Evaluation of rheumatoid arthritis-associated interstitial lung disease in patients treated with JAK inhibitors: a MAJIK-SFR cohort study.

Objective: To examine the course of interstitial lung disease associated with rheumatoid arthritis (RA-ILD) in France on treatment with Janus kinase inhibitors (JAKis) using the MAJIK-SFR registry.

Methods: Prospective national multicentre observational study identifying patients with RA-ILD from the MAJIK-SFR registry. Pulmonary assessment data were collected at JAKi initiation and follow-up visits (6 months, 12 months and a median of 21 months postinclusion), including chest high-resolution CT (HRCT), pulmonary function tests (forced vital capacity (FVC) and diffusing capacity of the lungs for carbon monoxide (DLCO)), acute exacerbations of ILD, respiratory infections and lung cancers.

AMETIS Preplanned Ancillary Study: Impact of Agitation During Mechanical Thrombectomy Under Sedation.

Background: Mechanical thrombectomy is the treatment of choice for ischemic strokes of the anterior circulation with proximal occlusion. Mechanical thrombectomy can be performed under sedation, which can lead to episodes of periprocedural agitation. The aim of this study is to describe the prevalence of agitation and determine the consequences during and after mechanical thrombectomy.

Hypereosinophilia and Hypereosinophilic Syndromes: First Findings From a Nationwide Multicenter Cohort.

Background: Hypereosinophilic syndromes (HES) are a heterogenous group of eosinophilic disorders. To date, only retrospective studies of limited sample-size and/or follow-up duration are available.

Methods: The COHESion study is a national prospective multicenter multidisciplinary cohort recruiting both adults or children with the spectrum of eosinophilic disorders (including reactive HE/HES [HE/HES-R], idiopathic HES [HES-I], lymphocytic HES [HES-L], neoplastic HE/HES [HE/HES-N], HE of unknown significance [HE-US], as well as IgG4-related disease [IgG4RD] or ANCA-negative eosinophilic granulomatosis with polyangiitis [EGPA] overlaps).

Catheterization Techniques for Anomalous Aortic Origin of Coronary Arteries.

Anomalous aortic origin of a coronary artery (AAOCA) is a rare congenital anomaly with a large spectrum of anatomical variations. Selective engagement of an AAOCA can present challenges during cardiac catheterization. A comprehensive understanding of the characteristics of major AAOCA can effectively assist operators for selecting and maneuvering catheters.

Same-day discharge strategy in a heart rhythm management clinic: The patient-reported experience.

Background: Same-day discharge (SDD) has been adopted for interventional cardiology procedures, however, data on patient experience are scarce.

Aims: To investigate patient-reported experience after various SDD electrophysiology procedures.

Methods: Consecutive patients undergoing electrophysiology procedures, who fulfilled pre-defined eligibility criteria for SDD, completed the questionnaire before discharge.

CIROZ is dispensable in ancestral vertebrates but essential for left-right patterning in humans.

Four genes-DAND5, PKD1L1, MMP21, and CIROP-form a genetic module that has specifically evolved in vertebrate species that harbor motile cilia in their left-right organizer (LRO). We find here that CIROZ (previously known as C1orf127) is also specifically expressed in the LRO of mice, frogs, and fish, where it encodes a protein with a signal peptide followed by 3 zona pellucida N domains, consistent with extracellular localization. We report 16 individuals from 10 families with bi-allelic CIROZ inactivation variants, which cause heterotaxy with congenital heart defects.

Phenotype Spectrum of TRPM3-Associated Disorders.

Objective: Monoallelic variants in the transient receptor potential melastatin-related type 3 gene (TRPM3) have been associated with neurodevelopmental manifestations, but knowledge on the clinical manifestations and treatment options is limited. We characterized the clinical spectrum, highlighting particularly the epilepsy phenotype, and the effect of treatments.

Methods: We analyzed retrospectively the phenotypes and genotypes of 43 individuals with TRPM3 variants, acquired from GeneMatcher and collaborations (n = 21), and through a systematic literature search (n = 22).

[Epidemiology of burns in mayotte in 2022: an exhaustive study on a particular health area].

The last French department, created in 2011, Mayotte still lacks its integration into official health statistics, making it difficult to adapt care facilities to needs, as well as planning for prevention. We conducted a retrospective epidemiological study including all patients treated in the Mayotte burns unit between February 1, 2022 and January 31, 2023 (339 patients). We first studied burn patients on the island, i.

Effects of intravascular administration of mesenchymal stromal cells derived from Wharton's Jelly of the umbilical cord on systemic immunomodulation and neuroinflammation after traumatic brain injury (TRAUMACELL): study protocol for a multicentre randomised controlled trial.

Introduction: Traumatic brain injury (TBI) is one of the leading causes of death and disability worldwide. Treatments for TBI patients are limited and none has been shown to provide prolonged and long-term neuroprotective or neurorestorative effects. A growing body of evidence suggests a link between TBI-induced neuro-inflammation and neurodegenerative post-traumatic disorders.

Transjugular Intrahepatic Portosystemic Shunt (TIPS): A Bridge to Bariatric Surgery in Morbidly Obese Patients with Cirrhosis and Clinically Significant Portal Hypertension.

Background: In cirrhotic patients, portal hypertension increases mortality after surgery. We evaluated the impact of pre-operative transjugular intrahepatic portosystemic shunt (TIPS) on the outcomes of bariatric surgery in cirrhosis.

Methods: Multicentric retrospective cohort.

Impact of Nucleos(t)ide Analogs on Major Clinical Outcomes in Patients With Chronic Hepatitis B: A 10-Year French Nationwide Cohort Study.

Background: Conflicting results have been reported on the impact of tenofovir versus entecavir on liver-related outcomes.

Aims: To explore trends in clinical outcomes in chronic hepatitis B virus (HBV)-infected patients and compare the impact of tenofovir versus entecavir on the risk of hepatocellular carcinoma (HCC), liver transplantation (LT) and mortality.

Methods: We used the French National Health Insurance Databases (SNDS) to identify HBV-infected patients.

Cryptococcus neoformans/gattii and Histoplasma capsulatum var. capsulatum infections on tissue sections: diagnostic pitfalls and relevance of an integrated histomolecular diagnosis.

Cryptococcus neoformans/gattii and Histoplasma capsulatum var. capsulatum may present atypical histopathological features inducing diagnostic errors. We aimed to estimate the frequency of these atypical features on formalin-fixed tissue samples (FT) and to assess the relevance of an integrated histomolecular diagnosis using specific Histoplasma capsulatum PCR and panfungal PCR followed by Sanger sequencing and/or targeted-massive parallel sequencing (MPS).

Clinical Spectrum and Prognosis of Atypical Autosomal Dominant Polycystic Kidney Disease Caused by Monoallelic Pathogenic Variants of IFT140.

Rationale & Objective: Monoallelic predicted Loss-of-Function (pLoF) variants in IFT140 have recently been associated with an autosomal dominant polycystic kidney disease (ADPKD)-like phenotype. This study sought to enhance the characterization of this phenotype.

Study Design: Case series.

Safety of baricitinib in vaccinated patients with severe and critical COVID-19 sub study of the randomised Bari-SolidAct trial.

Background: The Bari-SolidAct randomized controlled trial compared baricitinib with placebo in patients with severe COVID-19. A post hoc analysis revealed a higher incidence of serious adverse events (SAEs) among SARS-CoV-2-vaccinated participants who had received baricitinib. This sub-study aimed to investigate whether vaccination influences the safety profile of baricitinib in patients with severe COVID-19.

Selected Health Characteristics are Associated with Urban Canadians' Acceptability of Policies Promoting Healthier Restaurant Food Environments.

Objective: Adoption of policies promoting healthier restaurant food environments (RFEs) is contingent on their acceptability. Limited evidence exists regarding individual characteristics associated with RFE policy acceptability, especially health-related characteristics. This study examined associations between health characteristics and RFE policy acceptability among urban Canadians.

Next Generation Phenotyping and Synthetic Faces in Coffin Siris Syndrome.

Diagnostic wandering and delayed management are major issues in rare diseases. Here, we report a new Next-Generation Phenotyping (NGP) model for diagnosing Coffin Siris syndrome (CSS) on clinical photographs among controls and distinguish the different genotypes. This retrospective and prospective study, conducted from 1998 to 2023, included frontal and lateral pictures of confirmed CSS.

New diseases linked to MEFV variants or pyrinopathies.

Autoinflammatory diseases (AIDs) are characterized by dysregulation of innate immunity, leading to systemic inflammation. Familial Mediterranean fever (FMF) is the most common AID, associated with variants in exon 10 of MEFV. This gene codes for pyrin, a key protein in the inflammasome of the same name, involved in the innate immune response.

COMPOSIT study: evaluating osimertinib combination with targeted therapies in EGFR-mutated non-small cell lung cancer.

Introduction: The emergence of diverse resistance mechanisms after osimertinib therapy, including on-target epidermal growth factor receptor (EGFR) mutations and off-target alterations, warrants investigation of novel therapeutics to overcome these challenges and improve patient outcomes.

Methods: COMPOSIT was a French, retrospective, multicenter, cohort study of the effectiveness and tolerability of osimertinib in combination with other targeted therapies in patients with advanced EGFR-mutant (EGFRm) non-small cell lung cancer (NSCLC) who harbored other oncogenic drivers as primary or acquired resistance mechanisms. Real-world progression-free survival (rwPFS), overall survival (OS), and objective response rate (ORR) were the primary endpoints.

Early safety after TAVR according to VARC-3 criteria: incidence, predictors, and clinical impact.

Introduction And Objectives: The Valve Academic Research Consortium (VARC)-3 definition of the early safety (ES) composite endpoint after transcatheter aortic valve replacement (TAVR) lacks clinical validation. The aim of this study was to determine the incidence, predictors, and clinical impact of ES after TAVR as defined by VARC-3 criteria.

Methods: We performed a multicenter study including 10 078 patients with severe aortic stenosis undergoing transarterial TAVR.

Distinct pathophysiological pathways support stratification of Sjögren's disease based on symptoms, clinical, and routine biological data.

Objective: Recently, three distinct phenotypes of Sjögren's disease (SjD) patients have been described, based on cluster analysis: B-cell active with low symptoms (BALS), high systemic activity (HSA), and low systemic activity with high symptoms (LSAHS). We aimed to assess whether these clusters were associated with distinct biomarkers and the prognostic value of IFN signature.

Methods: The ASSESS cohort is a 20-year prospective cohort of SjD patients.

Effect of oral nintedanib vs placebo on epistaxis in hereditary hemorrhagic telangiectasia: the EPICURE multicenter randomized double-blind trial.

Epistaxis greatly affects patients with hereditary hemorrhagic telangiectasia (HHT). Although few systemic treatment exist, nintedanib, is a good candidate thanks to its anti-angiogenic activity. Our main objective was to evaluate the efficacy of oral nintedanib on epistaxis duration in HHT patients with moderate to severe epistaxis.

Innovative use of the new pulsed-thulium: YAG laser for ureteroscopic lithotripsy: can the "kidney stone calculator" predict lithotripsy duration?

To assess the accuracy of Kidney Stone Calculator(KSC), a software designed for surgical planning, in predicting the duration of lithotripsy during flexible ureteroscopy(FURS) when using the novel pulsed-Thulium: YAG(p-Tm: YAG) laser. From February to August 2023, a single-center prospective study was conducted, including patients with kidney or ureteral stones through non-contrast computed tomography(NCCT), who underwent FURS with p-Tm: YAG laser lithotripsy. KSC used three-dimensional segmentation of the stones from NCCT images, along with an interactive user interface for laser settings, to estimate the stone volume(SV) and the lithotripsy duration(LD).

Optimal dosage regimens of cefiderocol administered by short, prolonged or continuous infusion: a PK/PD simulation study.

Background And Objectives: Cefiderocol approved dosages are based on a prolonged infusion (PI) of 3 h that may not be adequate in all settings The objective of this study was to identify alternative cefiderocol dosage regimens based on short infusion (SI) or continuous infusion (CI).

Methods: We performed 1000-patient pharmacokinetic/pharmacodynamic (PK/PD) simulations based on a reference population model. Drug penetration into the epithelial lining fluid (ELF) was considered for pneumonia.