr-Jun;17(2):132-135. Epub 2022 Apr 19.

Children's Department B, Bechir Hamza Children's Hospital of Tunis, University El Manar, Tunis, Tunisia.

A female-term neonate showed a severe respiratory distress syndrome (RDS) at hour 3 of life requiring her transfer to intensive care. She was intubated and started on assist-control mechanical ventilation associated with inhaled nitric oxide then high-frequency oscillation ventilation at day 12. Chest X-ray was gradually deteriorating. Read More

EBioMedicine 2022 Jun 26;80:104077. Epub 2022 May 26.

Gustave Roussy Cancer Center, Bioinformatics platform, Université Paris-Saclay, INSERM US23, CNRS UMS 3655, Villejuif, France.

Background: Severe COVID-19 is associated with a high circulating level of calprotectin, the S100A8/S100A9 alarmin heterodimer. Baseline calprotectin amount measured in peripheral blood at diagnosis correlates with disease severity. The optimal use of this biomarker along COVID-19 course remains to be delineated. Read More

J Histochem Cytochem 2022 Jun 31;70(6):415-426. Epub 2022 May 31.

Univ Lyon, UJM-Saint-Etienne, Laboratoire Interuniversitaire de Biologie de la Motricité, Saint-Etienne, France.

We previously demonstrated that 8 weeks of moderate-intensity endurance training is safe and improves muscle function and characteristics of sickle cell disease (SCD) patients. Here, we investigated skeletal muscle satellite cells (SCs) in SCD patients and their responses to a training program. Fifteen patients followed the training program while 18 control patients maintained a normal lifestyle. Read More

Infect Dis Now 2022 May 27. Epub 2022 May 27.

Service de maladies infectieuses et d'immunologie, université Paris-Est, CHU Henri-Mondor, Créteil, France.

Background: Optimized antibiotic plasma predictor efficacy is essential in systemic infections. The uricosuric agent probenecid inhibits tubular excretion of antibiotics and may be used as β-lactam pharmacokinetic enhancer (BLPKE), even though few data are currently available for this purpose.

Methods: We conducted a monocentric and retrospective observational study including all patients who received probenecid in combination with parenteral β-lactam antibiotics for systemic infections from Jan 1, 2014 to Dec 31, 2019. Read More

J Hepatol 2022 May 27. Epub 2022 May 27.

Université Paris Est Créteil, INSERM, IMRB, Créteil, France; INSERM, U955, Equipe 18 "Physiopathologie et Thérapeutiques des Hépatites Virales Chroniques et des cancers liés", Créteil, France; Assistance Publique-Hôpitaux de Paris, Hôpital Henri Mondor, Service d'Hépatologie, Créteil, France.

Background & Aims: Acute intermittent porphyria (AIP), caused by heterozygous germline mutations of the heme synthesis pathway enzyme HMBS (hydroxymethylbilane synthase), confers high risk of hepatocellular carcinoma (HCC) development. Yet, the role of HMBS in liver tumorigenesis remains unclear.

Methods: Here we explore HMBS alterations in a large series of 758 HCC including 4 AIP patients, we quantify the impact of HMBS mutations on heme biosynthesis pathway intermediates and we investigate the molecular and clinical features of HMBS-mutated tumors. Read More

Anaesth Crit Care Pain Med 2022 Jun 25;41(3):101060. Epub 2022 May 25.

Department of Anaesthesiology and Intensive Care (DAR B), University Hospital Center Saint Eloi Hospital, Montpellier, France; PhyMedExp, Montpellier University, INSERM, CNRS, CHU de Montpellier, Montpellier, France.

Objective: To provide guidelines for the management of the intensive care patient with severe acute pancreatitis.

Design: A consensus committee of 22 experts was convened. A formal conflict-of-interest (COI) policy was developed at the beginning of the process and enforced throughout. Read More

Breast Dis 2022 ;41(1):273-278

Division of Hospital Medicine, University of Tsukuba Hospital, 2-1-1 Amakubo, Tsukuba, Ibaraki, Japan.

Background: Mondor disease is superficial thrombophlebitis of the thoracoabdominal wall, mid-upper arm, and penis. Although it is usually a benign disease requiring no specific treatment, little is known about this disease owing to its rarity.

Objective: The aim of this retrospective observational study was to investigate the epidemiology and prognosis of Mondor disease. Read More

J Clin Med 2022 May 10;11(10). Epub 2022 May 10.

Service Douleur, Soins palliatifs et de Support et UIC22, Hôpital Laennec, CHU, 44093 Nantes, France.

Background: Deep brain stimulation (DBS) is an effective technique to treat patients with advanced Parkinson's disease. The surgical procedure of DBS implantation is generally performed under local anesthesia due to the need for intraoperative clinical testing. However, this procedure is long (5-7 h on average) and, therefore, the objective that the patient remains co-operative and tolerates the intervention well is a real challenge. Read More

Neurochirurgie 2022 May 24. Epub 2022 May 24.

Department of Neurosurgery, DMU Care, FHU adapt, Mondor Institute of Biomedical Research, Translational psychiatry, AP-HP, Inserm Unit 955 Team 15, University of Paris-Est Creteil (UPEC), Créteil, France. Electronic address:

Background: The global healthcare system has been overwhelmed by the Coronavirus disease-2019 (COVID-19). In order to mitigate the risk of spread of the virus, most elective surgical procedures have been cancelled especially during the lockdown periods. The purpose of this study was to assess the financial impact of the COVID outbreak due to the supposed reduced workload from our neurosurgery department in 2020. Read More

J Eur Acad Dermatol Venereol 2022 May 25. Epub 2022 May 25.

University of Massachusetts Medical School, Worcester, Massachusetts, USA.

Background: Vitiligo, an autoimmune disorder characterised by skin depigmentation, is associated with reduced quality of life (QoL). Vitiligo may be under-reported, in part because of misconceptions that it is a cosmetic disease.

Objectives: This survey sought to characterise vitiligo prevalence and explore the relationship between sociodemographic and clinical characteristics with QoL in a population-based, multinational study. Read More

Dig Liver Dis 2022 May 21. Epub 2022 May 21.

Service d'Hépato-gastroentérologie, CHU de Poitiers et Université de Poitiers, Poitiers 86021, France. Electronic address:

Immune checkpoint inhibitors (ICI) have high efficacy in metastatic colorectal cancer (mCRC) with microsatellite instability (MSI) but not in microsatellite stable (MSS) tumour due to the low tumour mutational burden. Selective internal radiation therapy (SIRT) could enhance neoantigen production thus triggering systemic anti-tumoral immune response (abscopal effect). In addition, Oxalipatin can induce immunogenic cell death and Bevacizumab can decrease the exhaustion of tumour infiltrating lymphocyte. Read More

Eur Heart J 2022 May 24. Epub 2022 May 24.

National Amyloidosis Centre, Division of Medicine, University College London, London, UK.

Aims: Transthyretin amyloid cardiomyopathy (ATTR-CM) is increasingly diagnosed at an early stage of the disease natural history, defined as National Amyloidosis Centre (NAC) ATTR Stage I. The natural history of early-stage ATTR-CM remains poorly characterized.

Methods And Results: A retrospective multi-centre observational study of 879 patients with ATTR-CM, either wild-type TTR genotype or carrying the p. Read More

J Eur Acad Dermatol Venereol 2022 May 24. Epub 2022 May 24.

Referral Center for Auto-Immune Bullous Diseases (MALIBUL), AP-HP, Paris, France.

Background: Erythema multiforme (EM) is a muco-cutaneous inflammatory disease mainly triggered by herpes simplex virus (HSV) recurrences. Association of EM and circulating auto-antibodies against plakins (anti-PLK-Abs [EM-PLK+]) has been reported. However, little is known about this subset of EM. Read More