24 results match your criteria: "Robert Debré Mother-Child University Hospital[Affiliation]"

Article Synopsis
  • Familial Mediterranean fever (FMF) is a genetic condition marked by inflammation episodes, usually treated with colchicine to prevent complications like amyloid A amyloidosis, but its necessity for heterozygous patients is debated.* -
  • A study analyzed 747 FMF patients' demographic and inflammatory biomarker data, revealing that heterozygous patients generally exhibited lower inflammatory markers compared to those with more severe genetic variants.* -
  • The findings suggest that certain biomarkers (S100A8/A9 and S100A12) can indicate disease activity, and some heterozygous patients may be eligible to safely stop colchicine treatment.*
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Article Synopsis
  • Familial Mediterranean fever (FMF) is a hereditary inflammatory disease commonly affecting digestion, but its digestive issues during attack-free periods have been under-researched.
  • This study looked at 10 pediatric FMF patients who had endoscopy or colonoscopy and found common features such as the M694V mutation, chronic abdominal pain, iron deficiency, and growth retardation.
  • Histological findings showed low-grade mucosal inflammation, indicating a unique intestinal profile that differs from typical inflammatory bowel diseases, suggesting these patients need specialized gastroenterological care.
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To describe critically ill neonates with premature closure of the ductus arteriosus (DA) and assess the frequency as well as predictive factors for extracorporeal membrane oxygenation (ECMO) support in the latter. This was a monocentric retrospective observational study conducted in the NICU of a French academic medical center between 01/01/2013 and 01/01/2023. All neonates diagnosed with premature closure of the DA were included.

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Introduction: In children, respiratory distress due to upper airway obstruction (UAO) is a common complication of extubation. The quantitative cuff-leak test (qtCLT) is a simple, rapid and non-invasive test that has not been extensively studied in children. The objective of the ongoing study whose protocol is reported here is to investigate how well the qtCLT predicts UAO-related postextubation respiratory distress in paediatric intensive care unit (PICU) patients.

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Article Synopsis
  • Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are serious skin reactions mainly triggered by drugs, with limited research on their occurrence in children.
  • A study analyzed pediatric cases of SJS-TEN from the WHO VigiBase database to identify drugs associated with these reactions, covering data from 1967 to 2022.
  • The research found significant signals for 165 drugs, predominantly antiepileptics and anti-infectious medications, with lamotrigine and carbamazepine being the most notable, while it ruled out vaccines as a significant risk factor.
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Background: Paediatric sepsis is the leading cause of death in children under 5 years. No studies have evaluated the application of the Surviving Sepsis Campaign 2020 (SSC-2020) guidelines in paediatric emergency departments (PEDs).

Objective: To assess physician adherence to the SSC-2020 fluid resuscitation guidelines in children with suspected septic shock in PEDs.

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Juvenile neuropsychiatric systemic lupus erythematosus: A specific clinical phenotype and proposal of a probability score.

Lupus

April 2024

General Paediatrics, Department of Infectious Disease and Internal Medicine, Robert Debré Mother-Child University Hospital, Reference Centre for Rheumatic, AutoImmune and Systemic Diseases in Children (RAISE), AP-HP, Paris, France.

Article Synopsis
  • Juvenile systemic lupus erythematosus (j-SLE) is a rare autoimmune disease affecting multiple organs, with neuropsychiatric involvement (j-NPSLE) leading to higher morbidity and mortality rates in affected youth.
  • *In a retrospective study of j-SLE patients, 44% were diagnosed with j-NPSLE, showcasing common symptoms such as cognitive issues, hallucinations, and mood disorders, with imaging revealing nonspecific brain changes.
  • *The study developed a risk score based on clinical features and cerebrospinal fluid analysis to improve the diagnosis and management of j-NPSLE, emphasizing the importance of early recognition and treatment.
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Assessment of type I interferon response in routine practice in France in 2022.

RMD Open

June 2023

Immunology Laboratory, Hôpital Lyon-Sud, Hospices Civils de Lyon, Lyon, France

An European Alliance of Associations for Rheumatology task force recently recommended specific points to consider for exploring type I interferon pathway in patients, highlighting the lack of analytical assays validated for clinical routine. We report here the French experience on a type I interferon pathway assay that has been set up and used routinely since 2018 in Lyon, France.

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Introduction: Severe sialorrhoea is a common, distressing problem in children/adolescents with neurodisabilities, which has adverse health and social consequences. The SALIVA trial is designed to evaluate the efficacy and safety of a paediatric-specific oral solution of glycopyrronium along with its impact on quality-of-life (QoL), which has been lacking from previous trials of sialorrhoea treatments.

Methods And Analysis: A double-blind, placebo-controlled, randomised phase IV trial is ongoing in several centres across France.

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Fluid balance in pediatric postoperative liver transplant recipients.

Pediatr Transplant

June 2023

Division of Critical Care, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.

Background: Positive fluid balance (FB) is associated with poor outcomes in critically ill children but has not been studied in pediatric liver transplant (LT) recipients. Our goal is to investigate the relationship between postoperative FB and outcomes in pediatric LT recipients.

Methods: We performed a retrospective cohort study of first-time pediatric LT recipients at a quaternary care children's hospital.

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Survival and neurodevelopmental impairment of outborn preterm infants at 5.5 years of age: an EPIPAGE-2 prospective, matched study using multiple imputation.

BMJ Paediatr Open

December 2022

Obstetrical, Perinatal and Pediatric Epidemiology Research Team (EPOPé), Centre of Research in Epidemiology and StatisticS (CRESS), Université Paris Cité, INSERM, INRAE, Paris, France

Objective: To determine whether birth outside a level-3 centre (outborn) is associated with a difference in the combined outcome of mortality or moderate-to-severe neurological impairment at 5.5 years of age compared with birth in a level-3 centre (inborn) when antenatal steroids and gestational age (GA) are accounted for.

Design: Individual matched study nested within a prospective cohort.

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Introduction: Juvenile systemic lupus erythematosus (j-SLE) is a rare chronic autoimmune disease affecting multiple organs. Ranging from minor features, such as headache or mild cognitive impairment, to serious and life-threatening presentations, j-neuropsychiatric SLE (j-NPSLE) is a therapeutic challenge. Thus, the diagnosis of NPSLE remains difficult, especially in pediatrics, with no specific biomarker of the disease yet validated.

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Phenotype and cytokine profile in a TRAPS Syndrome family with TNFRSF1A p.(Thr79Met): Association with sacro-iliitis.

Joint Bone Spine

October 2022

Department of rheumatology, 2, rue de la Milétrie, 86000 Poitiers, France; LITEC EA4331 pole biologie santé - B361, rue-Georges Bonnet, TSA 51106, 86073 Poitiers cedex 9, France; Laboratoire immunologie et inflammation, 2, rue de la Milétrie, 86000 Poitiers, France.

Objective: A comparative retrospective and analytic study was performed in 13 members of a family, affected or not by tumor necrosis factor TNF-α receptor-associated periodic syndrome (TRAPS), including one patient with sacro-illitis.

Methods: Clinical features and TNFRSF1A gene analysis were reported for each family member, symptomatic or asymptomatic. Biological features including CRP/SAA, IgD and ex vivo T lymphocytes and myeloid-derived cytokine profile (IL1-β, IL-1α, IL-1ra, IL-4, IL-10, IL-17, IFN-γ, TNF-α, IL-6) were characterized for all family members.

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Severe imported malaria involving hyperparasitemia (≥ 10%) in non-immune children: Assessment of French practices.

Arch Pediatr

May 2022

Assistance Publique - Hôpitaux de Paris, Armand Trousseau University Hospital, Pediatric Emergency Department, 26 Avenue du Dr Arnold Netter 75012 Paris, France.

Article Synopsis
  • The study focused on managing children with Plasmodium falciparum hyperparasitemia (≥10%) in French hospitals, as per pediatric guidelines, aiming to understand their clinical course and treatment approaches.
  • Conducted over eight hospitals from 2007 to 2014, the research analyzed data from 61 non-immune children, comparing outcomes of those treated in pediatric intensive care units versus other settings.
  • Findings revealed that most children (77%) were treated outside intensive care, primarily with oral antimalarial medication, with no reported deaths, highlighting the importance of thorough clinical evaluation and hospital supervision for effective management.
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Conjoined twining is one of the most fascinating and challenging situations which a pediatric surgeon may face in his career. Only few surgeons may have the opportunity to share in separation of such cases. In this report, we aim to share our experience with the successful separation of ventrally fused male conjoined twins (omphaloischiopagus).

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Article Synopsis
  • Postoperative delirium (POD) is common in elderly surgical patients, leading to serious complications, longer recovery times, and higher healthcare costs; researchers believe melatonin might help prevent it due to its antioxidant and immune-boosting properties.
  • The DELIRLESS trial will study the effects of melatonin versus a placebo on POD incidence in 718 patients aged 70+ undergoing surgery for lower limb fractures, comparing results based on validated delirium assessment scores.
  • The trial has received ethical approval and aims to publish findings to contribute to medical knowledge on preventing POD in older patients.
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Introduction: Bronchiolitis is the leading cause of hospitalization for infants but its economic burden is not well documented. Our objective was to describe the clinical evolution and to assess the 1-month cost of a first episode of acute bronchiolitis presenting to the emergency department (ED).

Methods: Our study was an epidemiologic analysis and a cost study of the cohort drawn from the clinical trial GUERANDE, conducted in 24 French pediatric EDs.

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Aims: In 2018, 1.07 million pregnant women received antiretroviral drugs, raising whether this affects pregnancy outcomes. We assessed the adverse pregnancy outcomes associated with prenatal antiretroviral drug exposure, notified to the French ANRS pharmacovigilance system.

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The Potential and the Limitations of Esophageal Robotic Surgery in Children.

Eur J Pediatr Surg

April 2022

Department of Pediatric Surgery, Hôpital des Enfants, Limoges, France.

Introduction:  There have been numerous reports of robotic pediatric surgery in the literature, particularly regarding urological procedures for school-aged children. Thoracic procedures appear to be less common, despite the fact that encouraging results were reported more than 10 years. Our aim was to report a national experience of esophageal robotic-assisted thoracoscopic surgery (ERATS) and to discuss the most appropriate indications.

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Objectives: To develop and validate a prediction rule to identify well-appearing febrile infants aged ≤90 days with an abnormal urine dipstick at low risk of invasive bacterial infections (IBIs, bacteraemia or bacterial meningitis).

Design: Ambispective, multicentre study.

Setting: The derivation set in a single paediatric emergency department (ED) between 2003 and 2017.

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Meckel's diverticulum (MD) is the most common congenital abnormality of the gastrointestinal tract. Intestinal occlusion due to MD is a commonly observed consequence of intussusception or volvulus. Here, we report a case of an 11-year-old boy who presented to the emergency department with acute abdominal pain, bilious vomiting, and abdominal rebound tenderness.

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Article Synopsis
  • The study aimed to compare the effectiveness of two different flow rates (2 L/kg/min and 3 L/kg/min) for high-flow nasal cannula therapy in infants with acute viral bronchiolitis.
  • Results showed similar failure rates in both groups, with respiratory distress being the main reason for failure; however, discomfort and length of stay in the pediatric ICU were greater in the 3 L/kg/min group.
  • The conclusion indicated that increasing the flow rate to 3 L/kg/min did not provide any additional benefits over 2 L/kg/min for managing respiratory support in these infants.
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Article Synopsis
  • Acute bronchiolitis is a major reason for hospitalizations in infants, and previous research suggested limited effectiveness of nebulized hypertonic saline (HS) treatment in emergency settings.
  • The GUERANDE study was a large, multicenter clinical trial that aimed to determine if nebulized HS could reduce hospital admissions for infants experiencing their first acute bronchiolitis episode.
  • Results showed no significant difference in hospital admission rates between the HS group (48.1% admitted) and the normal saline (NS) group (52.2% admitted), although the HS group did show greater improvement in respiratory distress symptom scores.
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