6,906 results match your criteria: "'Bambino Gesu' Children's Hospital[Affiliation]"

Toll-like receptors (TLRs) of human are considered as the most critical immunological mediators of inflammatory pathogenesis of COVID-19. These immunoregulatory glycoproteins are located on the surface and/or intracellular compartment act as innate immune sensors. Upon binding with distinct SARS-CoV-2 ligand(s), TLRs signal activation of different transcription factors that induce expression of the proinflammatory mediators that collectively induce 'cytokine storm'.

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Article Synopsis
  • Acute myocarditis cases in kids are increasing in Italy, with 65 suspected cases reported in 2024, largely linked to parvovirus B19 infection.
  • Among these cases, 29 children needed intensive care and there were eight fatalities, emphasizing the severity of the situation.
  • The study suggests a need for better diagnosis, enhanced monitoring, and standardized treatment strategies, as only about one-third of affected children received effective intravenous immunoglobulins.
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Biomarkers of vaccine safety and efficacy in vulnerable populations: Lessons from the fourth international precision vaccines conference.

Vaccine

January 2025

Clinical Immunology and Vaccinology Unit, Bambino Gesù Children's Hospital, IRCCS, 00165 Rome; Department of Systems Medicine, University of Rome "Tor Vergata", 00133 Rome.

Vaccination has been a cornerstone of public health, substantially reducing the global burden of infectious diseases, notably evident during the COVID-19 pandemic caused by SARS-CoV-2. However, vulnerable populations (VPs), including those in extreme age groups and those with underlying health conditions, have borne a disproportionate burden of morbidity and mortality from infectious diseases. Understanding vaccine immunogenicity in these populations is crucial for developing effective vaccines.

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Rubinstein-Taybi syndrome (RSTS) is a rare autosomal dominant neurodevelopmental disorder linked to haploinsufficiency of CREBBP (RSTS1) and EP300 (RSTS2) genes. Characteristic features often include distinctive facial traits, broad thumbs and toes, short stature, and various degrees of intellectual disability. The clinical presentation of RSTS is notably variable, making it challenging to establish a clear genotype-phenotype correlation, except for specific variants which cause the allelic Menke-Hennekam syndrome.

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Article Synopsis
  • - Pathogenic variants in the SETD5 gene are linked to a neurodevelopmental disorder presenting intellectual disability, autism, and facial dysmorphisms, with some symptoms not appearing in every individual (incomplete penetrance).
  • - A study of 28 patients revealed various neurological symptoms, including low muscle tone (hypotonia), movement disorders, gait issues, and epilepsy in 14% of cases; cognitive impairments ranged from mild to severe in most participants.
  • - The research expands on existing literature to propose a correlation between specific gene variations (genotype) and the observed symptoms (phenotype) in SETD5-related disorders.
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Purpose: Our aim was to explore the applicability of the definition of "large" primary spontaneous pneumothorax (PSP) of adult guidelines to pediatric patients.

Methods: We performed a retrospective monocentric analysis of first-episodes of PSP (Period: January 2010-January 2023). We defined large PSP according to French SPLF/SMFU/SRLF/SFAR/SFCTCV Guidelines.

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Significant Advancements and Evolutions in Chimeric Antigen Receptor Design.

Int J Mol Sci

November 2024

Scientific Institute for Research, Hospitalization and Healthcare, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", 40121 Meldola, Italy.

Recent times have witnessed remarkable progress in cancer immunotherapy, drastically changing the cancer treatment landscape. Among the various immunotherapeutic approaches, adoptive cell therapy (ACT), particularly chimeric antigen receptor (CAR) T cell therapy, has emerged as a promising strategy to tackle cancer. CAR-T cells are genetically engineered T cells with synthetic receptors capable of recognising and targeting tumour-specific or tumour-associated antigens.

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Inherited cardiac channelopathies are major causes of sudden cardiac death (SCD) in young people. Genetic testing is focused on the identification of single-nucleotide variants (SNVs) by Next-Generation Sequencing (NGS). However, genetically elusive cases can carry copy number variants (CNVs), which need specific detection tools.

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Bacterial meningoencephalitis in newborns is a severe and life-threatening pathology, which results from meningeal infection and the subsequent involvement of the brain parenchyma. The severity of the acute onset of symptoms and the risk of neurodevelopmental adverse sequelae in children strongly depend on the timing of the infection, the immunological protection transmitted by the mother to the fetus during pregnancy, and the neonate's inflammatory and immune system response after birth. Although the incidence of neonatal meningitis and meningoencephalitis and related mortality declined in the past twenty years with the improvement of prenatal care and with the introduction of intrapartum antibiotic prophylaxis against Streptococcus beta Hemolyticus group B (Streptococcus Agalactiae) in the 1990s, bacterial meningitis remains the most common form of cerebrospinal fluid infection in pediatric patients.

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Media Device Use and Vision Disorders in the Pediatric Age: The State of the Art.

Children (Basel)

November 2024

Association Internationale pour l'Enfance et la Réhabilitation Visuelle, 75016 Paris, France.

Introduction: Evidence is consistent with increased screen viewing time among children and adolescents, and anticipation at the age at which children interact with media devices. Incorrect use of technology, as well as overuse, may lead to serious consequences. This study aims to revise scientific international literature and to describe the potential eye risks correlated to screen viewing time in the pediatric age.

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To evaluate neurogenic bladder and bowel dysfunction (NBBD) in children with cerebral palsy (CP) and acquired brain injury (ABI), a condition considered less frequent in those patients than in children with spinal cord injury (SCI), and to study the relationship between NBBD and disability grade in this population. We retrospectively reviewed the clinical data of all patients (aged 3-18 years old) admitted during a three-month observation in our neurorehabilitation department. Data collected were as follows: demographic parameters; disability status (Wee-FIM Scale, Gross Motor Function Classification System (GMFCS) and the Communication Function Classification System); and gastrointestinal and urological symptoms (diaries, Bristol scale, Pad Test and International Consultation on Incontinence Modular Questionnaire).

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(1) Background: Helmet Continuous Positive Airway Pressure (H-CPAP) has primarily been used in intensive care settings to treat moderate-to-severe bronchiolitis in infants. We aim to report on the feasibility of H-CPAP for selected infants with bronchiolitis in a pediatric ward. (2) Methods: A retrospective, observational, consecutive case series was studied of 26 patients who received H-CPAP on the pediatric ward from October 2022 to February 2023, including a description of patient outcomes and costs.

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Background: C3 glomerulopathy is a rare clinical entity characterized by dysregulation of the alternative complement pathway in glomerular disease. Studies defining the natural history of C3G in the pediatric population are scarce.

Methods: Patients included in this retrospective study were diagnosed between 2011 and 2020 in 12 European pediatric nephrology units.

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Article Synopsis
  • - Growth hormone treatment has evolved from using pituitary-derived GH for specific cases of GH deficiency to recombinant human GH (rhGH), which is now approved for various disorders beyond just GH deficiency.
  • - RhGH significantly helps improve short stature in GH-deficient patients and has positive effects on metabolic and bone health, with long-term safety studies showing encouraging results, particularly for those with isolated GHD.
  • - New long-acting rhGH formulations are being developed to enhance adherence to treatment and lessen the burden on patients, while ongoing surveillance is needed for safety in higher doses or other clinical conditions.
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GH Replacement in Children and Adolescent following Surgery for Hypothalamic-Pituitary Neoplasia.

Front Horm Res

November 2024

Research Unit Innovative Therapies for Endocrinopaties, IRCCS Bambino Gesù Children's Hospital, Rome, Italy.

Article Synopsis
  • - Growth hormone deficiency (GHD) is a common issue in kids and teens who have surgery for tumors affecting the hypothalamus and pituitary gland, often resulting from the tumor itself or damage from treatment.
  • - Managing brain tumor patients is very complicated, needing a team of experts to carefully decide on the best treatment plan, including when to start or stop growth hormone replacement therapy (GH-rT).
  • - While there’s evidence showing GH-rT is generally safe, there is still ongoing debate about its use and timing in these patients to ensure the best outcomes and quality of life.
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Exclusive breastfeeding (eBF) in infancy appears to offer a developmental advantage for children's brains compared to formula-fed counterparts. Existing research has predominantly focused on global brain measures (i.e.

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Background: The ongoing COVID-19 pandemic has infected more than 500 million people worldwide. Several measures have been taken to reduce the spread of the virus and the saturation of intensive care units: among them, a lockdown (LD) was declared in Italy on 9 March 2020. As a result, gyms, public parks, sports fields, outdoor play areas, schools, and multiple commercial activities have been closed.

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The Role of Nt-Pro-Bnp in Predicting Outcome in Pediatric Hypertrophic Cardiomyopathy.

Pediatr Cardiol

November 2024

Pediatric Cardiology and Cardiac Arrhythmia/Syncope Unit, Bambino Gesù Children's Hospital, IRCCS, Via Della Torre Di Palidoro, 00054, Fiumicino, RM, Italy.

Hypothesis: The aim of the study was to evaluate the correlation between NTproBNP and left ventricular mass (LVM) in children affected by hypertrophic cardiomyopathy (HCM), both asymptomatic, symptomatic, and with implantable cardioverter-defibrillator (ICD).

Methods: A total of 225 pediatric patients were evaluated and 45 were enrolled in the study because met inclusion criteria (age < 18 years; diagnosis of hypertrophic cardiomyopathy; NTproBNP > 600 pg/ml).

Results: The analysis showed a significant correlation between NTproBNP level and LVM (p = 0.

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Global burden of disease attributable to metabolic risk factors in adolescents and young adults aged 15-39, 1990-2021.

Clin Nutr

December 2024

MAFLD Research Center, Department of Hepatology, The First Affiliated Hospital of Wenzhou Medical University, Wenzhou, China; Key Laboratory of Diagnosis and Treatment for the Development of Chronic Liver Disease in Zhejiang Province, Wenzhou, China. Electronic address:

Background: Metabolic risk factors are a significant cause of global burden among adolescents and young adults, but there is a lack of attention to the burden attributable to these metabolic risk factors globally.

Aims: This study aims to provide comprehensive estimates of five important metabolic risk factors and the attributable disease burden in people aged 15-39 years from 1990 to 2021, based on the Global Burden of Disease Study (GBD) database.

Methods: Global total deaths and disability-adjusted life years (DALYs) were used to describe the burden attributable to five common metabolic risk factors, including high fasting plasma glucose (FPG), high low-density lipoprotein cholesterol (LDL-C), high systolic blood pressure (SBP), high body mass index (BMI), and kidney dysfunction, in adolescents and young adults.

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Objective: Few studies have evaluated the efficacy of antiseizure medications (ASMs) according to the etiology of neonatal acute provoked seizures. We aimed to investigate the response to ASMs in term/near term neonates with acute arterial ischemic stroke (AIS), as well as the type of seizure at presentation and the monitoring approach.

Methods: We retrospectively evaluated neonates from 15 European level IV neonatal intensive care units who presented with seizures due to AIS and were monitored by continuous electroencephalography (cEEG) and/or amplitude-integrated EEG (aEEG) in whom actual recordings, timing, doses, and response to ASMs were available for review.

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Linking ferroptosis to thymic involution.

Nat Aging

December 2024

Research Area of Hematology and Oncology, Cell and Gene Therapy, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

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Background & Aims: Long-term effects of coronavirus disease 2019 (long COVID) develop in a substantial number of people following an acute COVID-19 episode. Red beetroot juice may have positive effects on multiple pathways involved in long COVID. The aim of this pilot study was to explore the impact of beetroot juice supplementation on physical function, gut microbiota, and systemic inflammation in adults with long COVID.

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Background & Aims: Glecaprevir/Pibrentasvir (GLE/PIB) has been approved by the European Medicine Agency (EMA) and by the US Food and Drug Administration (US-FDA) for the treatment of children and adolescents from 3 years of age with chronic hepatitis C virus (CHC) infection. The aim of this study was to confirm the real-world effectiveness and safety of GLE/PIB in children and adolescents (3 to < 18 years old) with CHC.

Methods: This prospective, multicentre study involved 11 Italian centres.

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mRNA vaccines demonstrate impaired immunogenicity and durability in vulnerable older populations. We hypothesized that human modeling and proteomics could elucidate age-specific mRNA vaccine actions. BNT162b2-stimulation changed the plasma proteome of blood samples from young (18-50Y) and older adult (≥60Y) participants, assessed by mass spectrometry, proximity extension assay, and multiplex.

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Article Synopsis
  • This study investigates how the electrophysiological properties of ventricular pre-excitation change in young athletes over time, particularly when assessing their eligibility for sports participation.
  • A total of 44 pediatric patients were monitored over a minimum of two years, showing significant changes in parameters such as the accessory pathway's effective refractory period during stress tests, suggesting a need for regular follow-ups.
  • The findings indicate that some patients shifted their risk profiles for cardiac issues, emphasizing the necessity of repeat electrophysiological evaluations to ensure accurate risk assessments for athletic activities.
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