[COVID-19 and mechanical circulatory support].

Extracorporeal membrane oxygenation (ECMO) is mainly used as a rescue therapy in COVID-19 patients with severe acute respiratory distress syndrome (ARDS). More rarely, COVID-19 can be complicated by hemodynamic failure due to fulminant myocarditis or massive pulmonary embolism necessitating the implantation of venous-arterial ECMO. The management of ECMO during the COVID-19 pandemic is challenging due to some specificities related to the disease characteristics, such as the management of anticoagulation in patients with a hypercoagulable state and an increased risk of venous thromboembolism.

A strobe multicenter descriptive study of 55 infectious aortitis.

Infectious aortitis (IA) is a rare and severe disease. The treatment classically associates open surgery with prolonged antibiotic therapy. This study aimed to describe clinical characteristics, medical and surgical supports in a large and current series of IA.

A juvenile ALS-like phenotype dramatically improved after high-dose riboflavin treatment.

Riboflavin transporter deficiency (RTD) was recently characterized as a cause of genetic recessive childhood-onset motor neuron disease (MND) with hearing loss, formerly described as Brown-Vialetto-Van-Lear syndrome. We describe a 18-year-old woman with probable RTD mimicking juvenile Amyotrophic Lateral Sclerosis (ALS) who presented with an inaugural respiratory failure and moderate distal four limbs weakness. Only one heterozygous SLC52A3 mutation was detected, but presence of a sub-clinical auditory neuropathy and dramatic improvement under high dose riboflavin argued for a RTD.

Clinical implications of CTNNA1 germline mutations in asymptomatic carriers.

In 2017, we implemented CTNNA1 germline analysis in probands suspected of having hereditary diffuse gastric cancer. Here, we report the results from a retrospective series of 41 cases, including the identification of a new family with a CTNNA1 mutation and the first prophylactic total gastrectomy in an asymptomatic carrier after a normal upper endoscopy. Diffuse gastric cancer foci with loss of catenin alpha-1 expression were seen in the resected tissue, suggesting that CTNNA1 and CDH1 germline mutations behave in a similar manner.

Unexpected good outcome in severe cerebral fat embolism syndrome.

In this case study, we report the longitudinal and multimodal follow-up of a catastrophic initial presentation of cerebral fat embolism syndrome. We show that despite the initial severity, the cognitive outcome was ultimately very good but with a highly nonlinear time-course and prolonged loss of consciousness (more than 2 months). Repeated clinical assessments and brain-imaging techniques (electroencephalography, event-related potential, -Fluoro-Deoxy-Glucose-PET and magnetic resonance imaging) allowed us to monitor and anticipate this dynamic, providing relevant information to guide decision making in front of withdrawal of life-sustaining therapy discussions.

T-cell prolymphocytic leukemia and tuberculosis: a puzzling association.

T-cell prolymphocytic leukemia can result in severe immune T-cell deficiency. Clinicians should be aware of this complication in this rare lymphoid malignancy, and opportunistic infections should be ruled out before the use of usual immunosuppressive procedures such as alemtuzumab and hematopoietic stem cell transplantation.

Body Mass Index Decline Is Related to Spinocerebellar Ataxia Disease Progression.

Background: Spinocerebellar ataxias (SCAs) are dominantly inherited, progressive ataxia disorders. Disease progression could be preceded by weight loss.

Objectives: We aimed to study the course of weight loss in patients who had the most common SCAs (SCA1, SCA2 SCA3, and SCA6).

Cost-effectiveness analysis on the use of fidaxomicin and vancomycin to treat Clostridium difficile infection in France.

Background: Fidaxomicin is a macrocyclic antibiotic with proven efficacy against Clostridium difficile infection (CDI) in adults. It was licensed in France in 2012, but, due to higher acquisition costs compared with existing treatments, healthcare providers require information on its cost/benefit profile.

Objective: To compare healthcare costs and health outcomes of fidaxomicin and vancomycin, as reference treatment for CDI.

Ophthalmic manifestations in IgG4-related disease: Clinical presentation and response to treatment in a French case-series.

IgG4-related disease (IgG4-RD) is characterized by variable tissue or organ involvements sharing common pathological findings. Orbital or orbital adnexa involvement of the disease has been reported in a few case series. The aim of our study was to characterize and analyze ophthalmic manifestations from a nationwide French case-series.

[Management of failures and complications in weight loss surgery].

The specific management of the surgical risks linked to obesity, as well as technological advances combined with the standardisation of techniques, have significantly reduced the morbidity and mortality associated with bariatric surgery over recent decades. However, as with all surgery, patients are exposed to medical and surgical failures and complications.

Late-onset neutropenia after treatment with rituximab for rheumatoid arthritis and other autoimmune diseases: data from the AutoImmunity and Rituximab registry.

Objectives: To evaluate the prevalence of late-onset neutropenia and its complications in patients treated with rituximab (RTX) for rheumatoid arthritis (RA) and other autoimmune diseases (AIDs) in a prospective registry.

Methods: The AutoImmunity and Rituximab registry is an independent 7-year prospective registry promoted by the French Society of Rheumatology. For each episode of neutropenia, data were validated by the clinician in charge of the patient.

The CUPIC algorithm: an accurate model for the prediction of sustained viral response under telaprevir or boceprevir triple therapy in cirrhotic patients.

Triple therapy using boceprevir or telaprevir remains the reference treatment for genotype 1 chronic hepatitis C in countries where new interferon-free regimens have not yet become available. Antiviral treatment is highly required in cirrhotic patients, but they represent a difficult-to-treat population. We aimed to develop a simple algorithm for the prediction of sustained viral response (SVR) in cirrhotic patients treated with triple therapy.

Plasma apolipoprotein H limits HCV replication and associates with response to NS3 protease inhibitors-based therapy.

Background & Aims: Chronic infection with HCV remains a public health problem with approximately 150 million people infected worldwide. HCV intersects with lipid metabolism for replication and entry; and plasma concentrations of apolipoproteins have been identified as predictors for response to therapy. Herein, we conducted a screen of plasma proteins, including all apolipoproteins, to identify correlates of response to pegylated-interferon/ribavirin (PR) and HCV non-structural protein 3 (NS3) inhibitors (i.

[Radiation therapy of sinonasal natural killer/T-cell lymphoma].

Natural killer (NK)/T-cell lymphoma are part of lymphoproliferative diseases, they are rare in Europe and the United States but relatively common in Asia and South America. Natural killer (NK)/T-cell lymphoma present clinically as destructive lesions of the upper aerodigestive tract with perforation of deep structures of the face, destruction of the palate or an invasion of orbits. Treatment modalities of these lymphomas are still discussed because of the lack of available studies and the rarity of this disease.

Genome-wide association study identifies variants associated with progression of liver fibrosis from HCV infection.

Background & Aims: Polymorphisms in IL28B were shown to affect clearance of hepatitis C virus (HCV) infection in genome-wide association (GWA) studies. Only a fraction of patients with chronic HCV infection develop liver fibrosis, a process that might also be affected by genetic factors. We performed a 2-stage GWA study of liver fibrosis progression related to HCV infection.

Dynamic relations between sedentary behavior, physical activity, and body composition after bariatric surgery.

Background: Physical activity has been shown to increase following obesity surgery; however, changes in sedentary behavior in this setting are not known. Our aim was to describe changes in both physical activity and sedentary behavior of obese patients after gastric bypass (GBP) and their relationships with changes in body composition.

Methods: Physical activity, time spent watching TV as typical sedentary behavior (self-report), and body composition (DXA) were assessed before and 6 and 12 months after GBP in 86 obese patients (67 women, 24-66 years old, BMI 41.

What did we learn on host's genetics by studying large cohorts of HIV-1-infected patients in the genome-wide association era?

Purpose Of Review: Genome-wide association studies (GWASs) performed in large cohorts of HIV-1-infected patients have shown that high throughput genomics can add valuable information in understanding disease progression. We report recent information gathered in the international field during the last few years and revisit the importance of well documented cohorts for genotype-phenotype association studies.

Recent Findings: The majority of GWASs in the HIV-1 field found that viral loads and disease progression are under the control of variants located in the major histocompatibility complex (MHC) in untreated patients.

IDH1 or IDH2 mutations predict longer survival and response to temozolomide in low-grade gliomas.

Objectives: Recent studies have shown that IDH1 and IDH2 mutations occur frequently in gliomas, including low-grade gliomas. However, their impact on the prognosis and chemosensitivity of low-grade gliomas remains unclear.

Methods: Search for IDH1 and IDH2 mutations, loss of heterozygosity on chromosomes 1p and 19q, MGMT promoter methylation, and p53 expression was performed in a series of 271 low-grade gliomas and correlated with overall survival.

[Guidelines for clinical practice for bariatric surgery].

Bariatric surgery is intended for subjects with BMI ≥ 40 kg/m(2) or ≥ 35 kg/m(2) with comorbidities. In any case, the indication can only be envisaged in patients who have had access to specialized medical care, and agree with a prolonged medical follow-up. After 60 years old, physiological age and comorbidities need to be highly considered.