ORPHEE: A Real-World Study on rIX-FP Prophylaxis Use in Adolescent/Adult Patients With Hemophilia B.

Objectives: To assess the real-world efficacy and safety of recombinant factor IX albumin fusion protein (rIX-FP) in patients with hemophilia B (HB) in France.

Methods: Data on dosing frequency, weekly consumption, and bleeds before-and-after switching to rIX-FP, were collected from December 2021 to February 2024. Annualized (spontaneous) bleeding rates [A(s)BRs] were calculated only in patients on prophylaxis with a follow-up ≥ 6 months.

Severe Optic Neuropathy Induced by Very Prolonged Tedizolid as Suppressive Therapy: Description of a Case Report and Implication for Better Assessment.

The long-term tolerability of linezolid is low because of mitochondrial toxicity, whereas tedizolid may represent a better option for suppressive therapy. We report a first presumed case of tedizolid-associated optic neuropathy after a very prolonged (18-month) intake and believe that screening for optic neuropathy should be considered for patients undergoing tedizolid suppression.

External validation of models to estimate gestational age in the second and third trimester using ultrasound: A prospective multicentre observational study.

Objectives: Accurate assessment of gestational age (GA) is important at both individual and population levels. The most accurate way to estimate GA in women who book late in pregnancy is unknown. The aim of this study was to externally validate the accuracy of equations for GA estimation in late pregnancy and to identify the best equation for estimating GA in women who do not receive an ultrasound scan until the second or third trimester.

Prospective multi-center evaluation of the incidence of unplanned extubation and its outcomes in French intensive care units. The Safe-ICU study.

Background: We aimed to determine the epidemiology and outcomes of unplanned extubation (UE), both accidental and self-extubation, in ICU.

Methods: A multicentre prospective cohort study was conducted in 47 French ICUs. The number of mechanical ventilation (MV) days, and planned and unplanned extubation were recorded in each center over a minimum period of three consecutive months to evaluate UE incidence.

How to do pancreatic transection during robotic pancreaticoduodenectomy. Is hanging manoeuvre always necessary?

Since its first description in 1898, pancreaticoduodenectomy has constantly been improved, allowing increasingly more complex operations to be performed even with a minimally invasive approach: laparoscopic and, in recent years, robotic approach. In most cases, similarly to open surgery, parenchymal transection is performed after the creation of a retropancreatic tunnel to ensure adequate control of the mesenteric vessels before sectioning the parenchyma. Sometimes tunnelling can be very difficult even dangerous to achieve, due to conditions such as: vascular involvement by the neoplasm of superior mesenteric vein (SMV) or portal vein (PV); fibrosis secondary to acute pancreatitis (AP) or radiotherapy.

Minimum follow-up for closed rhinoseptoplasties.

Background: There is no established consensus on the postoperative follow-up from which the aesthetic and functional outcomes of rhinoseptoplasty are considered as stable.

Objectives: To contribute to defining the postoperative follow-up from which rhinoseptoplasty outcomes cease to evolve.

Methods: Postoperative assessments of Nasal Obstruction Symptom Evaluation (NOSE) and Rhinoplasty Outcome Evaluation (ROE) scores from 357 closed structural rhinoseptoplasty procedures were prospectively gathered from January 2019 to December 2023.

Langerhans cell histiocytosis oral manifestation: A case report.

Unlabelled: Introduction and importance: Langerhans cell histiocytosis is a rare disorder characterized by the proliferation of Langerhans cells in various tissues. While it commonly affects bones, skin, and other organs, oral manifestations are less frequent but can be diagnostically challenging.

Case Presentation: we present a clinical case of LCH with an oral manifestation in a 14-year-old adolescent.

A ruptured mesenteric lymphangioma causing peritonitis: a case report.

Cystic lymphangioma is a rare disease that is mainly diagnosed in childhood. When diagnosed, the lesion presents an indication for surgery due to the risk of serious complications. Herein, we report the case of a 32-year-old patient who presented to the emergency room for abdominal pain that developed 2 days before with worsening symptoms and abdominal pain in the last 24 hr.

Classification of recombinant factor VIII products and implications for clinical practice: A systematic literature review.

Introduction: Consensus over the definition of recombinant factor VIII (rFVIII) product classification in haemophilia A is lacking. rFVIII products are often classified as standard half-life (SHL) or extended half-life (EHL); despite this, no universally accepted definition currently exists. One proposed definition includes half-life, area under the curve, and technology designed to extend half-life; however, the International Society on Thrombosis and Haemostasis defines activity over time as the most intuitive information for building treatment regimens and the World Federation of Hemophilia describes rFVIII product classification in terms of infusion frequency.

A French Real-World Evidence Study Evaluating the Efficacy, Safety, and Pharmacokinetic Parameters of rVIII-SingleChain in Patients with Hemophilia A Receiving Prophylaxis.

Background:  rVIII-SingleChain is a recombinant factor VIII (FVIII) with increased binding affinity to von Willebrand factor compared with other FVIII products. rVIII-SingleChain is indicated for the treatment and prevention of bleeding episodes in patients with hemophilia A.

Objectives:  To collect real-world evidence data from patients treated with rVIII-SingleChain to confirm the efficacy and safety established in the clinical trial program and carry out a population pharmacokinetic (PK) analysis.

Factors influencing post-surgical survival in degenerative mitral regurgitation.

Aims: Indications for surgery in patients with degenerative mitral regurgitation (DMR) are increasingly liberal in all clinical guidelines but the role of secondary outcome determinants (left atrial volume index ≥60 mL/m2, atrial fibrillation, pulmonary artery systolic pressure ≥50 mmHg and moderate to severe tricuspid regurgitation) and their impact on post-operative outcome remain disputed. Whether these secondary outcome markers are just reflective of the DMR severity or intrinsically affect survival after DMR surgery is uncertain and may have critical importance in the management of patients with DMR. To address these gaps of knowledge the present study gathered a large cohort of patients with quantified DMR, accounted for the number of secondary outcome markers and examined their independent impact on survival after surgical correction of the DMR.

Spontaneous Rupture of the Extensor Pollicis Longus Tendon in Console Video Game Players: A Case Series.

Introduction: Extensor pollicis longus (EPL) tendon spontaneous rupture has been related to numerous risk factors including wrist fractures, rheumatoid arthritis, and steroids administered locally or systemically, as well as repetitive and excessive wrist motion.

Case Report: Four cases of spontaneous rupture of the EPL tendon, diagnosed clinically, in console video game players, were reported by the authors. None of the patients had a predisposing factor.

The MIDA-Q Mortality Risk Score: A Quantitative Prognostic Tool for the Mitral Valve Prolapse Spectrum.

Background: Mitral valve prolapse (MVP) is responsible for a considerable disease burden but is widely heterogeneous. The lack of a comprehensive prognostic instrument covering the entire MVP spectrum, encompassing the quantified consequent degenerative mitral regurgitation (DMR), hinders clinical management and therapeutic trials.

Methods: The new Mitral Regurgitation International Database Quantitative (MIDA-Q) registry enrolled 8187 consecutive patients (ages 63±16 years, 47% women, follow-up 5.

Outpatient laparoscopic sacrocolpopexy: Feasibility and patient satisfaction.

Study Objective: To assess the feasibility and patient satisfaction of outpatient management of laparoscopic sacrocolpopexy.

Design: Observational study.

Setting: Multicentric study in four French centers: La Conception University Hospital, Marseille; Simone Veil Hospital, Cannes; Simone Veil Hospital, Eaubonne; and Jules Verne Clinic, Nantes.

Lunate Dislocation with Flexor Pollicis Longus Avulsion, and Thumb Collateral Ligament Injury: A Case Report.

Introduction: Lunate dislocation is a rare and devastating injury of the wrist that requires prompt management and surgical repair. The association of this lesion with a flexor pollicis longus (FPL) injury and a Stener lesion appears to be extremely uncommon.

Case Report: We present the case of a 31-year-old man who was riding his motorbike when he was struck by a car, resulting in a volar dislocation of the lunate, a concomitant fracture of the second phalanx of the thumb, a FPL avulsion, and a thumb ulnar collateral ligament (UCL) injury.

Clinical experience of switching patients with severe hemophilia to rVIII-SingleChain or rIX-FP.

Objective: Prophylaxis treatment is the current standard of care for patients with severe hemophilia. Factor concentrates with improved pharmacokinetics have offered more options for individualizing treatment. The treatment focus may be on increased protection, aiming for higher trough factor levels or longer dosing intervals to reduce the burden of hemophilia.

Foramen of Winslow hernia: a case report of a rare life-threatening situation.

Internal hernias through the foramen of Winslow are considered as rare events. A 66-year-old female patient presented to our emergency department with epigastric and right upper quadrant pain associated with abdominal distention, nausea and vomiting and signs of shock. A computed tomography scan showed bowel strangulation with distended loops identified within the lesser sac.

Exploration of the fetal skeleton by ultra-low-dose computed tomography: guidelines from the Fetal Imaging Task Force of the European Society of Paediatric Radiology.

Skeletal anomalies are rare, requiring a systematic ultrasound (US) examination of each skeletal part when there is suspicion of a skeletal dysplasia. Although US examination can provide good evaluation of the fetal bones and cartilage, ultra-low-dose three-dimensional (3-D) multi-detector computed tomography (CT) is a useful complementary tool that can significantly improve prenatal diagnostic accuracy in select cases. Given that ultra-low-dose fetal CT remains an irradiating technique, indications should result from a multidisciplinary consensus, acquisition protocols should be optimized and the reporting standardized.

Outcome of consistent guideline-based tricuspid management in patients undergoing degenerative mitral regurgitation correction.

Objectives: Despite coherent guidelines, management of functional tricuspid regurgitation (FTR) consequences on outcome in the context of degenerative mitral regurgitation (DMR) remains controversial due to lacking series of large magnitude with rigorous application of tricuspid guidelines and strict long-term echocardiographic follow-up. Thus, we aimed at gathering such a cohort to examine outcomes of patients undergoing DMR surgery following tricuspid surgery guidelines.

Methods: All consecutive patients with isolated DMR 2005-2015 operated on with baseline FTR assessment and tricuspid annulus diameter measurement were identified.

Benefit of a flash dose of corticosteroids in digestive surgical oncology: a multicenter, randomized, double blind, placebo-controlled trial (CORTIFRENCH).

Background: The modulation of perioperative inflammation seems crucial to improve postoperative morbidity and cancer-related outcomes in patients undergoing oncological surgery. Data from the literature suggest that perioperative corticosteroids decrease inflammatory markers and might be associated with fewer complications in esophageal, liver, pancreatic and colorectal surgery. Their benefit on cancer-related outcomes has not been assessed.