Nasal High Flow to Modulate Dyspnea in Orally Intubated Patients.

Rationale: High flow therapy reduces dyspnea in acute respiratory failure but the underlying mechanisms are not fully elucidated.

Objectives: To compare dyspnea, airway occlusion pressure (P) and inspiratory work with and without nasal high flow (NHF, FiO 21%, temperature 31°C) in intubated patients under pressure support ventilation and during a spontaneous breathing trial (SBT).

Methods: Dyspnea (numerical rating scale, NRS and Mechanical Ventilation - Respiratory Distress Observational Scale, MV-RDOS), P, esophageal pressure, respiratory muscles EMG, arterial blood gas were compared in intubated patients on pressure support ventilation presenting a dyspnea-NRS > 3 during two sequences: 1) pressure support ventilation with NHF at 0 L/min followed by 30, 50 and 60 L/min (the last three were randomized) and 2) a SBT with NHF at 0 and 50 L/min (randomized).

Pain anticipation is a new behavioural sign of minimally conscious state.

Probing cognition and consciousness in the absence of functional communication remains an extremely challenging task. In this perspective, we imagined a basic clinical procedure to explore pain anticipation at bedside. In a series of 61 patients with a disorder of consciousness, we tested the existence of a nociceptive anticipation response by pairing a somaesthetic stimulation with a noxious stimulation.

Primary Meningeal Melanocytic Tumors of the Central Nervous System: A Review from the Ultra-Rare Brain Tumors Task Force of the European Network for Rare Cancers (EURACAN).

Background: Primary meningeal melanocytic tumors are ultra-rare entities with distinct histological and molecular features compared with other melanocytic or pigmented lesions, such as brain and leptomeningeal metastases from metastatic melanoma.

Methods: The European Network for Rare Cancers (EURACAN) Task Force on Ultra-Rare Brain Tumors (domain 10, subdomain 10) performed a literature review from January 1985 to December 2023 regarding the epidemiologic and clinical characteristics, histological and molecular features, radiological findings, and efficacy of local treatments (surgery and radiotherapy) and systemic treatments for these entities.

Results: Molecular analysis can detect specific mutations, including GNAQ, GNA11, SF3B1, EIF1AX, BAP1, that are typically found in circumscribed primary meningeal melanocytic tumors and not in other melanocytic lesions, whereas NRAS and BRAF mutations are typical for diffuse primary meningeal melanocytic tumors.

An innovative 3D hydroxyapatite patient-specific implant for maxillofacial bone reconstruction: A case series of 13 patients.

The study aimed to evaluate and discuss the use of an innovative PSI made of porous hydroxyapatite, with interconnected porosity promoting osteointegration, called MyBone Custom® implant (MBCI), for maxillofacial bone reconstruction. A multicentric cohort of 13 patients underwent maxillofacial bone reconstruction surgery using MBCIs for various applications, from genioplasty to orbital floor reconstruction, including zygomatic and mandibular bone reconstruction, both for segmental defects and bone augmentation. The mean follow-up period was 9 months (1-22 months).

Ventricular Tachycardia Ablation in Patients With Desmoplakin Cardiomyopathy.

Background: Desmoplakin (DSP) pathogenic variants are rare causes of arrhythmogenic cardiomyopathy and often involve the right and left ventricles. Ventricular tachycardia (VT) ablations may be required in these patients, but procedural characteristics have not been reported.

Objectives: In this study, the authors sought to report a multicenter experience of VT ablation in patients with DSP pathogenic variants.

Proposed diagnostic criteria for arrhythmogenic cardiomyopathy: European Task Force consensus report.

Arrhythmogenic cardiomyopathy (ACM) is a heart muscle disease characterized by prominent "non-ischemic" myocardial scarring predisposing to ventricular electrical instability. Diagnostic criteria for the original phenotype, arrhythmogenic right ventricular cardiomyopathy (ARVC), were first proposed in 1994 and revised in 2010 by an international Task Force (TF). A 2019 International Expert report appraised these previous criteria, finding good accuracy for diagnosis of ARVC but a lack of sensitivity for identification of the expanding phenotypic disease spectrum, which includes left-sided variants, i.

[Flow cytometry in chronic lymphproliferative disorders: report of a survey on practices on behalf of CytHem].

Flow cytometric immunophenotyping is nowadays an essential tool for diagnosis, classification and monitoring of chronic lymphoproliferative disorders (CLPD). Several recommendations on multicolor panels have been proposed in the literature but little is known about their application in routine laboratories. The CytHem group (Cytométrie Hématologique francophone), created in 2018, is organized in multiple thematic groups: among them one is dedicated to CLPD, "Cythem-SLP".

Inhaled nitric oxide in patients with acute respiratory distress syndrome caused by COVID-19: treatment modalities, clinical response, and outcomes.

Background: Inhaled nitric oxide (iNO) has been widely used in patients with COVID-19-related acute respiratory distress syndrome (C-ARDS), though its physiological effects and outcome are debated in this setting. The objective of this cohort study was to describe the modalities of iNO use, clinical response, and outcomes in a large cohort of C-ARDS patients.

Methods: Multicentre, retrospective cohort study conducted in France.

Norovirus and sapovirus infections after allogeneic hematopoietic stem cell transplantation: is it worth it to look for them?

Norovirus (NoV) and Sapovirus (SaV) are potential causative agents of diarrhea after allogeneic HSCT but little is known in this population. We performed a retrospective analysis by RT-PCR of calicivirus (NoV and SaV), Human adenovirus (HAdV), rotavirus (RV), Aichi virus (AiV), enterovirus (EV), human parechovirus (HPeV) and Human bocavirus (HBoV) in the diarrheal stools of patients after allogeneic HSCT. 49/162 patients had positive viral assays: HAdV (17%), EV (7%), NoV (4.

Diagnosis and Treatment of Peripheral and Cranial Nerve Tumors with Expert Recommendations: An EUropean Network for RAre CANcers (EURACAN) Initiative.

The 2021 WHO classification of the CNS Tumors identifies as "Peripheral nerve sheath tumors" (PNST) some entities with specific clinical and anatomical characteristics, histological and molecular markers, imaging findings, and aggressiveness. The Task Force has reviewed the evidence of diagnostic and therapeutic interventions, which is particularly low due to the rarity, and drawn recommendations accordingly. Tumor diagnosis is primarily based on hematoxylin and eosin-stained sections and immunohistochemistry.

Innovative treatments for meningiomas.

Multi-recurrent high-grade meningiomas remain an unmet medical need in neuro-oncology when iterative surgeries and radiation therapy sessions fail to control tumor growth. Nevertheless, the last 10years have been marked by multiple advances in the comprehension of meningioma tumorigenesis via the discovery of new driver mutations, the identification of activated intracellular signaling pathways, and DNA methylation analyses, providing multiple potential therapeutic targets. Today, Anti-VEGF and mTOR inhibitors are the most used and probably the most active drugs in aggressive meningiomas.

Primary central nervous system lymphoma: advances in its pathogenesis, molecular markers and targeted therapies.

Purpose Of Review: Primary central nervous system lymphoma (PCNSL) is a rare subtype of diffuse large B-cell lymphoma (DLBCL) located in the CNS with a less favorable prognosis. Recent information addressing the disease molecular heterogeneity is paving the way for tailored treatment strategies. This article reviews current work on the pathogenesis of the disease, potential biomarkers, and treatments.

Sjögren syndrome and RFC1-CANVAS sensory ganglionopathy: co-occurrence or misdiagnosis?

Background And Aims: RFC1-CANVAS and primary Sjögren syndrome (pSS) are among the most frequent causes of sensory ganglionopathy (SG) and can present simultaneously in a given patient, sharing confounding signs and symptoms. We describe the clinical characteristics of patients with SG due to CANVAS who were suspected of having or had received a previous diagnosis of pSS.

Methods: Patients with SG and a genetically confirmed RFC1-CANVAS followed in our centre were ascertained and their personal history of pSS was collected.

Description and Outcome of Severe Hypoglycemic Encephalopathy in the Intensive Care Unit.

Background: Disorders of consciousness due to severe hypoglycemia are rare but challenging to treat. The aim of this retrospective cohort study was to describe our multimodal neurological assessment of patients with hypoglycemic encephalopathy hospitalized in the intensive care unit and their neurological outcomes.

Methods: Consecutive patients with disorders of consciousness related to hypoglycemia admitted for neuroprognostication from 2010 to 2020 were included.

Safety analysis of glecaprevir/pibrentasvir in patients with markers of advanced liver disease in clinical and real-world cohorts.

Chronic hepatitis C virus (HCV) infection has the greatest health impact in patients with advanced liver disease. The direct-acting antiviral (DAA) regimen glecaprevir/pibrentasvir (G/P) is approved for treatment of HCV-infected patients without cirrhosis and with compensated cirrhosis. However, events of liver decompensation/failure have been reported in patients treated with protease-inhibitor-containing DAA regimens, often in patients with advanced liver disease.

Growth of home respiratory equipment from 2006 to 2019 and cost control by health policies.

Background: Home respiratory equipment (HRE) designed for the management of chronic respiratory failure includes oxygen therapy (O), noninvasive ventilation (NIV) and mechanical insufflation-exsufflation (MI-E). The growth of the number of patients treated by HRE, the prevalence and the associated costs in France have not been determined.

Methods: The French open access national health insurance aggregated data was used to estimate the evolution of theses parameters from 2006 to 2019.

Evaluation of Rituximab for Induction and Maintenance Therapy in Patients 75 Years and Older With Antineutrophil Cytoplasmic Antibody-Associated Vasculitis.

Importance: Older patients are underrepresented in studies of rituximab for the treatment of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Little is known about outcomes and adverse events associated with the use of rituximab therapy among patients 75 years and older with ANCA-associated vasculitis.

Objective: To examine outcomes and adverse events associated with the use of rituximab therapy in patients 75 years and older with ANCA-associated vasculitis, specifically granulomatosis with polyangiitis (GPA) or microscopic polyangiitis (MPA).

Cryptococcal Meningitis in Kidney Transplant Recipients: A Two-Decade Cohort Study in 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.