337 results match your criteria: "Hopital Universitaire Robert-Debre[Affiliation]"

[Child and adolescent obesity: what assessment?].

Rev Prat

January 2023

Service d'endocrinologiediabétologie pédiatrique, hôpital universitaire Robert-Debré, AP-HP, Paris, France, et Groupe de recherche en médecine et santé de l'adolescent.

WHAT ASSESSMENT? The prescription of laboratory tests or imaging in obese children and adolescents should be guided by the clinical features. It recognizes two main objectives: to eliminate a differential diagnosis (in particular, due to hypercorticism or hypothyroidism) and to detect the presence of comorbidities, assuming that some complications of obesity in adults can be sometimes present even in young age.

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Heterozygous pathogenic variation in GCH1 associated with treatable severe spastic tetraplegia.

Parkinsonism Relat Disord

April 2023

Université de Lorraine, INSERM UMR_S 1256, Nutrition, Genetics, and Environmental Risk Exposure (NGERE), 54000, Nancy, France; Service de neurologie, CHRU de Nancy, Nancy, France; Service de génétique clinique, CHRU de Nancy, Nancy, France. Electronic address:

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Risk of Admission to the Pediatric Intensive Care Unit for SARS-CoV-2 Delta and Omicron Infections.

J Pediatric Infect Dis Soc

April 2023

Pediatric Intensive Care Unit, Hôpital Universitaire Robert-Debré, Université de Paris, Paris, France.

Background: The severity of SARS-CoV-2-related diseases in children remains unclear. This study aimed to describe the incidence of French pediatric intensive care units (PICUs) admissions with acute COVID-19, incidental positive SARS-CoV-2 test result, and multisystem inflammatory syndrome in children (MIS-C) during the delta and omicron variant periods.

Methods: This study used the French PICU registry to obtain data on all patients admitted to 41 French PICUs diagnosed with acute COVID-19, incidental positive SARS-CoV-2 test result, or MIS-C between August 30, 2021 and April 20, 2022.

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Objectives: PERFUSE is a non-interventional study of 1233 patients [inflammatory rheumatic disease, n = 496; inflammatory bowel disease (IBD), n = 737] receiving infliximab (IFX) biosimilar SB2 therapy. This analysis describes response to treatment and persistence on SB2 for up to 12 months in pediatric IBD patients (n = 126).

Methods: Pediatric IBD patients with Crohn disease (CD) or ulcerative colitis (UC), either naïve or switched from originator IFX, who started SB2 in routine practice after September 2017 were eligible.

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Early urine output monitoring in very preterm infants to predict in-hospital neonatal outcomes: a bicentric retrospective cohort study.

BMJ Open

January 2023

Département de la Femme, de l'Enfant et de l'Adolescent, Service des soins intensifs pédiatriques et néonatals, Hôpitaux Universitaires de Genève, Geneve, Switzerland

Objective: To evaluate whether urine output (UO), rarely assessed in the literature, is associated with relevant neonatal outcomes in very preterm infants, and which UO threshold may be the most clinically relevant.

Design: Retrospective cohort study.

Setting: Two Level IV neonatal intensive care units.

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Introduction: Adolescents living with HIV are more likely to experience mental health challenges compared to their peers who do not have HIV. However, there is a lack of data regarding the mental health of adolescents living with HIV in Cameroon. Understanding risk factors and protective factors that influence mental health amongst adolescents is critical for effective programming.

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Vitamin D sufficiency is associated with a reduced risk of fractures, diabetes mellitus, cardiovascular events, and cancers, which are frequent complications after renal transplantation. The VITALE (VITamin D supplementation in renAL transplant recipients) study is a multicenter double-blind randomized trial, including nondiabetic adult renal transplant recipients with serum 25-hydroxy vitamin D (25(OH) vitamin D) levels of <30 ng/mL, which is randomized 12 to 48 months after transplantation to receive high (100 000 IU) or low doses (12 000 IU) of cholecalciferol every 2 weeks for 2 months and then monthly for 22 months. The primary outcome was a composite endpoint, including diabetes mellitus, major cardiovascular events, cancer, and death.

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The excess cancer mortality in persons with severe mental illness (SMI) has been well documented, and research suggests that it may be influenced by care-related factors. Our objective was to assess breast cancer care pathways in women with SMI in France, using an exhaustive population-based data-linkage study with a matched case-control design. The cases were 1346 women with incident breast cancer in 2013/2014 and preexisting SMI who were matched with three controls without SMI presenting similar demographics, initial breast cancer type, and year of incidence.

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Innovative methods for smoking prevention interventions need to be investigated to increase attractiveness, access hard-to-reach populations, and increase effectiveness. We studied the feasibility and immediate effects of an intervention to reinforce norms and behaviors of young people related to antismoking, integrated into a popular online community game. A pilot randomized controlled trial was conducted through the HABBO online community.

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Objectives: Glucocorticoid-induced adrenal insufficiency (GI-AI) is a common side effect of glucocorticoid therapy. However, its diagnosis currently relies on the realization of a Low Dose Short Synacthen Test (LD-SST) that requires an outpatient hospital and several blood samples. Our goal was to evaluate whether morning cortisol values could predict the response to LD-SST, in children, to avoid useless dynamic tests and facilitate diagnosis of glucocorticoid induced adrenal insufficiency.

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Aim: Considering the persistent excess mortality of people living with a mental disorder, this article provides an overview of potential causes to identify relevant research perspectives and to support the development of short-term measures in the French context.

Methods: We rely on a narrative review of the literature, both quantitative and qualitative, to define a conceptual framework of the different factors which could contribute to this excess mortality. Particular attention is given to research carried out in France to identify possible measures to implement in line with the specificities of the national context.

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Article Synopsis
  • Delayed haemolytic transfusion reaction (DHTR) is a serious condition that can happen in kids with sickle cell disease after they get a blood transfusion.
  • In a study of 41 children, DHTR usually showed up around 8 days after the transfusion, and 93% of the kids had painful crises.
  • Most kids had severe anemia and some faced major complications like breathing issues, liver problems, and strokes, but luckily, no one died. The study suggests needing better guidelines for treating DHTR in children.
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Ph+ () B-ALL was considered to be high risk, but recent advances in -targeting TKIs has shown improved outcomes in combination with backbone chemotherapy. Nevertheless, new treatment strategies are needed, including approaches without chemotherapy for elderly patients. LIMK1/2 acts downstream from various signaling pathways, which modifies cytoskeleton dynamics via phosphorylation of cofilin.

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We report three siblings from a non-consanguineous family presenting with contractural limb-girdle phenotype with intrafamilial variability. Muscle MRI showed posterior thigh and quadriceps involvement with a sandwich-like sign. Whole-exome sequencing identified two compound heterozygous missense TTN variants and one heterozygous LAMA2 variant.

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Introduction: Vitamin D-dependent rickets type 1A (VDDR1A) is a rare genetic disease associated with loss-of-function variations in the gene encoding the vitamin D-activating enzyme 1α-hydroxylase (CYP27B1). Phenotype-genotype correlation is unclear. Long-term outcome data are lacking.

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Article Synopsis
  • The study explores the effectiveness and safety of off-label targeted therapies in patients with rare autoimmune and inflammatory diseases.
  • A total of 100 patients were enrolled, primarily middle-aged women, with a variety of targeted therapies being used and a significant proportion still on corticosteroid treatments.
  • Results indicated that 56% of patients found the treatment effective, leading to a significant reduction in corticosteroid dosage, while the overall tolerance of these therapies was deemed acceptable despite some serious health incidents.
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Here we present the 3-year results of ZUMA-4, a phase I/II multicenter study evaluating the safety and efficacy of KTEX19, an autologous anti-CD19 chimeric antigen receptor (CAR) T-cell therapy, in pediatric/adolescent patients with relapsed/refractory B-cell acute lymphoblastic leukemia. Phase I explored two dose levels and formulations. The primary endpoint was the incidence of dose-limiting toxicities.

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Background: Acute lymphoblastic leukemia (ALL) is the most common cancer diagnosed in childhood. Survival for patients following relapse remains poor, and achieving complete remission (CR) after relapse is the first critical step to cure. Carfilzomib is a proteasome inhibitor with an acceptable safety profile and clinical activity in adults with multiple myeloma but has not been assessed in children.

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Genetic and psychosocial stressors have independent effects on the level of subclinical psychosis: findings from the multinational EU-GEI study.

Epidemiol Psychiatr Sci

September 2022

Univ Paris Est Créteil, INSERM, IMRB, AP-HP, Hôpitaux Universitaires H. «Mondor», DMU IMPACT, Fondation FondaMental, Créteil F-94010, France.

Article Synopsis
  • This study investigated how genetic factors (specifically, polygenic risk scores for schizophrenia) interact with psychosocial stressors (like childhood trauma and discrimination) to influence the risk of subclinical psychosis in individuals without diagnosed psychotic disorders.* -
  • Researchers analyzed data from the EU-GEI study, which included European descendants, focusing on different dimensions of subclinical psychosis measured by the CAPE scale, and used various statistical models to explore these associations.* -
  • The findings revealed no significant gene-environment interactions; however, the polygenic risk score was linked to positive psychosis dimensions, while psychosocial stressors negatively impacted all subclinical dimensions.*
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Article Synopsis
  • The objective of the study was to evaluate how epidemics and pandemics affect the use of pediatric emergency care services to inform health policy.
  • A systematic review of 131 articles was conducted, finding that most studies (80%) focused on COVID-19, which resulted in a significant 63.86% reduction in pediatric emergency department visits, although other epidemics showed varied effects.
  • The findings suggest that public fear of disease significantly influences how people seek emergency care, and policymakers need to consider this anxiety while also recognizing gaps in reported data on the impact of epidemics on pediatric care usage.
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ROHHAD syndrome without rapid-onset obesity: A diagnosis challenge.

Front Pediatr

August 2022

Pediatric Pulmonology and Allergology Department, Hôpitaux pédiatriques de Nice CHU-Lenval, Nice, France.

Background: ROHHAD syndrome (Rapid-onset Obesity with Hypothalamic dysfunction, Hypoventilation and Autonomic Dysregulation) is rare. Rapid-onset morbid obesity is usually the first recognizable sign of this syndrome, however a subset of patients develop ROHHAD syndrome without obesity. The prevalence of this entity is currently unknown.

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Background: Patients with inflammatory rheumatic and musculoskeletal diseases (iRMD) receiving mycophenolic acid (MPA) may have a less favourable outcome from COVID-19 infection. Our aim was to investigate whether MPA treatment is associated with severe infection and/or death.

Methods: IRMD patients with and without MPA treatment with highly suspected/confirmed COVID-19 were included in this observational multicentre study.

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Background: Children aged younger than 3 years were excluded from the ELIANA phase 2 trial of tisagenlecleucel in children with acute lymphoblastic leukaemia. The feasibility, safety, and activity of tisagenlecleucel have not been defined in this group, the majority of whom have high-risk (KMT2A-rearranged) infant acute lymphoblastic leukaemia and historically poor outcomes despite intensification of chemotherapy, and for whom novel therapies are urgently needed. We aimed to provide real-world outcome analysis of the feasibility, activity, and safety of tisagenlecleucel in younger children and infants with acute lymphoblastic leukaemia.

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