265 results match your criteria: "Necker University Hospital[Affiliation]"

Article Synopsis
  • One-third of COVID-19 patients deteriorate after emergency department admission, necessitating effective prognosis assessments to predict worsening conditions.
  • Current predictive biomarkers include lymphopenia and the PaO2/FiO2 ratio, while markers like immature granulocytes and monocyte differentiation did not show predictive value.
  • A combined score using decreased P/F ratio, lymphopenia, and loss of mHLA-DR proved effective in predicting patient outcomes, pointing to the importance of early immunosuppression monitoring in COVID-19 cases.
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Context: Outcome of craniopharyngioma is related to its locoregional extension, which impacts resectability and the risk of surgical complications. To maximize resection and minimize complications, optic tract localization, temporal lobe extension, and hypothalamic involvement are essential factors for surgical management.

Objective: To assess the outcome of craniopharyngiomas depending on their relation to the hypothalamus location.

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Craniofacial pain syndromes exhibit a high prevalence in the general population, with a subset of patients developing chronic pain that significantly impacts their quality of life and results in substantial disabilities. Anatomical and functional assessments of the greater occipital nerve (GON) have unveiled its implication in numerous craniofacial pain syndromes, notably through the trigeminal-cervical convergence complex. The pathophysiological involvement of the greater occipital nerve in craniofacial pain syndromes, coupled with its accessibility, designates it as the primary target for various interventional procedures in managing craniofacial pain syndromes.

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Background: Epinephrine increases the chances of return of spontaneous circulation (ROSC) in out-of-hospital cardiac arrest (OHCA), especially when the initial rhythm is non-shockable. However, this drug could also worsen the post-resuscitation syndrome (PRS). We assessed the association between epinephrine use during cardiopulmonary resuscitation (CPR) and subsequent intensive care unit (ICU) mortality in patients with ROSC after non-shockable OHCA.

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Letermovir for CMV Prophylaxis in Very High-Risk Pediatric Hematopoietic Stem Cell Transplantation Recipients for Inborn Errors of Immunity.

J Clin Immunol

December 2023

Pediatric Hematology-Immunology and Rheumatology Department, Assistance Publique Hôpitaux de Paris (APHP), Necker University Hospital, Paris, France.

The burden of CMV infection and disease is important in pediatric hematopoietic stem cell transplantation (HSCT), notably in the subgroup of patients with inborn errors of immunity (IEIs). Letermovir (LMV) is now a standard of care for CMV prophylaxis in adult sero-positive (R+) recipients, but is not yet labeled for children. Published pediatric studies are still scarce.

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Prospective Comparison of Attenuation Imaging and Controlled Attenuation Parameter for Liver Steatosis Diagnosis in Patients With Nonalcoholic Fatty Liver Disease and Type 2 Diabetes.

Clin Gastroenterol Hepatol

May 2024

Department of Adult Radiology, Necker University Hospital, AP-HP, Paris, France; Université Paris Cité, Paris, France; Sorbonne Université, CNRS, INSERM Laboratoire d'Imagerie Biomédicale, Paris, France.

Background & Aims: Similarly to the controlled attenuation parameter (CAP), the ultrasound-based attenuation imaging (ATI) can quantify hepatic steatosis. We prospectively compared the performance of ATI and CAP for the diagnosis of hepatic steatosis in patients with type 2 diabetes and nonalcoholic fatty liver disease using histology and magnetic resonance imaging-proton density fat fraction (MRI-PDFF) as references.

Methods: Patients underwent ATI and CAP measurement, MRI, and biopsy on the same day.

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Background: The value of formative objective structured clinical examinations (OSCEs) during the pre-clinical years of medical education remains unclear. We aimed to assess the effectiveness of a formative OSCE program for medical students in their pre-clinical years on subsequent performance in summative OSCE.

Methods: We conducted a non-randomized controlled prospective pilot study that included all medical students from the last year of the pre-clinical cycle of the Université Paris-Cité Medical School, France, in 2021.

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Objectives: There are no established criteria for stiffness after fusionless surgery for neuromuscular scoliosis (NMS). As a result, there is no consensus regarding the surgical strategy to propose at long-term follow-up. This study reports the first use of shear wave elastography for assessing the mechanical response of lumbar intervertebral discs (IVDs) after fusionless bipolar fixation (FBF) for NMS and compares them with healthy controls.

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Background: Accidental hypothermia designates an unintentional drop in body temperature below 35 °C. There is a major risk of ventricular fibrillation below 28 °C and cardiac arrest is almost inevitable below 24 °C. In such cases, conventional cardiopulmonary resuscitation is often inefficient.

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Long-term heart function in cardiac-arrest survivors.

Resusc Plus

December 2023

Intensive Care Unit, Versailles Hospital, 77 rue de Versailles, 78150 Le Chesnay, France.

Purpose: To assess outcomes and predictors of long-term myocardial dysfunction after cardiac arrest (CA) of cardiac origin.

Methods: We retrospectively included consecutive, single-center, prospective-registry patients who survived to hospital discharge for adult out-of-hospital and in-hospital CA of cardiac origin in 2005-2019. The primary objective was to collect the 1-year New York Heart Association Functional Class (NYHA-FC) and major adverse cardiovascular events (MACE).

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Introduction: This study presents the results of a real-life, multicenter, prospective, post-approval safety evaluation of Clairyg® 50 mg/mL, a 5% intravenous immunoglobulin (IVIg) liquid, in 59 children (aged < 12 years) with primary immunodeficiency diseases (PID) ( = 32) or immune thrombocytopenia (ITP) ( = 27) in France.

Methods: The primary objective of the study was to assess the safety and tolerability of Clairyg®, recording all serious and non-serious adverse events (AEs), whether related (rAEs) or not related to the product. Secondary objectives aimed at evaluating the administration of Clairyg® under routine conditions and the available efficacy data to better document the benefit/risk ratio in this pediatric population.

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Background: Advances in vertical take-off and landing (VTOL) technologies may enable drone-like crewed air ambulances to rapidly respond to out-of-hospital cardiac arrest (OHCA) in urban areas. We estimated the impact of incorporating VTOL air ambulances on OHCA response intervals in two large urban centres in France and Canada.

Methods: We included adult OHCAs occurring between Jan.

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Aim: We aimed to investigate the developmental outcome of children with Robin sequence (RS) for whom continuous positive airway pressure was the main strategy to release upper airway obstruction.

Methods: We included children with isolated RS or RS associated with Stickler syndrome who were aged 15 months to 6 years. We used the French version of the Child Development Inventory and calculated the developmental quotient (DQ) for eight different domains and the global DQ (DQ-global).

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Article Synopsis
  • Accurate and standardized phenotypic descriptions are crucial for diagnosing rare diseases, leading to the development of the Human Phenotype Ontology (HPO) system, which organizes these descriptions hierarchically.
  • This study investigates whether improved HPO terms for systemic autoinflammatory diseases (SAIDs) enhance the identification of these conditions among patients.
  • Results indicate that HPO curation significantly boosted correct SAID diagnoses from 66% to 86% and streamlined genome diagnostics, reducing the average candidate diseases for interpretation from 35 to 2.
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Background: Over 16 000 children under the age of 15 died worldwide in 2017 because of liver disease. Pediatric liver transplantation (PLT) is currently the standard of care for these patients. The aim of this study is to describe global PLT activity and identify variations between regions.

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Dynamic contrast-enhanced ultrasound (DCE-US) is a technique to quantify tissue perfusion based on phase-specific enhancement after the injection of microbubble contrast agents for diagnostic ultrasound. The guidelines of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB) published in 2004 and updated in 2008, 2011, and 2020 focused on the use of contrast-enhanced ultrasound (CEUS), including essential technical requirements, training, investigational procedures and steps, guidance regarding image interpretation, established and recommended clinical indications, and safety considerations. However, the quantification of phase-specific enhancement patterns acquired with ultrasound contrast agents (UCAs) is not discussed here.

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Article Synopsis
  • Weill-Marchesani syndrome (WMS) is a genetic disorder marked by short stature, brachydactyly, joint limitations, and specific eye abnormalities like spherophakia and ectopia lentis. Cardiovascular issues can also occur. It has both dominant and recessive genetic forms caused by mutations in different genes.
  • A study involved 61 patients, including 18 from new research and 43 from existing literature, assessing the correlation between their genetic variations and clinical features. It was found that the majority exhibited eye anomalies and a significant portion had short stature and valvulopathy.
  • The research indicates that while eye abnormalities are crucial for diagnosing WMS, its overall symptoms appear more
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Severe adult hemophagocytic lymphohistiocytosis (HLHa) correlates with HLH-related gene variants.

J Allergy Clin Immunol

January 2024

Laboratory of Cellular and Molecular Mechanisms of Hematological Disorders and Therapeutical Implications, INSERM UMR1163/CNRS URL 8254, Paris, France; French National Center for Primary Immunodeficiencies, Necker University Hospital, AP-HP, Paris, France; Imagine Institute, Université Paris Cité, Paris, France; Clinical Hematology, Necker University Hospital, AP-HP, Paris, France. Electronic address:

Article Synopsis
  • - The study investigates the relationship between genetic factors and the severity of adult hemophagocytic lymphohistiocytosis (HLHa), focusing on gene variants related to HLH.
  • - Clinical data from 130 HLHa patients, including their genotypes for 8 specific HLH-related genes, were analyzed to assess disease outcomes, with findings showing a significant association between the number of genetic variants and severity of HLHa.
  • - Results indicate that HLH gene variants can impact the severity and treatment resistance in HLHa patients, highlighting their potential role in understanding the condition better.
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258th ENMC international workshop Leigh syndrome spectrum: genetic causes, natural history and preparing for clinical trials 25-27 March 2022, Hoofddorp, Amsterdam, The Netherlands.

Neuromuscul Disord

August 2023

Genetics and Genomic Medicine Department, UCL Great Ormond Street Institute of Child Health, London WC1N 1EH, UK and Metabolic Unit, Great Ormond Street Hospital, London WC1N 3JH, United Kingdom. Electronic address:

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Objective: Placental infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can lead to placental insufficiency and in-utero fetal death (IUFD). The objective of this study was to confirm and quantify the extent to which fetoplacental infection with SARS-CoV-2 is a cause of fetal death.

Methods: This was a multicenter retrospective cohort study of fetal deaths that underwent postmortem examination between January 2020 and January 2022 in three fetal pathology units in Paris, France.

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Background And Objectives: Activation of the complement system is involved in the pathogenesis of anti-glomerular basement membrane (anti-GBM) disease. Glomerular deposits of complement 3 (C3) are often detected on kidney biopsies. The primary objective of this study was to analyze the prognostic value of the serum C3 level and the presence of C3 glomerular deposits in patients with anti-GBM disease.

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Motor outcomes in patients with infantile and juvenile Pompe disease: Lessons from neurophysiological findings.

Mol Genet Metab

August 2023

Reference Center for neuromuscular diseases, Necker-Enfants-Malades University Hospital, APHP, FILNEMUS, Paris, France; Department of Pediatric Neurophysiology, Necker-Enfants malades Hospital, University of Paris, AP-HP, Paris, France.

In Infantile Onset Pompe Disease (IOPD), enzyme replacement therapy (ERT) may improve survival, cardiac function, and motor development. However, even with early enzyme replacement therapy, some patients experienced poor response to ERT and abnormal motor milestones that could be due to motor neuron involvement. In this long-term retrospective study, we analyzed concomitant clinical motor outcomes and electroneuromyography (ENMG) findings in patients with IOPD and Juvenile Onset Pompe Disease (JOPD).

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