1,718 results match your criteria: "Robert Debre Hospital[Affiliation]"

A new step toward non-genotoxic conditioning prior to hematopoietic stem cell transplantation.

Mol Ther

June 2024

Université Paris Cité, Paris, France; Department of Paediatric Immunology and Haematology, Robert Debré Hospital, Assistance Publique-Hôpitaux de Paris, Paris, France; Institut de Recherche Saint-Louis, INSERM U976, Paris, France.

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Global Sensory Features are Linked to Executive and Attentional Impairments in Autism Spectrum Disorders.

J Autism Dev Disord

May 2024

Department of Child and Adolescent Psychiatry, Robert Debré Hospital, APHP, Paris-Cité University, Paris, France.

Sensory features, executive and attentional impairments are frequently reported in individuals with autism spectrum disorders (ASD). However, little is known about their complex relationships. In this study, we aim to examine the executive and attentional difficulties related to distinct sensory profiles.

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Introduction: Cannabis currently stands as the most prevalent illicit substance used by adolescents in France. Its use is associated with an elevated risk of developing psychiatric disorder, affecting neuro-cognitive development, or leading to psycho-social challenges in the long run. Cognitive-behavioral therapies (CBT) have emerged as a preferred approach for treating cannabis use disorders (CUD) in adults.

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Objectives: Monitoring tools that could provide quick predictions of tuberculosis (TB) treatment outcomes are urgently needed. Here, we assessed whether the evolution of selected biomarkers of innate immunity may help monitoring TB treatment response within 2 weeks of treatment initiation.

Methods: ANRS12394-LILAC-TB was a proof-of-concept prospective study: adults with a rifampicin-susceptible TB who are HIV-negative and HIV-infected documented by a positive Xpert MTB/RIF test were enrolled in Cambodia and Côte d'Ivoire.

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Complement Terminal Pathway Activation and Intrarenal Immune Response in C3 Glomerulopathy.

J Am Soc Nephrol

August 2024

Inflammation, Complement and Cancer Team, Centre de Recherche des Cordeliers, Sorbonne Université, Inserm, Université Paris Cité, Paris, France.

Key Points: We evidenced terminal pathway activation (C5b-9 deposits) in most of the glomeruli on kidney biopsy of C3 glomerulopathy. The amount of C5b-9 deposits correlated with disease prognosis in C3 glomerulopathy. Increased terminal pathway activation was found predominantly in a subgroup exhibiting an immuno-fibroblastic signature.

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Article Synopsis
  • Autoimmune lymphoproliferative syndrome (ALPS) is a condition where the body's immune system doesn't work properly, causing problems like too many immune cells and a higher chance of cancer.
  • This research focused on understanding a specific gene called CASP10 to see if it affects ALPS, looking at different changes in that gene in some patients.
  • The results showed that changes in the CASP10 gene didn't really affect how a process called apoptosis (cell death) works in people with ALPS, suggesting that CASP10 isn't important for causing this condition.
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Diagnosis, treatment, and surveillance of Diamond-Blackfan anaemia syndrome: international consensus statement.

Lancet Haematol

May 2024

Pediatric Immunology and Hematology Department and CRMR aplasies médullaires, Robert Debré Hospital, Groupe Hospitalier Universitaire, Assistance Publique-Hôpitaux de Paris Nord, Université de Paris Cité, Paris, France.

Diamond-Blackfan anaemia (DBA), first described over 80 years ago, is a congenital disorder of erythropoiesis with a predilection for birth defects and cancer. Despite scientific advances, this chronic, debilitating, and life-limiting disorder continues to cause a substantial physical, psychological, and financial toll on patients and their families. The highly complex medical needs of affected patients require specialised expertise and multidisciplinary care.

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Population-based study of eclampsia: Lessons learnt to improve maternity care.

PLoS One

May 2024

CRESS, Obstetrical Perinatal and Pediatric Epidemiology Research Team, EPOPé, INSERM, INRA, Université Paris Cité, Paris, France.

Article Synopsis
  • Eclampsia, a rare and serious complication of hypertensive disorders in pregnancy, shows an incidence of 2.8 per 10,000 births, highlighting its potential for being preventable with better care.
  • A study analyzed the care quality provided to women with eclampsia compared to those with severe maternal morbidity from non-eclamptic high blood pressure and found significant inadequacies in antenatal (39% inadequate), pre-eclampsia (76% inadequate), and eclampsia care (50% inadequate).
  • The findings indicate a critical need for improved, evidence-based standards for managing hypertensive disorders during pregnancy to prevent adverse outcomes for mothers and their babies.
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Recombinant ADAMTS13 in Congenital Thrombotic Thrombocytopenic Purpura.

N Engl J Med

May 2024

From the Department of Haematology, University College London Hospitals, London (M.S.); the Department of Hematology and Medical Oncology, Emory University School of Medicine, Atlanta (A.A.); the Department of Internal Medicine, Ohio State University, Columbus (S.R.C.); the Department of Hematology and National Reference Center for Thrombotic Microangiopathies, Hôpital Saint-Antoine, Assistance Publique-Hôpitaux de Paris (AP-HP) and Sorbonne Université (P.C.), the Department of Pediatric Nephrology, Robert Debré Hospital, AP-HP and University of Paris (C.D.), and the Department of Pediatric Nephrology, Hôpital Universitaire Necker-Enfants Malades, AP-HP (N.B.) - all in Paris; the Department of Pediatric Hematology and Oncology, University Medical Center Hamburg-Eppendorf, Hamburg (W.-A.H.), and the Section of Hematology and Oncology, Department of Pediatrics and Adolescent Medicine, Universitätsklinikum Jena, Jena (K.K.) - both in Germany; the Department of Medicine 1, Division of Hematology and Hemostasis, Medical University of Vienna, Vienna (P.K.); the Department of Hematology and Central Hematologic Laboratory, Bern University Hospital, University of Bern, Bern, Switzerland (J.A.K.H.); the Hematology and Hemotherapy Service, Mother and Child Hospital, Biomedical Research Institute of A Coruña, University Hospital Complex of A Coruña, A Coruña, Spain (M.F.L.-F.); the Department of Blood Transfusion Medicine, Nara Medical University, Kashihara, Japan (M.M.); the Division of Hematology, Department of Medicine, and Department of Pathology, Duke University, Durham, NC (T.L.O.); the Department of Hemostasis Disorders and Internal Medicine, Institute of Hematology and Transfusion Medicine, Warsaw, Poland (J.W.); and Takeda Development Center Americas, Cambridge, MA (I.B., M.C., H.L., B.M., M.P., P.P, S.X., P.Z., L.T.W.).

Article Synopsis
  • Congenital thrombotic thrombocytopenic purpura (TTP) is caused by a severe deficiency of the ADAMTS13 enzyme, and the study compared the effectiveness of recombinant ADAMTS13 to standard therapies in preventing TTP events in patients.
  • The phase 3 trial involved patients receiving either recombinant ADAMTS13 or standard therapy in alternating 6-month periods, measuring outcomes like acute TTP events, safety, and pharmacokinetics.
  • Results showed no acute TTP events during recombinant ADAMTS13 prophylaxis, whereas standard therapy had one event; adverse events were lower with recombinant ADAMTS13, and no patients discontinued due to side effects from it, indicating it may be
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It has been known for a long time that epileptic seizures provoke brain neuroinflammation involving the activation of microglial cells. However, the role of these cells in this disease context and the consequences of their inflammatory activation on subsequent neuron network activity remain poorly understood so far. To fill this gap of knowledge and gain a better understanding of the role of microglia in the pathophysiology of epilepsy, we used an established zebrafish Dravet syndrome epilepsy model based on Scn1Lab sodium channel loss-of-function, combined with live microglia and neuronal Ca imaging, local field potential (LFP) recording, and genetic microglia ablation.

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Objective: Pharmacological prevention of postoperative pancreatic fistula (POPF) after pancreatectomy is open to debate. The present study compares clinically significant POPF rates in patients randomized between somatostatin versus octreotide as prophylactic treatment.

Methods: Multicentric randomized controlled open study in patient's candidate for pancreaticoduodenectomy (PD) or distal pancreatectomy (DP) comparing somatostatin continuous intravenous infusion for 7 days versus octreotid 100 μg, every 8 hours subcutaneous injection for 7 days, stratified by procedure (PD vs DP) and size of the main pancreatic duct (>4 mm) on grade B/C POPF rates at 90 days based on an intention-to-treat analysis.

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Anti-CFH-associated hemolytic uremic syndrome: do we still need plasma exchange?

Pediatr Nephrol

November 2024

Pediatric Nephrology Department, Centre de référence des Maladies Rénales Rares MARHEA, Filières Maladies Rares ORKID et ERK-Net, Robert Debré Hospital, APHP, 48 bd Sérurier, 75019, Paris, France.

Article Synopsis
  • A significant portion of atypical hemolytic uremic syndrome (aHUS) in children is linked to autoantibodies against complement factor H, prompting treatments like plasma exchange and eculizumab.
  • In a study of 12 children treated with eculizumab, varying immunosuppressive strategies were used, but the decrease in anti-FH antibody levels was consistent regardless of the regimen.
  • The study concluded that eculizumab is a safe and effective long-term treatment for aHUS related to anti-FH antibodies, and combining it with mycophenolate mofetil (MMF) may be sufficient for management.
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Clinical spectrum and outcome of Takayasu's arteritis in children.

Joint Bone Spine

September 2024

Department of paediatric rheumatology and CEREMAIA, Kremlin-Bicêtre Hospital, AP-HP, université de Paris Saclay, Kremlin-Bicêtre, France.

Objectives: We aimed to compare clinical spectrum and outcome between adults and children with Takayasu's arteritis (TAK) in a European population.

Methods: We made a nationwide retrospective observational study between 1988 and 2019. All adult patients met the ACR diagnostic criteria for TAK and all children met the EULAR/PRINTO/PRES criteria for paediatric TAK.

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Introduction: Evidence regarding the potentiating effects of intravenous dexamethasone on peripheral regional anesthesia in children is sparse. The objective of the current study was to investigate the potentiating effect of intravenous dexamethasone upon pudendal block during surgical correction of hypospadias using Snodgrass technique.

Methods: The study consisted of a monocentric, randomized controlled, double-blinded study.

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This retrospective study evaluated 35 children (median age 5.2 years; range 0.4-18) with myelofibrosis (MF), including 33 with primary myelofibrosis and 2 with secondary myelofibrosis transplanted from matched sibling donor (MSD) (n = 17) or non-MSD (n = 18) between 2000 and 2022.

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Article Synopsis
  • The study aimed to clarify the dermatological features of Costello syndrome (CS) and distinguish them from other similar syndromes like cardiofaciocutaneous syndrome (CFCS) and types of Noonan syndrome (NS).
  • A ten-year multi-center study involving 31 patients found common skin and hair anomalies in CS, such as excessive eyebrows and various types of skin growths, which can help differentiate CS from CFCS and NS.
  • The findings propose multiple melanocytic naevi as a potential marker for a milder form of CS and suggest that acitretin could be beneficial for treating certain skin conditions, although no clear genotype-phenotype link was identified.
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Obstructive sleep apnea (OSA) is common in sickle cell disease (SCD) despite the absence of overweight, suggesting a specific pathophysiology. We previously showed that otherwise healthy children with increased pharyngeal compliance, a main endotype of OSA, exhibited decreased sympathetic modulation. Our objective was to assess whether modifications of heart rate variability (HRV) and compliance are associated in SCD.

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Immunologic aspects of preeclampsia.

AJOG Glob Rep

February 2024

Fundamental Research Division, CEA, Institut de biologie François Jacob, Hemato-Immunology Research Unit, Inserm UMR-S 976, Institut de Recherche Saint-Louis, Paris University, Saint-Louis Hospital, Paris, France (Dr Rouas-Freiss).

Article Synopsis
  • Preeclampsia is a complex condition linked to high blood pressure and organ dysfunction, which can be diagnosed even without protein in urine.
  • The condition involves immune interactions between the mother and fetus, where mechanisms like reduced major histocompatibility complex expression help the fetus evade the mother's immune response.
  • Regulatory T cells and decidual natural killer cells play crucial roles in regulating fetal development and promoting necessary changes in the blood vessels of the uterus for a healthy pregnancy.
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There is growing evidence that imbalance immune activity plays a role in the development of neurodevelopmental and psychiatric disorders in children. Mood dysregulation (MD) is a debilitating transnosographic syndrome whose underlying pathophysiological mechanisms could be revealed by studying its biomarkers using the Research Domain Criteria (RDoC) model. Our aim was to study the association between the network of cord serum cytokines, and mood dysregulation trajectories in offsprings between 3 and 8 years of age.

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Immunovirological status in people with perinatal and adult-acquired HIV-1 infection: a multi-cohort analysis from France.

Lancet Reg Health Eur

May 2024

Assistance Publique-Hôpitaux de Paris (AP-HP), Immunology-Infectious Diseases Unit, Hôtel-Dieu Hospital, Université Paris Cité, Paris, France.

Background: No study has compared the virological and immunological status of young people with perinatally-acquired HIV infection (P-HIV) with that of people with HIV adulthood (A-HIV) having a similar duration of infection.

Methods: 5 French cohorts of P-HIV and A-HIV patients with a known date of HIV-infection and receiving antiretroviral treatment (ART), were used to compare the following proportions of: virological failure (VF) defined as plasma HIV RNA ≥ 50 copies/mL, CD4 cell percentages and CD4:CD8 ratios, at the time of the most recent visit since 2012. The analysis was stratified on time since infection, and multivariate models were adjusted for demographics and treatment history.

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The recent advisory issued by the United States Food and Drug Administration, cautioning against the routine administration of probiotics in preterm neonates, has sparked a lively debate within the scientific community. This commentary presents a perspective from members of the Special Interest Group on Gut Microbiota and Modifications within the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN) and other authors who contributed to the ESPGHAN position paper on probiotics for preterm infants, as well as representatives from the European Foundation for the Care of Newborn Infants. We advocate for a more nuanced and supportive approach to the use of certain probiotics in this vulnerable population, balancing the demonstrated benefits and risks.

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Background: In juvenile systemic lupus erythematosus (j-SLE) with neuropsychiatric (NP) symptoms, there is a lack of diagnostic biomarkers. Thus, we study whether PET-FDG may identify any metabolic dysfunction in j-NPSLE.

Methods: A total of 19 FDG-PET exams were consecutively performed using PET-MRI system in 11 non-sedated patients presenting with j-NPSLE (11-18y) for less than 18 months (m) and without any significant lesion at MRI.

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Parental involvement and adjustment during the diagnostic evaluation of autism spectrum disorder at two diagnostic centers.

Encephale

March 2024

Pôle de psychiatrie de l'enfant et de l'adolescent 93I05, EPS Ville-Evrard, 93330 Neuilly-sur-Marne, France.

Objectives: The primary objective of this study was to determine the effects of permanent, mediated parental presence during all autism spectrum disorder diagnostic evaluations on parental adjustment (perceived parental stress and sense of parental competence) compared with procedures that traditionally only involve parents in pivotal periods of the diagnosis. The level of satisfaction with the diagnostic procedure and parents' needs were also evaluated to complete this first objective. The secondary objective was to assess the effects of psychosocial, individual, and contextual variables on perceived parental stress and sense of parental competence.

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Stomach size in anorexia nervosa: A new challenge?

Eur Eat Disord Rev

July 2024

Department of Diabetes, Endocrinology and Nutrition, Robert-Debré Hospital, Reims, France.

Background & Aims: Changes in stomach size may impact eating behaviour. A recent study showed gastric dilatation in restrictive eating disorders using computed tomography scans. This study aimed to describe stomach size in the standing position in women with anorexia nervosa (AN).

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