91 results match your criteria: "Centre Hospitalier Universitaire Necker[Affiliation]"

Article Synopsis
  • * Out of 75 patients with successful BCR expression, a significant majority had mutated immunoglobulin heavy chains (IgVH), and a subgroup of 12 patients showed a highly dominant BCR clone associated with worse clinical outcomes.
  • * The results indicated that high clonal dominance correlates with reduced BCR diversity and increased expression of specific genes, leading to poorer progression-free survival (PFS) and overall survival (OS) rates, corroborated by an independent patient cohort analysis.
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In Reply to "'Atypical' Anti-GBM Disease: Just Atypical, or Not Anti-GBM Disease at All?".

Am J Kidney Dis

July 2024

Department of Pathology, Centre Hospitalier Universitaire Necker-Enfants Malades, Assistance Publique - Hopitaux de Paris (AP-HP), Paris, France; University of Paris Cité, INSERM U1151, CNRS UMR 8253, Institut Necker-Enfants Malades, Département Croissance et Signalisation, Paris, France.

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Atypical Anti-Glomerular Basement Membrane Nephritis: A Case Series From the French Nephropathology Group.

Am J Kidney Dis

June 2024

Department of Pathology, Centre Hospitalier Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, Paris, France; INSERM U1151, CNRS UMR 8253, Institut Necker-Enfants Malades, Département Croissance et Signalisation, University of Paris Cité, Paris, France.

Article Synopsis
  • Atypical anti-glomerular basement membrane (GBM) nephritis is a rare kidney condition that presents differently than typical cases, showing bright linear immunoglobulin staining without the usual serum anti-GBM antibodies.
  • In a study involving patients diagnosed from 2003 to 2022, 25 out of 38 potential cases were confirmed, with a majority exhibiting symptoms like hematuria and varying forms of glomerulonephritis.
  • The findings suggest that atypical anti-GBM nephritis often progresses slower than typical cases, but further research is necessary to understand its complete nature and implications.
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Article Synopsis
  • This study focuses on the effectiveness of biologic therapies for children with palmoplantar plaque psoriasis, assessing data from 170 patients in the BiPe cohorts.
  • Results show that children with palmoplantar psoriasis tended to be male, had an earlier onset of psoriasis, and experienced more nail involvement compared to those with generalized plaque psoriasis.
  • After three months of treatment, those with palmoplantar psoriasis had higher mean PGA scores and were more likely to discontinue treatment due to inefficacy, suggesting that adalimumab may be the most effective option, but more research is needed for better management guidelines.
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Allogeneic transplantation in advanced cutaneous T-cell lymphomas (CUTALLO): a propensity score matched controlled prospective study.

Lancet

June 2023

Department of Hematology and Bone Marrow Transplantation, Saint-Louis Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Université Paris Cité, Paris, France. Electronic address:

Background: Advanced-stage cutaneous T-cell lymphomas (CTCLs) are rare, usually refractory, and fatal diseases. Case series have suggested that allogeneic haematopoietic stem cell transplantation (HSCT) might improve the prognosis of advanced-stage CTCLs. The objective of this study was to investigate the effect of allogeneic HSCT compared with non-HSCT therapy on the outcome of individuals with advanced-stage CTCLs.

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A specific molecular signature in SARS-CoV-2-infected kidney biopsies.

JCI Insight

March 2023

University of Paris Cité, INSERM U1151, CNRS UMR 8253, Institut Necker-Enfants Malades, Département Croissance et Signalisation, Paris, France.

Acute kidney injury is one of the most important complications in patients with COVID-19 and is considered a negative prognostic factor with respect to patient survival. The occurrence of direct infection of the kidney by SARS-CoV-2, and its contribution to the renal deterioration process, remain controversial issues. By studying 32 renal biopsies from patients with COVID-19, we verified that the major pathological feature of COVID-19 is acute tubular injury (ATI).

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Article Synopsis
  • - Combined therapies in treating childhood psoriasis involve using multiple drugs to enhance effectiveness and minimize side effects, with a study evaluating their use in 170 children across France and Italy.
  • - Out of the participants, 13% received various combinations of conventional and biologic medications, achieving significant improvements in psoriasis scores, despite reporting a few serious adverse events with positive outcomes.
  • - A survey of 61 dermatologists revealed that 64% have used or intend to use these combined therapies, primarily to boost the effectiveness of biologic treatments when initial results are insufficient.
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Background: Dupilumab is the first biotherapy available for the treatment of moderate-to-severe childhood atopic dermatitis (AD).

Objective: The aim of this study was to evaluate the effectiveness and safety of dupilumab in daily practice.

Methods: Patients aged 6-11, who had received a first dose of dupilumab, were included in this multicentre retrospective cohort study.

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[National protocol for diagnosis and care of congenital aniridia: Summary for the attending physician].

J Fr Ophtalmol

June 2022

Inserm 1138, T17, université de Paris, Paris, France; Laboratoire de génétique moléculaire, centre hospitalier universitaire Necker-Enfants Malades, AP-HP, université de Paris, Paris, France.

Congenital aniridia is a rare panocular disease defined by a national diagnostic and care protocol (PNDS) validated by the HAS. In most cases, it is due to an abnormality in the PAX6 gene, located at 11p13. Aniridia is a potentially blinding autosomal dominant disease with high penetrance.

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Norepinephrine (NE) is commonly used in combination with fluid during resuscitation of hemorrhagic shock, but its impact on kidney microcirculation, oxygenation, and function is still unknown in this setting. During hemorrhagic shock resuscitation, does a combination of fluid and NE affect kidney oxygenation tension, kidney microcirculatory perfusion, and 48-hour kidney function, as compared with fluid alone? Hemorrhagic shock was induced in 24 pigs, and 8 pigs were included as a sham group. Resuscitation of hemorrhagic shock was performed, using a closed-loop device, either by fluid alone (0.

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Brachytherapy for Pediatric Patients at Gustave Roussy Cancer Campus: A Model of International Cooperation for Highly Specialized Treatments.

Int J Radiat Oncol Biol Phys

July 2022

Paris-Saclay University, Assistance Publique-Hôpitaux de Paris, Bicêtre Hospital, Department of Pediatric Surgery, Le Kremlin Bicêtre, France.

Purpose: Childhood cancer is rare, and treatment is frequently associated with long-term morbidity. Disparities in survival and long-term side effects encourage the establishment of networks to increase access to complex organ-conservative strategies, such as brachytherapy. We report our experience of an international cooperation model in childhood cancers.

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Article Synopsis
  • The study aimed to analyze the clinical practice and safety of switching biologics among pediatric psoriasis patients in the BiPe cohort, which included 134 patients.
  • Out of the patients, 29 switched biologics mainly due to loss of efficacy, with most transitions occurring from etanercept to adalimumab or ustekinumab.
  • The research found no significant differences in drug survival rates based on whether the biologics were used as first-line or second-line treatments, indicating a need for more data to guide switching in clinical practice.
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Leg ulcers in childhood: A multicenter study in France.

Ann Dermatol Venereol

March 2022

Service de dermatologie et médecine vasculaire, hôpital Victor-Dupouy, 69, rue du Lieutenant-Colonel Prud'hon, 95100 Argenteuil, France.

Background: Leg ulcers in adults are a major public health concern. Their incidence increases with age and many causes have been identified, predominantly associated with vascular diseases. Leg ulcers in children and teenagers are less frequent.

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Clinical gait analysis and physical examination don't correlate with physical activity of children with cerebral palsy. Cross-sectional study.

Int Biomech

December 2020

Pôle Recherche et Innovation, Fondation Ellen Poidatz, Ellen Poidatz Research Lab , Saint Fargeau-Ponthierry, France.

Gait analysis and physical clinical measures are usually performed in children with cerebral palsy to help the surgeons make therapeutic decision. However, the level of physical activity in daily life is not systematically assessed. The aim of this cross sectional study was to examine the correlations between: three-dimensional gait analysis kinematic and spatiotemporal parameters, clinical measures and physical activity.

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These European Resuscitation Council Advanced Life Support guidelines, are based on the 2020 International Consensus on Cardiopulmonary Resuscitation Science with Treatment Recommendations. This section provides guidelines on the prevention of and ALS treatments for both in-hospital cardiac arrest and out-of-hospital cardiac arrest.

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[Prevalence of visual impairment in school-age children: Data analysis from PlanVue® pilot project].

J Fr Ophtalmol

March 2021

Service d'ophtalmologie, Hôpital Ambroise Paré, AP-HP, IHU FOReSIGHT, Université de Versailles Saint-Quentin-en-Yvelines, 9, avenue Charles De Gaulle, 92100 Boulogne Billancourt; Service d'ophtalmologie 3, Centre Hospitalier National d'Ophtalmologie des Quinze-Vingts, IHU FOReSIGHT, Université de Versailles Saint-Quentin-en-Yvelines, 28, rue de Charenton, 75012 Paris. Electronic address:

Purpose: To assess the prevalence of visual disturbances among school-aged children in prioritized education zones in France.

Methods: The PlanVue® pilot project was designed to detect and manage visual disturbances in school-aged children in the prioritized education areas of the city of Nanterre, France. During this pilot study, a cohort of 515 children aged 4 to 13 years underwent a school vision screening between January and March 2019, consisting of an overall evaluation of the child's visual behavior, measurement of uncorrected visual acuity in each eye, objective refraction with a photoscreener and strabismus screening.

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Success and complications by team composition for prehospital pediatric intubation: children also need physicians!

Crit Care

June 2020

SAMU de Paris, SMUR Pédiatrique Necker, Service d'Anesthésie-Réanimation, Centre Hospitalier Universitaire Necker - Enfants Malades, AP-HP.Centre, and Université de Paris, Paris, France.

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Prehospital Plasma Transfusion and Survival in Trauma Patients With Hemorrhagic Shock.

JAMA Surg

August 2020

SAMU de Paris, Service d'Anesthésie Réanimation, Centre Hospitalier Universitaire Necker-Enfants Malades, Assistance Publique-Hôpitaux de Paris, and Université Paris Descartes, Paris, France.

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Tuberculosis contact investigation and short-course preventive therapy among young children in Africa.

Int J Tuberc Lung Dis

April 2020

International Union Against Tuberculosis and Lung Disease, Paris.

The largest cities in Benin, Burkina Faso, Cameroon and Central African Republic. To demonstrate the feasibility and document the effectiveness of household contact investigation and preventive therapy in resource-limited settings. Children under 5 years living at home with adults with bacteriologically confirmed pulmonary tuberculosis (TB) were screened using questionnaire, clinical examination, tuberculin skin test and chest X-ray.

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[Off-label drugs in childhood psoriasis].

Ann Dermatol Venereol

May 2021

Service de dermatologie, hôpital Victor Dupouy, 69, rue du Lieutenant-Colonel Prud'hon, 95100 Argenteuil, France; Cabinet libéral, 5, avenue Maréchal-Foch, 21000 Dijon, France; Cabinet libéral, 3, avenue de Chanzy, 94210 La Varenne-Saint-Hilaire, France; Inserm U1163, service de dermatologie, institut imagine, centre hospitalier universitaire Necker-Enfants-Malades, université Paris Descartes - Sorbonne, Paris Cité, Assistance publique-Hôpitaux de Paris, 149, rue de Sèvres, 75015 Paris, France; Cabinet libéral, Le Bateau Blanc, 26, chemin de Paradis, 13117 Martigues, France; Unité de dermatologie pédiatrique, hôpital Pellegrin-Enfants, centre hospitalier universitaire de Bordeaux, place Amélie-Raba-Léon, 33000 Bordeaux, France; Service de dermatologie, Hôtel-Dieu, 1, place Alexis-Ricordeau, 44000 Nantes, France; Service de dermatologie, hôpitaux de Brabois, centre hospitalier universitaire de Nancy, rue du Morvan, 54500 Vandœuvre-lès-Nancy, France; Cabinet libéral, 6, rue du Helder, 64200 Biarritz, France; Service de dermatologie, hôpital Larrey, centre hospitalier universitaire de Toulouse, 24, chemin de Pouvourville, 31000 Toulouse, France; Service de dermatologie, université de Franche Comté, centre hospitalier régional universitaire de Besançon, 3, boulevard Alexandre-Fleming, 25000 Besançon, France; Service de dermatologie, centre hospitalier d'Auxerre, 2, boulevard de Verdun, 89011 Auxerre, France; Département de santé publique, université Versailles-Saint-Quentin-en-Yvelines, centre hospitalier universitaire Ambroise-Paré, Assistance publique-Hôpitaux de Paris, 9, avenue Charles-de-Gaulle, 92100 Boulogne-Billancourt, France.

Introduction: Psoriasis affects 0.5% of children in Europe, with moderate to severe clinical forms in 15-35% of cases warranting the use of systemic treatments. Few treatments are licensed for childhood psoriasis.

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Prehospital Severe Trauma Management in Tactical Medicine.

JAMA Surg

May 2020

SAMU de Paris, Service d'Anesthésie Réanimation, Centre Hospitalier Universitaire Necker Enfants Malades, Assistance Publique Hôpitaux de Paris, and Université Paris Descartes, Paris, France.

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Severe chronic rhinosinusitis in children should alert clinicians and extensive genotyping should be performed. We propose that thorough clinical and functional assessment in severe chronic rhinosinusitis is valuable to discover rare mutations which could be treated by CFTR correctors to postpone pulmonary infection.

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Long-term safety and efficacy of rituximab in 248 adults with immune thrombocytopenia: Results at 5 years from the French prospective registry ITP-ritux.

Am J Hematol

December 2019

Service de Médecine Interne, Centre National de Référence des Cytopénies Auto-Immunes de l'Adulte, Centre Hospitalier Universitaire Henri-Mondor, Assistance Publique-Hôpitaux de Paris, Université Paris Est Créteil, Créteil, France.

Rituximab is a second-line option in adults with immune thrombocytopenia (ITP), but the estimated 5-year response rate, only based on pooled retrospective data, is about 20%, and no studies have focused on long-term safety. We conducted a prospective multicenter registry of 248 adults with ITP treated with rituximab with 5 years of follow-up to assess its long-term safety and efficacy. The median follow-up was 68.

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