Laparoscopic pancreatic enucleation: how far should we go for parenchyma preservation? A study by the French National Association of Surgery.

Background: Pancreatic enucleation is indicated for selected patients and tumours with very low oncological risk to preserve a maximum of healthy pancreatic parenchyma. Minimally invasive pancreatic enucleation (MIPE) is increasingly performed. This study aims to assess the impact of tumor location and center experience on textbook outcomes (TBO) in patients undergoing MIPE.

[Recommendations for clinical practice: Prevention and management of varicella zoster virus (VZV) infection during pregnancy and the perinatal period (extended version)].

The Société de Pathologie Infectieuse de Langue Française released in 2024 a new national recommendation for clinical practice on the prevention and management of varicella zoster virus (VZV) infection during pregnancy and the perinatal period. The previous recommendation was issued in 1998, at a time of anti-VZV immunoglobulins shortage; it has hence become obsolete. This recommendation is a formalized expert consensus focusing on infectious diseases management; it is drawn up by a multidisciplinary working group (infectiologists, obstetricians, pediatricians, microbiologists, midwives, hygienists).

Safety and Efficacy of IV Onasemnogene Abeparvovec for Pediatric Patients With Spinal Muscular Atrophy: The Phase 3b SMART Study.

Background And Objectives: Safety and efficacy of IV onasemnogene abeparvovec has been demonstrated for patients with spinal muscular atrophy (SMA) weighing <8.5 kg. SMART was the first clinical trial to evaluate onasemnogene abeparvovec for participants weighing 8.

Effect of Triheptanoin on Caudate Atrophy and Motor Scores in Patients With Early-Stage Huntington Disease: A Phase II Study.

Background And Objectives: Brain energy deficiency occurs at the early stage of Huntington disease (HD). Triheptanoin, a drug that targets the Krebs cycle, can restore a normal brain energetic profile in patients with HD. In this study, we aimed at assessing its efficacy on clinical and neuroimaging structural measures in HD.

Water vapor thermal therapy for treatment of lower urinary tract symptoms due to large benign prostatic hyperplasia (≥ 80 g).

Introduction: Water vapor thermal therapy (WVTT; REZUM™; Boston, USA) offers symptom relief with reduced risks of complications in patients with lower urinary tract symptoms (LUTS) related to benign prostatic obstruction (BPO). WVTT therapy has been validated in the pivotal study in men with smaller prostates (< 80 cc). Yet, its feasibility for larger prostates (≥ 80 cc) remains underexplored.

Comparison of the immunological and virological responses to cART between HIV-1/O and HIV-1/M patients followed up in France.

Background: Therapeutic outcomes for patients infected by genetically divergent HIV-1/O are not well-known due to scarce data and the lack of an appropriate comparison with patients infected by pandemic HIV-1/M. We aimed to compare the immunological and virological response to cART between HIV-1/O and HIV-1/M patients followed in France.

Methods: All naïve HIV-1/O subjects initiating cART in France in ANRS-ORIVAO study were compared to naïve HIV-1/M subjects initiating cART in ANRS-COPANA cohort.

Structural variation in nebulin and its implications on phenotype and inheritance: establishing a dominant distal phenotype caused by large deletions.

Introduction: Structural variants (SVs) of the nebulin gene (), including intragenic duplications, deletions, and copy number variation of the triplicate region, are an established cause of recessively inherited nemaline myopathies and related neuromuscular disorders. Large deletions have been shown to cause dominantly inherited distal myopathies. Here we provide an overview of 35 families with muscle disorders caused by such SVs in .

Identifying biomarker-driven subphenotypes of cardiogenic shock: analysis of prospective cohorts and randomized controlled trials.

Background: Cardiogenic shock (CS) is a heterogeneous clinical syndrome, making it challenging to predict patient trajectory and response to treatment. This study aims to identify biological/molecular CS subphenotypes, evaluate their association with outcome, and explore their impact on heterogeneity of treatment effect (ShockCO-OP, NCT06376318).

Methods: We used unsupervised clustering to integrate plasma biomarker data from two prospective cohorts of CS patients: CardShock (N = 205 [2010-2012, NCT01374867]) and the French and European Outcome reGistry in Intensive Care Units (FROG-ICU) (N = 228 [2011-2013, NCT01367093]) to determine the optimal number of classes.

Usefulness of Cerebrospinal Fluid Alzheimer's disease biomarkers in older patients: Evidence from a national multicenter prospective study.

Background: The use of cerebrospinal (CSF) biomarkers in the diagnosis of Alzheimer's disease (AD) has been gaining interest in clinical practice. Although their usefulness has been demonstrated, their potential value in older patients remains debated.

Objectives: To assess whether knowledge of the results of CSF AD biomarkers was associated with the same gain in diagnostic confidence in older adults > 80 than in younger patients.

Water treatment-free prolonged intermittent kidney replacement therapy: A new approach for kidney replacement therapy in the ICU setting. A retrospective study.

The optimal modalities of kidney replacement therapy (KRT) in the ICU remain debated. Intermittent haemodialysis (IHD) and continuous veno-venous haemofiltration (CVVH) are the two main methods. Intermittent haemodialysis requires a water treatment system, which may not be available in all jurisdictions.

Modern clinical genetics in cardiology.

Advances in molecular genetics during the past decades led to seminal discoveries in the genetic basis of cardiovascular diseases, resulting in a new understanding of their pathogenesis, determinants of natural history and more recently paved the way for innovative therapies. A significant gap, however, exists between the rapidly increasing knowledge, especially of cardiovascular Mendelian disorders, and the medical applications in daily practice. This paper will focus on the practical issues the cardiologist may be faced with when suspecting a Mendelian disorder.

Aarskog-Scott syndrome: a clinical study based on a large series of 111 male patients with a pathogenic variant in and management recommendations.

Background: Aarskog-Scott syndrome (AAS) is a rare condition with multiple congenital anomalies, caused by hemizygote variants in the gene. Its description was based mostly on old case reports, in whom a molecular diagnosis was not always available, or on small series. The aim of this study was to better delineate the phenotype and the natural history of AAS and to provide clues for the diagnosis and the management of the patients.

Impact of comorbidities on the mortality benefits of lung cancer screening: a post-hoc analysis of PLCO and NLST trials.

Hypothesis: To evaluate how comorbidities affect mortality benefits of lung cancer screening (LCS) with low-dose computed-tomography (LDCT).

Methods: We developed a comorbidity index (PLCO-ci) using LCS-eligible participants' data from the Prostate Lung Colorectal and Ovarian (PLCO) trial (training set) and the National Lung Screening Trial (NLST) (validation set). PLCO-ci predicts 5-year non-lung cancer (LC) mortality using a regularized Cox model; with performance evaluated by the area under the ROC curve (ROC).

DNA-binding affinity and specificity determine the phenotypic diversity in BCL11B-related disorders.

BCL11B is a Cys2-His2 zinc-finger (C2H2-ZnF) domain-containing, DNA-binding, transcription factor with established roles in the development of various organs and tissues, primarily the immune and nervous systems. BCL11B germline variants have been associated with a variety of developmental syndromes. However, genotype-phenotype correlations along with pathophysiologic mechanisms of selected variants mostly remain elusive.

Comparative evaluation of two simulation technologies for obstetric ultrasound trainees' assessment.

Objective: The objective was to evaluate the performance of two different obstetric ultrasound simulators in assessing the practical skills of trainees compared to the standard method of practical assessment conducted on pregnant women volunteers.

Methods: We performed a prospective observational study. Two simulators were compared: the Simbionix simulator which uses a mannequin and heteroevaluation and the OPUS simulator which uses optical positioning technology with automatic assessment.

Chronic pain as a presenting feature of dysferlinopathy.

Dysferlinopathies, caused by mutations in the dysferlin gene (DYSF) encoding the dysferlin protein, are a clinically heterogeneous group of autosomal recessive muscular dystrophies whose phenotypic spectrum is still evolving. Here we described a patient reporting diffuse muscular pain non related to physical exercise, mimicking fibromyalgic syndrome. Electroneuromyography was normal.

A scoping review evaluating the current state of gut microbiota and its metabolites in valvular heart disease physiopathology.

Background: The human microbiome is crucial in regulating intestinal and systemic functions. While its role in cardiovascular disease is better understood, the link between intestinal microbiota and valvular heart diseases (VHD) remains largely unexplored.

Methods: Peer-reviewed studies on human, animal or cell models analysing gut microbiota profiles published up to April 2024 were included.

Prognosis for fetuses with isolated severe growth retardation from 23 gestational weeks with an initial assessment unfavorable to an active management.

Objective: To study perinatal outcomes for newborns with early, isolated, severe FGR, for whom initial active management was considered unreasonable or impossible at an obstetric-pediatric assessment and to identify the determinants associated with a course that made active management reasonable.

Material And Methods: This retrospective observational single-center study occurred in a level-3 university hospital maternity unit. It included all pregnancies with a singleton fetus presenting isolated FGR <3rd percentile at 23 weeks or more of gestation with an obstetric-pediatric assessment (OPA) initially unfavorable to active management.

[Lactic acidosis associated with metformin: A case series from the Saint-Denis hospital].

Introduction: Metformin is a first line treatment for type II diabetes. Cases of metformin-associated lactic acidosis are regularly reported. A direct causal link between metformin overdose and lactic acidosis is not clearly established.

Cardiovascular risk associated with glucagon-like peptide-1 receptor agonists versus other conventional glucose-lowering drugs in patients with type-2 diabetes: protocol for a nationwide observational comparative study in routine care.

Introduction: Several cardiovascular outcome trials have been conducted to assess the cardiovascular safety and efficacy of glucagon-like peptide-1 receptor agonists (GLP1-RAs) on cardiorenal outcomes in patients with type-2 diabetes (T2D). However, the strict requirements of randomised controlled trials to avoid most confounding factors are at the expense of external validity. Using national real-world data, we aimed to evaluate the effectiveness of GLP-1RAs in association with metformin especially on cardiovascular events, hospitalisation for heart failure and all-cause death in comparison with other diabetes treatment schemes using dipeptidyl peptidase IV inhibitors, sulfonylureas/glinides or insulin also associated with metformin.

Optimizing surgical outcomes in gastric cancer: a comparison of laparoscopic and open total gastrectomy.

Background: The efficacy of the laparoscopic approach for total gastrectomy (TG) in Western countries remains under discussion. Recently, textbook outcome (TO) has gained recognition as a comprehensive measure of quality of care in upper gastrointestinal surgery. Although predictive factors for TO after TG are well documented, the influence of the surgical approach requires further analysis.