5,101 results match your criteria: "Hospital Sant Joan De Deu[Affiliation]"

[Sleep in chronic neuropediatric diseases].

Medicina (B Aires)

September 2024

Unidad Académica de Neuropediatría, Centro Hospitalario Pereira Rossell, Facultad de Medicina Universidad de la República Oriental del Uruguay (UDELAR), Montevideo, Uruguay. E-mail:

The prevalence of sleep disorders (SD) is notoriously increased in children with chronic neurological disease, with a negative bidirectional link that aggravates their symptomatology and has a negative impact on the quality of life of the child and their families. Identifying and recognizing this association is key for the child neurologist since the treatment of SD significantly improves daytime symptomatology in neurodevelopmental disorders, epilepsy, primary headaches, cerebral palsy and neuromuscular diseases.

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[Rare diseases: unraveling the biological bases to find future therapies].

Medicina (B Aires)

September 2024

Departamento de Neurología Pediátrica, Hospital Sant Joan de Déu, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Barcelona, España. E-mail:

Rare diseases are characterized by low prevalence and high complexity, affecting millions globally. Although technologies like massive sequencing improve diagnose, therapeutic options remain largely symptomatic or palliative, with few curative treatments approved. In the context of rare diseases, especially genetic neurodevelopmental disorders, therapy development faces obstacles such as phenotypic variability, diverse molecular mechanisms, and complexities in assessing neurodevelopment in natural history and clinical trials.

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Article Synopsis
  • The study focused on establishing continuous reference intervals for cerebrospinal fluid (CSF) biomarkers homovanillic acid (HVA) and 5-hydroxyindoleacetic acid (5-HIAA), which are important for diagnosing neurological diseases related to dopamine and serotonin.
  • Utilizing data from a large cohort of pediatric patients, researchers created age-specific models to define these intervals more accurately, addressing the issue of not having healthy control groups.
  • The results showed that the continuous reference intervals reduced the number of secondary deficiencies identified and found no significant links between HVA and 5-HIAA levels and the effects of antiepileptic or neuroleptic medications, indicating that these biomarkers may not always correlate with neurological disorders.
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Longitudinal trajectories of the neural encoding mechanisms of speech-sound features during the first year of life.

Brain Lang

November 2024

Brainlab - Cognitive Neuroscience Research Group. Department of Clinical Psychology and Psychobiology, University of Barcelona, Catalonia, Spain; Institute of Neurosciences, University of Barcelona, Catalonia, Spain; Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Barcelona, Spain. Electronic address:

Infants quickly recognize the sounds of their mother language, perceiving the spectrotemporal acoustic features of speech. However, the underlying neural machinery remains unclear. We used an auditory evoked potential termed frequency-following response (FFR) to unravel the neural encoding maturation for two speech sound characteristics: voice pitch and temporal fine structure.

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Sacubitril/Valsartan in Pediatric Heart Failure (PANORAMA-HF): A Randomized, Multicenter, Double-Blind Trial.

Circulation

November 2024

M3C-Necker, Congenital and Paediatric Cardiology Department, Hospital Necker-Enfants Malades, University of Paris Cité, France (D.B.).

Background: Sacubitril/valsartan, an angiotensin receptor-neprilysin inhibitor (ARNI), is an established treatment for heart failure (HF) with reduced left ventricular ejection fraction. It has not been rigorously compared with angiotensin-converting enzyme inhibitors in children. PANORAMA-HF (Prospective Trial to Assess the Angiotensin Receptor Blocker Neprilysin Inhibitor LCZ696 Versus Angiotensin-Converting Enzyme Inhibitor for the Medical Treatment of Pediatric HF) is a randomized, double-blind trial that evaluated the pharmacokinetics and pharmacodynamics (PK/PD), safety, and efficacy of sacubitril/valsartan versus enalapril in children 1 month to <18 years of age with HF attributable to systemic left ventricular systolic dysfunction (LVSD).

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Unlabelled: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing.

Methods: In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS).

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Introduction And Objectives: We report the results of the 2023 Spanish catheter ablation registry.

Methods: Procedural data were collected and incorporated into the REDCap platform by all participating centers through a specific form.

Results: There were 104 participating centers in 2023 compared with 103 in 2022.

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To describe the characteristics of systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), compare their presentation and evolution, and analyse possible complication predictors. Multicenter study. Data were retrieved from a hospital-based study of patients with a diagnosis or suspected diagnosis of sJIA or AOSD according to the responsible physician and followed-up for at least one year.

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Cardiac channelopathies in pediatrics: a genetic update.

Eur J Pediatr

November 2024

Arrhythmias, Inherited Cardiac Diseases and Sudden Death Unit, Hospital Sant Joan de Déu, Esplugues de Llobregat, 08950, Barcelona, Catalonia, Spain.

Article Synopsis
  • - Cardiac channelopathies are inherited syndromes in children that can lead to serious heart issues like malignant arrhythmias and sudden cardiac death, often diagnosed through a 12-lead electrocardiogram.
  • - Identifying these conditions early is important, especially since symptoms may only appear after triggers like exercise or emotions; complementary tests, including genetic analysis, can help diagnose and assess risks in affected families.
  • - Advances in genetic testing and sequencing technologies are aiding in discovering new genetic causes and refining the interpretation of uncertain genetic variants, crucial for effective risk management in pediatric patients.
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The TeLeo Program offers a free-access 2-year online learning program to support fellowship programs in pediatric oncology, enhance networking opportunities, and facilitate the exchange of context-specific, educational content within the pediatric oncology community in training in Latin America. In its first edition beginning in 2021, 185 fellows from 40 centers in 12 Latin American countries were enrolled. Additional courses for other healthcare professionals related to oncology in the region were produced to further support the program.

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The ESPA Pain Management Ladder Initiative is a clinical practice advisory based upon expert consensus supported by the current literature to help ensure a basic standard of perioperative pain management for all children. In 2018 the perioperative pain management of six common pediatric surgical procedures was summarised. The current Pain Management Ladder recommendations focus on five more complex pediatric surgical procedures and suggest basic, intermediate, and advanced pain management methods.

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Introduction: poor dietary habits and lack of physical activity are associated with non-communicable diseases. A healthy diet during childhood is important for the prevention of these diseases in the short and long term. Objectives: improve eating habits, promote the Mediterranean diet (MD) and prevent and/or reverse overweight and obesity in children aged 3 to 12 years.

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Therapeutic Drug Monitoring of antibiotic and antifungical drugs in paediatric and newborn patients. Consensus Guidelines of the Spanish Society of Hospital Pharmacy (SEFH) and the Spanish Society of Paediatric Infectious Diseases (SEIP).

An Pediatr (Engl Ed)

September 2024

Grupo de trabajo de Atención Farmacéutica en Enfermedades Infecciosas de la Sociedad Española de Farmacia Hospitalaria (SEFH), Madrid, Spain; Servicio de Farmacia Hospitalaria, Hospital Universitari Vall d'Hebron, Passeig de la Vall d'Hebron, 119-129, 08035 Barcelona, Spain; Grupo de investigación "Infección e inmunidad en el paciente pediátrico", Vall d'Hebron Institut de Recerca, Edifici Collserola Hospital Universitari Vall d'Hebron, Pg. de la Vall d'Hebron, 129, 08035 Barcelona, Spain. Electronic address:

Therapeutic monitoring of antibiotics and antifungals based on pharmacokinetic and pharmacodynamic (PK/PD) parameters is a strategy increasingly used for the optimization of therapy to improve efficacy, reduce the occurrence of toxicities, and prevent the selection of antimicrobial resistance, particularly in vulnerable patients including neonates and the critical or immunocompromised paediatric host. In neonates and children, infections account for a high percentage of hospital admissions, and anti-infectives are the most used drugs. However, paediatric PK/PD studies and the evidence regarding the efficacy and safety of some newly marketed antibiotics and antifungals-usually used off-label in paediatrics-to determine the optimal drug dosage regimens are limited.

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Article Synopsis
  • Immune effector cell-associated hematotoxicity (ICAHT) is a common complication in CAR-T cell therapy, leading to significant thrombocytopenia in about one-third of patients, often necessitating platelet transfusions.
  • Eltrombopag, a thrombopoietin receptor agonist, was evaluated in a study involving 38 patients from 10 hospitals in Spain who developed platelet transfusion dependence after CAR-T treatment.
  • The results showed that 76.3% of patients achieved platelet transfusion independence after using eltrombopag, with additional improvements noted in neutrophil and red blood cell counts, and no serious side effects reported.
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Objective: Congenital viral infection may result in fetal anemia and thrombocytopenia. While intrauterine blood transfusions (IUTs) are more commonly performed for Rh alloimmunization, reports using IUT for infection have varying success. Our primary objective was to characterize the outcomes of patients undergoing IUT for infectious etiologies at our center compared with Rh disease.

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[Translated article] Therapeutic Drug Monitoring of antibiotic and antifungical drugs in paediatric and newborn patients. Consensus Guidelines of the Spanish Society of Hospital Pharmacy (SEFH) and the Spanish Society of Paediatric Infectious Diseases (SEIP).

Farm Hosp

September 2024

Grupo de trabajo de Atención Farmacéutica en Enfermedades Infecciosas de la Sociedad Española de Farmacia Hospitalaria (SEFH), Madrid, Spain; Servicio de Farmacia Hospitalaria, Hospital Universitari Vall d'Hebron, Passeig de la Vall d'Hebron, 119-129, 08035 Barcelona, Spain; Grupo de investigación "Infección e inmunidad en el paciente pediátrico", Vall d'Hebron Institut de Recerca, Edifici Collserola Hospital Universitari Vall d'Hebron, Pg. de la Vall d'Hebron, 129, 08035 Barcelona, Spain. Electronic address:

Therapeutic monitoring of antibiotics and antifungals based on pharmacokinetic and pharmacodynamic (PK/PD) parameters is a strategy increasingly used for the optimization of therapy to improve efficacy, reduce the occurrence of toxicities, and prevent the selection of antimicrobial resistance, particularly in vulnerable patients including neonates and the critical or immunocompromised paediatric host. In neonates and children, infections account for a high percentage of hospital admissions, and anti-infectives are the most used drugs. However, paediatric PK/PD studies and the evidence regarding the efficacy and safety of some newly marketed antibiotics and antifungals-usually used off-label in paediatrics-to determine the optimal drug dosage regimens are limited.

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Spanish Society of Hospital Pharmacy and the Spanish Society of Pediatric Infectious Diseases (SEFH-SEIP) National Consensus Guidelines for therapeutic drug monitoring of antibiotic and antifungal drugs in pediatric and newborn patients.

Farm Hosp

September 2024

Grupo de Trabajo de Atención Farmacéutica en Enfermedades Infecciosas, Sociedad Española de Farmacia Hospitalaria (SEFH), España; Servicio de Farmacia Hospitalaria, Hospital Universitari Vall d'Hebron, Barcelona, España; Grupo de Investigación Infección e Inmunidad en el Paciente Pediátrico, Vall d'Hebron Institut de Recerca, Hospital Universitario Vall d'Hebron, Barcelona, España.

Therapeutic monitoring of antibiotics and antifungals based on pharmacokinetic and pharmacodynamic parameters, is a strategy increasingly used for the optimization of therapy to improve efficacy, reduce the occurrence of toxicities, and prevent the selection of antimicrobial resistance, particularly in vulnerable patients including neonates and the critical or immunocompromised host. In neonates and children, infections account for a high percentage of hospital admissions and anti-infectives are the most used drugs. However, pediatric pharmacokinetic and pharmacodynamic studies and the evidence regarding the efficacy and safety of some newly marketed antibiotics and antifungals -usually used off-label in pediatrics- to determine the optimal drug dosage regimens are limited.

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Introduction: Less-invasive surfactant administration (LISA) is associated with better respiratory outcomes in preterm infants with respiratory distress syndrome. However, mechanical ventilation (MV) shortly after the LISA procedure has been related to lower survival. This study aimed to analyze the trends and main predictors of continuous positive airway pressure (CPAP) failure after LISA.

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Article Synopsis
  • - Spain introduced universal RSV prophylaxis in the 2023-2024 season, allowing all newborns to receive nirsevimab, particularly benefiting infants under 6 months through extended immunization efforts.
  • - A study in 15 pediatric emergency departments showed significant decreases in respiratory infections: a 57.7% drop in lower respiratory tract infections and a 63.1% reduction in hospital and PICU admissions related to acute bronchiolitis.
  • - The findings indicate that nirsevimab is highly effective in protecting infants from RSV, with extended catch-up immunization yielding the best results, although cost-effectiveness remains a consideration.
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Background: Insular epilepsy is a well-known cause of drug-resistant epilepsy (DRE) in the pediatric population. It can be a source of surgical epilepsy treatment failures when not ruled out pre-operatively. Non-invasive methods often provide limited information about its existence, being the invasive methods necessary to diagnose it in the vast majority of cases.

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Prevalence of sexually transmitted infections after penetrative rape of pediatric patients.

Emergencias

June 2024

Influencia del entorno en el bienestar del niño y del adolescente, Institut de Recerca Sant Joan de Déu, Esplugues de Llobregat, Barcelona, España. Servicio de Urgencias, Hospital Sant Joan de Déu Barcelona, Esplugues de Llobregat, Barcelona, España. Universidad de Barcelona, Barcelona, España.

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Little is known about language development after late-to-moderate premature birth, the most significant part of prematurity worldwide. We examined minimal-pair word-learning skills in 18 eighteen-month-old healthy full-term (mean gestational age [GA] at birth = 39.6 weeks; 7 males; 100% Caucasian) and 18 healthy late-to-moderate preterm infants (mean GA at birth 33.

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GABAergic neuronal lineage development determines clinically actionable targets in diffuse hemispheric glioma, H3G34-mutant.

Cancer Cell

August 2024

Department of Pediatric Oncology, Dana-Farber Boston Children's Cancer and Blood Disorders Center, Boston, MA 02215, USA; Broad Institute of Harvard and MIT, Cambridge, MA 02142, USA. Electronic address:

Article Synopsis
  • Diffuse hemispheric gliomas, specifically H3G34R/V-mutant, are aggressive brain tumors with no current targeted therapies and come from neural precursor cells.
  • Researchers found that these tumors display developmental patterns similar to healthy brain interneurons and identified key genes that these tumor cells depend on, especially CDK6.
  • Targeting CDK6 with inhibitors showed promising results in reducing tumor growth and improving survival in experimental models, with one patient showing a significant response to treatment.
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