Neonatal Giant Cardiac Fibroma With Coronary Involvement: Successful Surgical Treatment.

Cardiac fibroma is the second most common childhood cardiac tumor. Coronary arteries are typically unaffected. A 5-month-old infant received a diagnosis of giant cardiac fibroma with coronary involvement.

Implementing digital respiratory technologies for people with respiratory conditions: A protocol for a scoping review.

The value of 'data-enabled', digital healthcare is evolving rapidly, as demonstrated in the COVID-19 pandemic, and its successful implementation remains complex and challenging. Harmonisation (within/between healthcare systems) of infrastructure and implementation strategies has the potential to promote safe, equitable and accessible digital healthcare, but guidance for implementation is lacking. Using respiratory technologies as an example, our scoping review process will capture and review the published research between 12th December 2013 to 12th December 2023.

Digital twins for chronic lung diseases.

Digital twins have recently emerged in healthcare. They combine advances in cyber-physical systems, modelling and computation techniques, and enable a bidirectional flow of information between the physical and virtual entities. In respiratory medicine, progress in connected devices and artificial intelligence make it technically possible to obtain digital twins that allow real-time visualisation of a patient's respiratory health.

The Bronchodilator and Anti-Inflammatory Effect of Long-Acting Muscarinic Antagonists in Asthma: An EAACI Position Paper.

As cholinergic innervation is a major contributor to increased vagal tone and mucus secretion, inhaled long-acting muscarinic antagonists (LAMA) are a pillar for the treatment of chronic obstructive pulmonary disease and asthma. By blocking the muscarinic receptors expressed in the lung, LAMA improve lung function and reduce exacerbations in asthma patients who remained poorly controlled despite treatment with inhaled corticosteroids and long-acting β2 agonists. Asthma guidelines recommend LAMA as a third controller to be added on before the initiation of biologicals.

Artificial intelligence in respiratory care: perspectives on critical opportunities and challenges.

Artificial intelligence (AI) is transforming respiratory healthcare through a wide range of deep learning and generative tools, and is increasingly integrated into both patients' lives and routine respiratory care. The implications of AI in respiratory care are vast and multifaceted, presenting both promises and uncertainties from the perspectives of clinicians, patients and society. Clinicians contemplate whether AI will streamline or complicate their daily tasks, while patients weigh the potential benefits of personalised self-management support against risks such as data privacy concerns and misinformation.

ORPHEE: A Real-World Study on rIX-FP Prophylaxis Use in Adolescent/Adult Patients With Hemophilia B.

Objectives: To assess the real-world efficacy and safety of recombinant factor IX albumin fusion protein (rIX-FP) in patients with hemophilia B (HB) in France.

Methods: Data on dosing frequency, weekly consumption, and bleeds before-and-after switching to rIX-FP, were collected from December 2021 to February 2024. Annualized (spontaneous) bleeding rates [A(s)BRs] were calculated only in patients on prophylaxis with a follow-up ≥ 6 months.

GPATCH11 variants cause mis-splicing and early-onset retinal dystrophy with neurological impairment.

Here we conduct a study involving 12 individuals with retinal dystrophy, neurological impairment, and skeletal abnormalities, with special focus on GPATCH11, a lesser-known G-patch domain-containing protein, regulator of RNA metabolism. To elucidate its role, we study fibroblasts from unaffected individuals and patients carrying the recurring c.328+1 G > T mutation, which specifically removes the main part of the G-patch domain while preserving the other domains.

Definitions and Characteristics of Patient Digital Twins Being Developed for Clinical Use: Scoping Review.

Background: The concept of digital twins, widely adopted in industry, is entering health care. However, there is a lack of consensus on what constitutes the digital twin of a patient.

Objective: The objective of this scoping review was to analyze definitions and characteristics of patient digital twins being developed for clinical use, as reported in the scientific literature.

Myocardial infarction in a 17-year-old patient diagnosed with MPOD II syndrome.

Introduction: Microcephalic osteodysplastic primordial dwarfism (MOPD) syndrome type 2, caused by a mutation in the PCNT gene (21q22.3), is a rare autosomal recessive disorder. Patients present with bone dysplasia, insulin resistance, kidney diseases, and cardiac malformations, making them prone to vascular diseases.

Determinants of Timely Access to Recanalization Treatments and Outcomes in Pediatric Ischemic Stroke.

Background: Timely revascularization in acute arterial ischemic stroke (AIS) is paramount for optimal outcomes. However, factors causing treatment delays in pediatric AIS remain understudied. We investigated determinants affecting the time from symptom onset or last-known-well to the start of recanalization treatment in pediatric AIS.

Radiation-free and injection-free imaging of the paediatric chest using a magnetic resonance imaging protocol including zero time echo sequence (3D-ZTE).

Unlabelled: Computed tomography (CT) is commonly used for paediatric thoracic diseases but involves radiation exposure and often requires intravenous contrast. We evaluated the performance of a magnetic resonance imaging (MRI) protocol including a 3D zero echo time (3D-ZTE) sequence for radiation-free and contrast-free imaging of the paediatric chest. In this prospective, single-centre study, children aged 6-16 years underwent chest CT and MRI within 48 h.

Evaluation of a short training course of chest X-ray interpretation for the diagnosis of paediatric TB.

Background: Chest X-ray (CXR) interpretation is challenging for the diagnosis of paediatric TB. We assessed the performance of a three half-day CXR training module for healthcare workers (HCWs) at low healthcare levels in six high TB incidence countries.

Methods: Within the TB-Speed Decentralization Study, we developed a three half-day training course to identify normal CXR, CXR of good quality and identify six TB-suggestive features.

Infection and Pediatric Arterial Ischemic Stroke Presumably Related to Focal Cerebral Arteriopathy: Data From the COVID-19 Pandemic.

Background: Infection may trigger pediatric arterial ischemic stroke (PAIS), notably when related to focal cerebral arteriopathy. Community- and individual-level nonpharmaceutical interventions during the COVID-19 pandemic resulted in a major decrease in pediatric viral infections. We explored the consequences on the incidence of PAIS.

ERS International Congress 2023: highlights from the Respiratory Clinical Care and Physiology Assembly.

It is a challenge to keep abreast of all the clinical and scientific advances in the field of respiratory medicine. This article contains an overview of laboratory-based science, clinical trials and qualitative research that were presented during the 2023 European Respiratory Society International Congress within the sessions from the five groups of Assembly 1 (Respiratory Clinical Care and Physiology). Selected presentations are summarised from a wide range of topics: clinical problems, rehabilitation and chronic care, general practice and primary care, electronic/mobile health (e-health/m-health), clinical respiratory physiology, exercise and functional imaging.

The immunopathological landscape of human pre-TCRα deficiency: From rare to common variants.

We describe humans with rare biallelic loss-of-function variants impairing pre-α T cell receptor (pre-TCRα) expression. Low circulating naive αβ T cell counts at birth persisted over time, with normal memory αβ and high γδ T cell counts. Their TCRα repertoire was biased, which suggests that noncanonical thymic differentiation pathways can rescue αβ T cell development.

Objectives for algorithmic decision-making systems in childhood asthma: Perspectives of children, parents, and physicians.

Objectives: To identify with children, parents and physicians the objectives to be used as parameters for algorithmic decision-making systems (ADMSs) adapting treatments in childhood asthma.

Methods: We first conducted a qualitative study based on semi-structured interviews to explore the objectives that children aged 8-17 years, their parents, and their physicians seek to achieve when taking/giving/prescribing a treatment for asthma. Following the grounded theory approach, each interview was independently coded by two researchers; reconciled codes were used to assess code frequency, categories were defined, and the main objectives identified.

Development of Collateral Vessels after Anterior Circulation Large Vessel Occlusion in Pediatric Arterial Ischemic Stroke Relates to Stroke Etiology: A Longitudinal Study.

Background And Purpose: The characteristics of large vessel occlusion (LVO) in the acute phase of pediatric arterial ischemic stroke and their natural history according to stroke etiology are poorly explored. This studied aimed at describing the prevalence and the radiological evolution of LVO in pediatric AIS.

Materials And Methods: This single-center retrospective study included consecutive non-neonate children with acute arterial ischemic stroke, intracranial proximal LVO in the anterior circulation (MCA, anterior cerebral artery, and/or ICA), and clinical and imaging follow-up for at least 18 months, during a 9-year period.

Inflammatory rebound and postinfectious inflammatory response in children with pleural infection: A single-center retrospective study.

Introduction: As pleural inflammation plays a central role in pleural infection (PI), corticosteroids are increasingly being considered as a potential therapy. However, the timing of treatment and the identification of patients who might benefit most remain unresolved. The aim of this study was therefore to investigate the inflammatory trajectories of children with PI.

Pharmacokinetic of ceftazidime-avibactam in a critically ill patient under high-volume continuous venovenous haemodiafiltration: A first paediatric case report.

Ceftazidime-avibactam is a novel cephalosporin/B-lactamase inhibitor developed in the context of increasing resistance. This case reports the pharmacokinetics of ceftazidime-avibactam in a critically ill child under continuous renal replacement (CRRT) therapy for fluid overload. The patient was a 6-month-old female with sepsis due to bloodstream infection to Stenotrophomonas maltophilia following stem cell transplantation for severe combined immunodeficiency.