Angioedema due to acquired C1-inhibitor deficiency without hematological condition: a multicenter French cohort study of 34 patients.

Background: Angioedema (AE) due to acquired C1-inhibitor deficiency (AAE-C1-INH) is a rare disease associating recurrent edema of mucosa and skin. Several underlying diseases have been reported, mainly lymphoproliferative diseases and monoclonal gammopathy. However, 15 to 20% of patients never exhibit such a hematological condition.

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.

Acclimatized Lowlanders Exhibit a Hypocoagulable Profile after a Passive Ascent at High Altitude.

Stauffer, Emeric, François Caton, Raphael Marlu, Aurélien Pichon, Landry Seyve, Michael Furian, Aymeric Paillisser, Florence Berquet, Jeremy De Abreu, Blandine Deschamps, Benoit Polack, Philippe Connes, Paul Robach, Stéphane Doutreleau, Julien V Brugniaux, Samuel Verges, and Benoit Champigneulle.Acclimatized lowlanders exhibit a hypocoagulable profile after a passive ascent at high altitude. 00:00-00, 2024.

Outcomes of ABO-incompatible kidney transplants with very high isoagglutinin titers: a single-center experience and literature review.

Background: ABO-incompatible kidney transplantation (ABOi-KTx) represents a possible solution to address the shortage of kidney donors. However, these transplants present immunological challenges, particularly when isoagglutinin titers are elevated pretransplant.

Methods: Single-center retrospective study describing clinical and biological outcomes of 8 patients who underwent ABOi-KTx with initial isoagglutinin titers ≥ 1/512.

Early-life exposures to phenols, parabens and phthalates and fat mass at 3 years of age in the SEPAGES cohort.

Background: Early-life exposure to short half-life chemicals may influence adiposity growth, a precursor to obesity. Previous studies often relied on limited urine samples that inadequately represent exposure during pregnancy or infancy. Additionally, childhood adiposity is commonly estimated using body mass index, which does not accurately reflect body composition.

Brain health: Pathway to primary prevention of neurodegenerative disorders of environmental origin.

While rising global rates of neurodegenerative disease encourage early diagnosis and therapeutic intervention to block clinical expression (secondary prevention), a more powerful approach is to identify and remove environmental factors that trigger long-latencybrain disease (primary prevention) by acting on a susceptible genotype or acting alone. The latter is illustrated by the post-World War II decline and disappearance of Amyotrophic Lateral Sclerosis and Parkinsonism-Dementia Complex (ALS/PDC), a prototypical often-familial neurodegenerative disease formerly present in very high incidence on the island of Guam. Lessons learned from 75 years of investigation on the etiology of ALS/PDC include: the importance of focusing field research on the disease epicenter and patients with early-onset disease; soliciting exposure history from patients, family, and community to guide multidisciplinary biomedical investigation; recognition that disease phenotype may vary with exposure history, and that familial brain disease may have a primarily environmental origin.

Hereditary angioedema in children: Review and practical perspective for clinical management.

Background: Hereditary angioedema (HAE) in children has specific features and requires multidisciplinary management.

Methods: We performed a literature search and underwent in-depth discussions to provide practical tools for physicians.

Results: HAE is a rare, life-threatening genetic disorder.

RELAY: Final Overall Survival for Erlotinib Plus Ramucirumab or Placebo in Untreated, EGFR-Mutated Metastatic NSCLC.

Introduction: RELAY, a global double-blind, placebo-controlled phase 3 study (NCT02411448) found statistically significant improvement in progression-free survival (primary end point) for ramucirumab (RAM) plus erlotinib (ERL) (RAM + ERL) in patients with untreated EGFR-mutated metastatic NSCLC (hazard ratio [HR] = 0.59, 95% confidence interval [CI]: 0.46-0.

An Extended Phenotype of PPP1R13L Cardiocutaneous Syndrome.

Dilated cardiomyopathy (DCM) is a rare disease in children and a leading cause of heart failure. There are numerous causes of DCM including genetic causes leading to isolated or syndromic presentations, with a wide variety of implicated genes. Among them, PPP1R13L is associated with a recessive syndrome leading to cardiac anomalies with skin, teeth, and hair abnormalities.

FLT3 ligand kinetic profile predicts response to treatment in patients with high-risk myelodysplastic syndrome / chronic myelomonocytic leukemia receiving CPX-351: a study from the Groupe Francophone des Myélodysplasies.

Not available.

Restoring mobility: roles of percutaneous consolidation for pelvic ring bone lesions-a multicenter study.

Objectives: This study aimed to assess the early functional rehabilitation outcomes following percutaneous consolidation for pelvic ring tumor lesions.

Materials And Methods: In this multicenter retrospective study, patients with pelvic bone tumor lesions, whether primitive or metastatic, underwent percutaneous consolidation (cementoplasty, screw fixation, or both). The primary outcome was postoperative weight-bearing ambulation.

Rationale and design of the multicentric randomized EVAOLD trial: Evaluation of a strategy guided by imaging versus routine invasive strategy in elderly patients with ischemia.

Background: The management of myocardial infarction without ST segment elevation (NSTEMI) in elderly patients remains challenging, in particular the benefit/risk balance of routine revascularization remains uncertain.

Study Design: EVAOLD is s a multicenter, prospective, open-label trial with 2 parallel arms in NSTEMI patients ≥80 years of age. The aim of the trial is to test whether a strategy of selective invasive management guided by ischemia stress imaging (IMG group) will be noninferior in preventing Major Adverse Cardiac and Cerebrovascular Events (MACCE, ie all-cause death, nonfatal myocardial infarction, nonfatal stroke) rates at 1 year compared with a routine invasive strategy (INV Group).

CIC/ATXN1-rearranged tumors in the central nervous system are mainly represented by sarcomas: A comprehensive clinicopathological and epigenetic series.

CIC fusions have been described in two different central nervous system (CNS) tumor entities. On one hand, fusions of CIC or ATXN1 genes belonging to the same complex of transcriptional repressors, were reported in the CIC-rearranged, sarcoma (SARC-CIC). The diagnosis of this tumor type, which was recently added to the World Health Organization (WHO) Classification of CNS tumors, is difficult mainly because the data concerning its histopathology (as compared to its soft tissue counterpart), immunoprofile, and clinical as well as radiological characteristics are scarce in the literature.

Management of ketamine cystitis: National guidelines from the French Association of Urology (CUROPF/CTMH).

Objective: The objective of the CUROPF and CTMH was to establish recommendations about ketamine induced uropathy management.

Methods: A systematic review of the literature was conducted on Pubmed/Medline by the members of the French committees of female urology and male lower urinary tract symptoms focusing on the epidemiology, pathophysiology, diagnosis and treatment of ketamine induced uropathy, evaluating references and level of evidence.

Results: Recommendations include epidemiology, pathophysiology, diagnosis and treatment of ketamine induced uropathy.

Capmatinib efficacy for METex14 non-small cell lung cancer patients: Results of the IFCT-2104 CAPMATU study.

Background: Capmatinib is a selective MET inhibitor with demonstrated efficacy in a phase II study of non-small cell lung cancer (NSCLC) patients harboring METex14 mutations. However, the real-world outcomes of capmatinib are largely unknown. From June 2019, the French Early Access Program (EAP) provided capmatinib to METex14 NSCLC patients who were ineligible for or for whom first-line standard therapies had failed.