Recurrences and rechallenges of suspected drugs in epidermal necrolysis patients.

Background: Epidermal necrolysis (EN) is a rare and severe condition, characterized by a diffuse skin and mucosal detachment and mainly induced by drugs. Literature is scarce regarding the rate of recurrences and culprit drug re-exposure.

Objectives: To assess the rate of EN recurrences as well as high notoriety drugs re-exposures in patients with EN.

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.

Barriers to influenza vaccination during pregnancy in France: A national population-based study.

Background: Despite French national recommendations since 2012 that all pregnant women be vaccinated against influenza, in 2021 this vaccine coverage is low - around 30 % - in France.

Objectives: To identify barriers to influenza vaccination during pregnancy by assessing how often women were offered this vaccination and how often they accepted it.

Study Design: We used data from the French national perinatal survey (ENP), which covered all births during one week in March 2021 (N = 12,614).

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).

The METACER national cohort study of brain metastases in gastrointestinal cancers prospectively establishes prognostic factors.

Purpose: Availability data are scarce and primarily retrospective in patients with brain metastasis (BM) from gastrointestinal (GI) cancers. The objective of this cohort was to determine prognostic factors for survival outcomes in patients with BM from GI cancers.

Methods: METACER is a national multicentric prospective cohort study which included patients with BM diagnosis during a histologically proven digestive cancer follow-up between 2010 and 2014.

A systematic review and meta-analysis of radiation exposure in spinal surgeries: Comparing C-Arm, CT navigation, and O-Arm techniques.

Introduction: Advanced imaging techniques, such as C-arm fluoroscopy, O-arm, and CT navigation, are integral to achieving precision in orthopedic surgeries. However, these technologies also expose patients, surgeons, and operating room staff to varying levels of radiation. This systematic review and meta-analysis evaluate the radiation exposure (RE) associated with these imaging modalities and their impact on surgical outcomes.

Epidemiology and Characteristics of Meniscal Tears in Patients With Combined ACL and Medial Collateral Ligament Injuries Versus Isolated ACL Tears: A Case-Control Study From the Francophone Arthroscopic Society.

Background: The co-occurrence of anterior cruciate ligament (ACL) rupture with medial collateral ligament (MCL) rupture is a compound injury that can be associated with meniscal tears.

Purpose: To report the characteristics of meniscal tears in knees with isolated ACL versus combined ACL and MCL injuries, analyzing their frequency, distribution by site, and lesion type.

Study Design: Cross-sectional study; Level of evidence, 3.

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.

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).

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.

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.

Erratum: Real-life efficacy of immunotherapy for Sézary syndrome: a multicenter observational cohort study.

[This corrects the article DOI: 10.1016/j.eclinm.

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).

Phenotypic traits and family history in patients with 22q11.2 deletion syndrome and generalized epilepsy: A multicenter case-control study.

Objective: This study was undertaken to characterize the clinical and genetic features of patients with 22q11.2 deletion syndrome (22q11.2DS) and generalized epilepsy compared with 22q11.

Extracorporeal membrane oxygenation in trauma patient in France: a retrospective nationwide registry.

Background: Indications for Veno-venous (VV) or veno-arterial (VA) extracorporeal membrane oxygenation (ECMO) after trauma rely on poor evidence. The main aims were to describe the population of trauma patients requiring either VV or VA ECMO and report their clinical management and outcomes.

Methods: An observational multicentre retrospective study was conducted in 17 Level 1 trauma centres in France between January 2010 and December 2021.