Medulloblastomas with pathogenic variants: A weakly penetrant syndrome with a restricted spectrum in a limited age window.

Background: pathogenic variants (PV) have been recently identified as the most frequent variants predisposing to Sonic Hedgehog (SHH) medulloblastomas (MB); however, guidelines are still lacking for genetic counseling in this new syndrome.

Methods: We retrospectively reviewed clinical and genetic data of a French series of 29 -mutated MB.

Results: All patients developed SHH-MB, with a biallelic inactivation of found in 24 tumors.

Late-onset pulmonary complications following allogeneic hematopoietic cell transplantation in pediatric patients: a prospective multicenter study.

The primary objective of our multicenter prospective study was to describe the incidence of late-onset non-infectious pulmonary complications (LONIPCs) in children undergoing hematopoietic cell transplantation (HCT) using sensitive criteria for pulmonary function test (PFT) abnormalities including the non-specific pattern of airflow obstruction. Secondary objectives were to assess the factors associated with LONIPC occurrence and the sensitivity of the 2014 NIH-Consensus Criteria of bronchiolitis obliterans syndrome (BOS). PFT and clinical assessment were performed prior to HCT and at 6, 12, 24, and 36 months post-HCT.

Molecular and clinicopathologic characterization of pediatric histiocytoses.

The spectrum of somatic mutations in pediatric histiocytoses and their clinical implications are not fully characterized, especially for non-Langerhans cell histiocytosis (-LCH) subtypes. A cohort of 415 children with histiocytosis from the French histiocytosis registry was reviewed and analyzed for BRAF . Most BRAF samples were analyzed by next-generation sequencing (NGS) with a custom panel of genes for histiocytosis and myeloid neoplasia.

[Hereditary angioedema and its new treatments: An update].

Hereditary angioedema, with or without deficient C1 inhibitor level or function, is a rare disease characterized by recurrent attacks of noninflammatory subcutaneous and/or submucosal edema. It may be life-threatening and substantially affects quality of life. Attacks may be spontaneous or induced, in a setting of emotional stress, by infections or physical trauma, in particular.

[Impact of out-of-hospital presentation remote areas of patients with myocardial infarction with ST segment elevation : From the Nord-Alpin Emergency Network [RENAU]].

Background: European guidelines order management of patients with ST-segment elevation myocardial infarction (STEMI) less than 12 hours. They encourage healthcare networks to rapid coronary reperfusion strategy depending on the distance of the patient from the PCI center.

Objective: To determine impact of out-of-hospital presentation of patients with STEMI in remote areas within the framework of a care network, and to define the degree of compliance with local recommendations, and its prognostic impact.

[Dasatinib-induced follicular lymphoid hyperplasia, an entity to know].

Follicular lymphoid hyperplasia induced by dasatinib is an entity recently described. It is sometimes difficult to rule out the diagnostic of small B-cell lymphoma. Usually, the node is swollen, with follicular architecture conserved, composed by germinal centers with variable size and shape, with a hight number of mitoses and tingible bodies macrophages inside.

Frequency and predictors for chronic thromboembolic pulmonary hypertension after a first unprovoked pulmonary embolism: Results from PADIS studies.

Background: Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening complication of a pulmonary embolism (PE) whose incidence and predictors are not precisely determined.

Objective: To determine the frequency and predictors for CTEPH after a first unprovoked PE.

Patients/methods: In a randomized trial comparing an additional 18-month warfarin versus placebo in patients after a first unprovoked PE initially treated with vitamin K antagonist for 6 months, we applied recommended CTEPH screening strategies through an 8-year follow-up to determine cumulative incidence of CTEPH.

Variability of Intraoperative Electrostimulation Parameters in Conscious Individuals: Language Fasciculi.

Objective: The authors analyzed the current-intensity thresholds for electrostimulation of language fasciculi and the possible consequences of threshold variability on brain mapping.

Methods: A prospective protocol of subcortical electrostimulation was used in 50 patients undergoing brain mapping, directly stimulating presumed language fasciculi identified by diffusion tensor imaging.

Results: The stimulation-intensity thresholds for identification of language fasciculi varied among patients (mean minimum current intensity of 4.

Duration of palliative care involvement and immunotherapy treatment near the end of life among patients with cancer who died in-hospital.

Background: Immune checkpoint inhibitors (ICIs) have revolutionised cancer treatment, but their use near the end of life in patients with advanced cancer is poorly documented. This study investigated the association between administration of ICI therapy in the last month of life and the duration of involvement of the palliative care (PC) team, among patients with advanced cancer who died in-hospital.

Methods: In a retrospective, multicentre study, we included all patients who died in 2018 of melanoma, head and neck carcinoma, non-small cell lung cancer or urothelial or renal cancer, in 2 teaching hospitals and one community hospital in France.

Intravenous immunoglobulins in patients with COVID-19-associated moderate-to-severe acute respiratory distress syndrome (ICAR): multicentre, double-blind, placebo-controlled, phase 3 trial.

Background: Acute respiratory distress syndrome (ARDS) is a major complication of COVID-19 and is associated with high mortality and morbidity. We aimed to assess whether intravenous immunoglobulins (IVIG) could improve outcomes by reducing inflammation-mediated lung injury.

Methods: In this multicentre, double-blind, placebo-controlled trial, done at 43 centres in France, we randomly assigned patients (1:1) receiving invasive mechanical ventilation for up to 72 h with PCR confirmed COVID-19 and associated moderate-to-severe ARDS to receive either IVIG (2 g/kg over 4 days) or placebo.

[X-Ray, false friend of the interventional cardiologist - Risks, evaluation, quality improvement - Status report from France-PCI registry].

Although indispensable, radiation exposure during cardiac catheterization procedures can cause specific radiation-induced diseases. These affect the patient but also the interventional cardiologist. Exposure to high or repeated radiation doses causes deterministic effects.

Duration of palliative care involvement and cancer care aggressiveness near the end of life.

Objectives: Previous studies have found an association between aggressive cancer care and lower quality end of life. Despite international recommendations, late or very late referral to palliative care seems frequent. This study aimed to evaluate the association between the duration of involvement of a palliative care team (PCT), and aggressive cancer care, and to identify factors associated with aggressive cancer care.

Assessment of Respiratory Bacterial Coinfections Among Severe Acute Respiratory Syndrome Coronavirus 2-Positive Patients Hospitalized in Intensive Care Units Using Conventional Culture and BioFire, FilmArray Pneumonia Panel Plus Assay.

Background: Approximately 15% of patients infected by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) present with severe forms of the disease and require hospitalization in intensive care units, which has been associated with high mortality rates. The prevalence of bacterial infections in these patients is not well established, and more data are needed to guide empiric antibiotic therapy and improve patient outcomes.

Methods: In this prospective multicenter study, we assessed bacterial coinfections identified in culture from 99 French patients infected by SARS-Cov-2 and hospitalized in intensive care units.

Childhood Langerhans cell histiocytosis with severe lung involvement: a nationwide cohort study.

Background: Lung involvement in childhood Langerhans cell histiocytosis (LCH) is infrequent and rarely life threatening, but occasionally, severe presentations are observed.

Methods: Among 1482 children (< 15 years) registered in the French LCH registry (1994-2018), 111 (7.4%) had lung involvement.

Long-term follow-up of children with risk organ-negative Langerhans cell histiocytosis after 2-chlorodeoxyadenosine treatment.

The nucleoside analogue, 2-chlorodeoxyadenosine (2CDA), was reported to be an active treatment for childhood Langerhans cell histiocytosis (LCH) without risk organ (RO-) involvement. However, we lack data on long-term effects of 2CDA treatment, including the disease reactivation rate, permanent sequelae and long-term tolerance. This study included 44 children from the French LCH registry, treated for a RO- LCH with 2CDA monotherapy (median number of six courses).

Evaluation of Venous Thromboembolism Recurrence Scores in an Unprovoked Pulmonary Embolism Population: A Post-hoc Analysis of the PADIS-PE trial.

Background: We aimed to validate the Men Continue and HERDOO2 (HERDOO2), D-dimer, age, sex, hormonal therapy (DASH), and updated Vienna recurrent venous thromboembolism prediction models in a population composed entirely of first unprovoked pulmonary embolism, and to analyze the impact of the addition of the pulmonary vascular obstruction index (PVOI) on score accuracy.

Methods: Analyses were based on the double-blind, randomized PADIS-PE trial, which included 371 unprovoked pulmonary embolism patients initially treated for 6 months, successively randomized to receive an additional 18 months of warfarin or placebo, and subsequently followed-up for 2 years.

Results: The HERDOO2, DASH, and updated Vienna scores displayed C-statistics of 0.

[Focus on the Odon Device™: Technical improvements, mechanical principles and progress of the clinical research program].

The Odon Device™ has been described in the literature since 2013 and the World Health Organization supports its development through the Human Reproduction Program. This innovative device could be easier to use than usual instruments and could be an alternative to caesarean section during the second stage of labor, especially in countries where access to obstetric care is limited. The aim of the Odon Device™ is to position an air cuff over the fetal head, past its widest point (around the level of the fetal mouth anteriorly and the nape of the fetal neck posteriorly).

[Para valvular leak closure in TAVI].

Literature concerning transcutaneous symptomatic para valvular cardiac leaks closure (PVLC) after trans aortic valve implantation (TAVI) is relatively scarce. Hereby we present 2 clinical cases, one on an Edwards® Sapien 3 valve and the other one on a Medtronic® Evolut R valve. We present also the preliminary results of the 7 PVLC on TAVI included in our prospective FFPP registry during the 2 first years of enrolment (2017-2018), for a total of 158 inclusions for all valves.

[Safety signal detection by the French Addictovigilance Network: Innovative methods of investigation, examples and usefulness for public health].

The French Addictovigilance Network aims to monitor all psychoactive substances with abuse potential including prescription drugs and other legal and illegal substances such as new psychoactive substances (NPS) and its consequences in humans. Created in 1990 through a network of regional addictovigilance centres covering the entire country, these pharmacologists with expertise in addictovigilance have developed interface with different partners (physicians, toxicologists, network of community pharmacies, addictology care centers…) and implemented several original tools of pharmacosurveillance (such as DRAMES [death related to the abuse of medicines] in strong collaboration with toxicologists or such OPPIDUM [observation of illegal products and misuse of psychotropic medications]), complementary to the spontaneous reporting. A such multidimensional approach including proactive surveillance by these tools and also among several heterogenous data sources (such as data from hospitals or claims database) is able to detect early addictovigilance signals and warnings as illustrated with three following examples: cannabis use and acute serious cardiovascular disorders, new synthetic opioids (ocfentanil, carfentanil) and severe opiate overdose or deaths, the diverted use of psychoactive drugs (codeine analgesics or sedative H1 antihistamines called purple drank) by adolescents and young adults.

Immunosuppression and Graft Rejection in Living-related HLA-identical Renal Transplantation: The RADOVFULL Study.

Background: We aimed to describe the immunosuppressive regimens and graft rejection rates in living-related HLA-identical (LR HLAid) renal transplantation.

Methods: We performed a retrospective multicenter analysis of the French national database for LR HLAid renal transplantations performed between 2002 and 2012. Univariate and multivariate analysis were performed to determine risk factors for graft rejection in LR HLAid recipients.

Evaluation of the predictive value of the bleeding prediction score VTE-BLEED for recurrent venous thromboembolism.

Introduction: VTE-BLEED is a validated score for identification of patients at increased risk of major bleeding during extended anticoagulation for venous thromboembolism (VTE). It is unknown whether VTE-BLEED high-risk patients also have an increased risk for recurrent VTE, which would limit the potential usefulness of the score.

Methods: This was a post hoc analysis of the randomized, double-blind, placebo-controlled PADIS-PE trial that randomized patients with a first unprovoked pulmonary embolism (PE) initially treated during 6 months to receive an additional 18-month of warfarin vs.

Predictors for Residual Pulmonary Vascular Obstruction after Unprovoked Pulmonary Embolism: Implications for Clinical Practice-The PADIS-PE Trial.

Background:  We aimed to identify risk factors for residual pulmonary vascular obstruction after a first unprovoked pulmonary embolism (PE).

Methods:  Analyses were based on data from the double-blind randomized "PADIS-PE" trial that included 371 patients with a first unprovoked PE initially treated during 6 uninterrupted months; all patients underwent baseline ventilation-perfusion lung scanning at inclusion (i.e.