ESR Essentials: imaging of common paediatric pulmonary diseases-practice recommendations by the European Society of Paediatric Radiology.

Chest imaging in children presents unique challenges due to varying requirements across age groups. For chest radiographs, achieving optimal images often involves careful positioning and immobilisation techniques. Antero-posterior projections are easier to obtain in younger children, while lateral decubitus radiographs are sometimes used when expiratory images are difficult to obtain and for free air exclusion.

MemScreen: A smartphone application for detection of mild cognitive impairment: A validation study: Smartphone App for MCI Detection.

Background And Objectives: Primary care is often the first point of contact for patients with cognitive complaints, making initial cognitive screening an essential step to avoid delays in diagnosing Alzheimer's disease (AD) at an early stage. We developed MemScreen, a self-administered smartphone application that assesses overall cognition and verbal memory, and evaluated its ability to detect mild cognitive impairment (MCI) in both general and clinical populations.

Methods: We conducted two validation cohort studies: (1) UK-based Whitehall II cohort study (13th wave, 2018-2022) involving a general population (MCI defined by poor performance on a global cognitive score), and (2) five French memory clinics involving patients without dementia (amnestic MCI defined by the Free and Cued Selective Reminding Test).

Coronary Events After Transcatheter Aortic Valve Replacement: Insights From the France TAVI Registry.

Background: The prevalence of coronary artery disease in patients undergoing transcatheter aortic valve replacement (TAVR) is high. Treatment of a coronary events (CE) after TAVR can be technically challenging.

Objectives: The authors sought to assess the incidence and prognostic impact of CE after TAVR.

Modifiable risk factors for culture positivity after primary Latarjet procedure. Should we change any practice?

Background: The prevalence of positive cultures in primarily operated shoulders represents a growing concern. Previous studies have documented high rates of culture positivity in primary prosthetic shoulder surgery, yet the Latarjet procedure has not been specifically explored in this context. The aim of this study was to identify the rate of culture positivity in patients treated with a primary Latarjet procedure, and to identify risk factors for culture positivity.

Safety and efficacy of tamoxifen in non-ambulant patients with Duchenne muscular dystrophy: a multicentre, randomised, double-blind, placebo-controlled, phase 3 trial (TAMDMD Group B).

Most patients with Duchenne muscular dystrophy (DMD) are non-ambulant. Preserving proximal motor function is crucial, rarely studied. Tamoxifen, a selective oestrogen receptor modulator, reduced signs of muscular pathology in a DMD mouse model.

Medical management pathways for Cushing's disease in pituitary tumors centers of excellence (PTCOEs).

Purpose: A recent update of consensus guidelines for the management of Cushing's disease (CD) included indications for medical therapy. However, there is limited evidence regarding their implementation in clinical practice. This study aimed to evaluate current medical therapy approaches by expert pituitary centers through an audit conducted to validate the criteria of Pituitary Tumors Centers of Excellence (PTCOEs) and provide an initial standard of medical care for CD.

[Total nasal reconstruction by folded forearm radial flap: Technical note].

Total nasal reconstruction represents a complex challenge, requiring restoration of all three anatomical planes while preserving respiratory function. This procedure has significant therapeutic, aesthetic, social and professional implications for patients. We share our academic experience of reconstruction using a folded microanastomosed radial forearm flap (FRFF) combined with a paramedian forehead flap, using the technique of F.

Prioritizing questions and topics for the development of guidelines and consensus-based recommendations supported by ILAE: A scoping review and proposal of prioritization criteria.

Clinical practice guidelines (CPGs) and consensus-based recommendations (CBRs) require considerable effort, collaboration, and time-all within the constraints of finite resources. Professional societies, such as the International League Against Epilepsy (ILAE), must prioritize what topics and questions to address. Implementing evidence-based care remains a crucial challenge in clinical practice.

Characterization of patients with clonal mast cells in the bone marrow with clinical significance not otherwise specified.

Background: Systemic mastocytosis (SM) diagnosis requires the presence of 3 minor criteria or 1 major and 1 minor criterion according to the WHO 2016 classification. The aim of this study was to characterize patients with 1 or 2 minor SM criteria including mutation and/or aberrant expression of CD2 and/or CD25 on bone marrow (BM) mast cells (MCs), but without MC activation syndrome (MCAS) criteria.

Methods: We included eligible patients from two countries diagnosed between 2011 and 2021.

Pediatric eosinophilic fasciitis: similarities and differences with adult forms.

The purpose of this study was to identify pediatric eosinophilic fasciitis, which is an extremely rare condition, in order to describe their clinical, paraclinical, and therapeutic characteristics. We made a call for observations via societies for pediatric rheumatology in France and surrounding countries and collected clinical and paraclinical data of the cases fulfilling the diagnostic criteria. Eight patients under 18 years of age with confirmed eosinophilic fasciitis followed between April 2004 and July 2022 in France, Germany, Italy, and Spain were included.

Chromatin environment-dependent effects of DOT1L on gene expression in male germ cells.

The H3K79 methyltransferase DOT1L is essential for multiple aspects of mammalian development where it has been shown to regulate gene expression. Here, by producing and integrating epigenomic and spike-in RNA-seq data, we decipher the molecular role of DOT1L during mouse spermatogenesis and show that it has opposite effects on gene expression depending on chromatin environment. On one hand, DOT1L represses autosomal genes that are devoid of H3K79me2 at their bodies and located in H3K27me3-rich/H3K27ac-poor environments.

Uncorrected ametropia in children with limited access to ocular health care.

Introduction: The primary objective of this study is to describe the refractive needs of vulnerable children according to their social security status. The secondary objective of this study is to describe the types of ametropia in this population of children with limited access to ocular health care.

Methods: Children with limited access to ocular health care were recruited.

Evaluation of professional practices in the use of mexiletine for the management of childhood myotonia in French pediatric neuromuscular centers (MEXI-PEDI survey).

Background: Myotonia is the main feature of both myotonic dystrophy (DM) and non-dystrophic myotonia (NDM). It is felt as stiffness, pain, fatigue, and weakness. In France, mexiletine, a non-selective voltage-gated sodium channel blocker, is approved for the treatment of myotonia in adults with NDM, and it has a temporary recommendation for use in the symptomatic treatment of DM in adults.

Pseudotumoural optic neuritis in myelin oligodendrocyte glycoprotein antibody-associated disease in children: Pseudotumoural optic neuritis in MOG antibody-associated disease.

Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD) is a neuro-inflammatory condition affecting adults and children. The presentations vary and include acute disseminated encephalomyelitis, optic neuritis or transverse myelitis. Optic neuritis associated with anti-MOG antibodies is typically bilateral, anterior and initially severe but usually resolves quickly and completely.

Food protein induced enterocolitis syndrome: French practices assessment in children.

Background: Food protein-induced enterocolitis syndrome (FPIES) is a specific non IgE-mediated food allergy. The international consensus guidelines defined diagnosis criteria and management plan in 2017.

Objectives: To assess practices regarding FPIES in France and in French-speaking countries, according to those guidelines.

Effects of blood-brain barrier opening using ultrasound on tauopathies: A systematic review.

Unlabelled: Blood-brain barrier opening with ultrasound can potentiate drug efficacy in the treatment of brain pathologies and also provides therapeutic effects on its own. It is an innovative tool to transiently, repeatedly and safely open the barrier, with studies showing beneficial effects in both preclinical models for Alzheimer's disease and recent clinical studies. The first preclinical and clinical work has mainly shown a decrease in amyloid burden in mice models and in patients.

Nivolumab plus ipilimumab versus nivolumab in microsatellite instability-high metastatic colorectal cancer (CheckMate 8HW): a randomised, open-label, phase 3 trial.

Background: CheckMate 8HW prespecified dual primary endpoints, assessed in patients with centrally confirmed microsatellite instability-high or mismatch repair-deficient status: progression-free survival with nivolumab plus ipilimumab compared with chemotherapy as first-line therapy and progression-free survival with nivolumab plus ipilimumab compared with nivolumab alone, regardless of previous systemic treatment for metastatic disease. In our previous report, nivolumab plus ipilimumab showed superior progression-free survival versus chemotherapy in first-line microsatellite instability-high or mismatch repair-deficient metastatic colorectal cancer in the CheckMate 8HW trial. Here, we report results from the prespecified interim analysis for the other primary endpoint of progression-free survival for nivolumab plus ipilimumab versus nivolumab across all treatment lines.

Clinical outcomes and management following progressive disease with anti-PD-(L)1 therapy in patients with advanced Merkel Cell Carcinoma.

Aim: Merkel Cell Carcinoma (MCC) is a rare skin cancer with a rising incidence worldwide. Anti-programmed death-1/ligand-1 (anti-PD-(L)1) therapies are effective for the treatment of advanced MCC. This study examines patterns of response / progression of advanced MCC to anti-PD-(L)1 therapies and describes subsequent management.

Imaging-based prediction of early recurrence and neoadjuvant therapy outcomes for resectable beyond Milan HCC.

Purpose: To develop and validate an MRI-based model for predicting postoperative early (≤2 years) recurrence-free survival (RFS) in patients receiving upfront surgical resection (SR) for beyond Milan hepatocellular carcinoma (HCC) and to assess the model's performance in separate patients receiving neoadjuvant therapy for similar-stage tumors.

Method: This single-center retrospective study included consecutive patients with resectable BCLC A/B beyond Milan HCC undergoing upfront SR or neoadjuvant therapy. All images were independently evaluated by three blinded radiologists.

Efficacy and safety of dapsone in adult primary immune thrombocytopenia.

To assess efficacy and safety of dapsone in adult immune thrombocytopenia (ITP), a multicenter randomized controlled trial (RCT) and a real-word study cohort were performed. Participants were adults with primary ITP, transient response to corticosteroids ± intravenous immunoglobulin, and a platelet count ≤ 30x109/L (or ≤ 50x109/L with bleeding). Patients in the RCT were randomized in arm A (prednisone x3weeks+dapsone for 12 months) or arm B (prednisone alone).