Low-dose lung CT: Optimizing diagnostic radiation dose - A phantom study.

Background/purpose: To investigate a quantitative method for assessing image quality of low dose lung computed tomography (CT) and find the lowest exposure dose providing diagnostic images.

Methods: Axial volumetric lung CT acquisitions (256 slice scanner) were performed on three different sized anthropomorphic phantoms at different dose levels. The maximum steepness of sigmoid curves fitted to line density profiles was measured at lung-to-pleura interfaces.

Smooth muscle spheroids: Superior building blocks for bladder tissue engineering.

Background: Congenital bladder disorders in children necessitate innovative approaches for bladder tissue regeneration, aiming to minimize complications associated with conventional therapies. This study focused on generating a cell-seeded scaffold using superior smooth muscle cells (SMCs) by exploring the potential of smooth muscle cell spheroids (3D SMCs) compared to conventionally cultured SMCs (2D SMCs) for bladder tissue engineering. Additionally, adipose-derived stem cells (ADSCs) were investigated for their impact on SMC proliferation and maturation, and pre-differentiated smooth muscle-like ADSCs (pADSCs) for their potential as alternative cell source.

European Society of Organ Transplantation (ESOT) Consensus Statement on Machine Perfusion in Cardiothoracic Transplant.

The machine perfusion (MP) of transplantable grafts has emerged as an upcoming field in Cardiothoracic (CT) transplantation during the last decade. This technology carries the potential to assess, preserve, and even recondition thoracic grafts before transplantation, so it is a possible game-changer in the field. This technology field has reached a critical turning point, with a growing number of publications coming predominantly from a few leading institutions, but still need solid scientific evidence.

Exploring ketogenic diet resistance in glucose transporter type 1 deficiency syndrome: A comprehensive review and critical appraisal.

Glucose transporter type 1 deficiency syndrome (GLUT1DS) commonly presents with early-onset epilepsy that often resists conventional pharmacological treatment. Ketogenic diet therapy (KDT) is the preferred approach to address the underlying metabolic anomaly. However, a subset of GLUT1DS patients presents resistance to KDT, with the causes remaining elusive.

Clinical characteristics and outcome of central nervous system tumors harboring NTRK gene fusions.

Purpose: TRK fusions are detected in less than 2% of central nervous system tumors. There are limited data on the clinical course of affected patients.

Experimental Design: We conducted an international retrospective cohort study of patients with TRK fusion-driven CNS tumors.

Mapping challenges in the accessibility of treatment products for urea cycle disorders: A survey of European healthcare professionals.

Current management guidelines for urea cycle disorders (UCDs) offer clear strategies, incorporating both authorized and non-authorized medicinal products (including intravenous formulations and products regulated as food). These varying product categories are subject to specific accessibility challenges related to availability, reimbursement, and pricing. The aim of this study is to identify potential obstacles to optimal UCD treatment implementation in European clinical practice.

Effects of Biologics on Temporomandibular Joint Inflammation in Juvenile Idiopathic Arthritis.

Objective: This prospective study investigates the efficacy of biologics in combination with methotrexate (MTX) or leflunomide (LEF) on juvenile idiopathic arthritis (JIA)-related temporomandibular joint (TMJ) arthritis measured by magnetic resonance imaging (MRI)-based inflammation score and deformity score.

Methods: A prospective, single-center observational cohort study of 18 consecutive patients was performed between September 2018 and April 2023. Inclusion criteria were (1) diagnosis of JIA, (2) MRI-verified TMJ arthritis leading to treatment with tumor necrosis factor inhibitor (TNFi), (3) MRI at 6 months and 24 months after treatment initiation, and (4) clinical follow-up together with an MRI by a pediatric rheumatologist and an orthodontist.

A deep learning approach for automated scoring of the Rey-Osterrieth complex figure.

Memory deficits are a hallmark of many different neurological and psychiatric conditions. The Rey-Osterrieth complex figure (ROCF) is the state-of-the-art assessment tool for neuropsychologists across the globe to assess the degree of non-verbal visual memory deterioration. To obtain a score, a trained clinician inspects a patient's ROCF drawing and quantifies deviations from the original figure.

COMPARABLE OUTCOMES AFTER BUSULFAN- OR TREOSULFAN-BASED CONDITIONING FOR ALLO-HSCT IN CHILDREN WITH ALL-RESULTS OF FORUM.

The superiority of TBI-based versus chemotherapy-conditioning for allo-HSCT in children with ALL has been established in the international, prospective phase-III FORUM study (#NCT01949129), randomizing 417 patients ≤ 18 years at diagnosis (4-21 years at HSCT) in CR, transplanted from HLA-matched sibling or unrelated donors. Due to the unavailability of TBI in some regions and to accommodate individual contraindications, this study reports the pre-specified comparison of outcomes of patients receiving busulfan-based (BU) or treosulfan-based (TREO) regimens from 2013 to 2018. 180 and 128 patients (median age 9.

Serum Sodium Concentration During Arginine Vasopressin Infusion in Critically Ill Children.

Background: Intravenous arginine vasopressin is increasingly used for the treatment of critically ill children. It bears the risk of hyponatraemia with potential severe long-term sequelae, but data on hyponatraemia as a side effect of continuous vasopressin infusion for paediatric intensive care patients is scarce.

Methods: In this retrospective analysis performed at a tertiary care paediatric intensive care unit with 2000 annual admissions, patients were included if they were treated with intravenous vasopressin between 2016 and 2022.

Hen's egg white allergy in adults leading to strong impairment of quality of life.

In adulthood, hen's egg white allergy (EWA) is a rare condition and rising in prevalence. Typically, EWA begins in early childhood and resolves at school age. Persistence into adulthood or newly onset of the allergy has been reported, but scientific data is scarce.

Towards a Risk-Based Follow-Up Surveillance Imaging Schedule for Children and Adolescents with Low-Grade Glioma.

The frequency and duration of imaging surveillance in children and adolescents with pediatric low-grade gliomas (pLGGs) aims for the early detection of recurrence or progression. Although surveillance of pLGGs is performed routinely, it is not yet standardized. The aim of the current review is to provide a comprehensive synthesis of published studies regarding the optimal frequency, intervals, and duration of surveillance.

Inotropes for the prevention of low cardiac output syndrome and mortality for paediatric patients undergoing surgery for congenital heart disease: a network meta-analysis.

Background: Paediatric patients undergoing surgery for congenital heart disease (CHD) are at risk for postoperative low cardiac output syndrome (LCOS) and mortality. LCOS affects up to 25% of children after heart surgery. It consists of reduced myocardial function and increases postoperative morbidity, prolongs mechanical ventilation, and lengthens the duration of intensive care unit (ICU) stay.

Neonatal transport: parental needs and potential for improvement - a single-center cross-sectional study.

Objective: Neonates with medical problems need transfer from the place of birth to a neonatal (intensive care) unit after birth by a specialized neonatal transport team. Neonatal transport is accompanied by a high emotional burden for the families due to spatial separation, uncertainty and care for the neonate. This survey of parents' needs was a quality control project of the neonatal transport team of the University Children's Hospital Zurich, Switzerland.

Early nasal microbiota and subsequent respiratory tract infections in infants with cystic fibrosis.

Background: Respiratory tract infections (RTIs) drive lung function decline in children with cystic fibrosis (CF). While the respiratory microbiota is clearly associated with RTI pathogenesis in infants without CF, data on infants with CF is scarce. We compared nasal microbiota development between infants with CF and controls and assessed associations between early-life nasal microbiota, RTIs, and antibiotic treatment in infants with CF.

Loss of tissue-type plasminogen activator causes multiple developmental anomalies.

Hydrocephalus and Dandy-Walker malformation are amongst the most common congenital brain anomalies. We identified three consanguineous families with both obstructive hydrocephalus and Dandy-Walker malformation. To understand the molecular basis of these anomalies, we conducted genome-wide sequencing in these families.

Evidence for interaction of 5,10-methylenetetrahydrofolate reductase (MTHFR) with methylenetetrahydrofolate dehydrogenase (MTHFD1) and general control nonderepressible 1 (GCN1).

5,10-Methylenetetrahydrofolate reductase (MTHFR) is a folate cycle enzyme required for the intracellular synthesis of methionine. MTHFR was previously shown to be partially phosphorylated at 16 residues, which was abrogated by conversion of threonine 34 to alanine (T34A) or truncation of the first 37 amino acids (i.e.

Safety of oral immunotherapy for cashew nut and peanut allergy in children - a retrospective single-centre study.

Aim Of The Study: Oral immunotherapy (OIT) is increasingly used for the treatment of childhood food allergies, with limited data available on cashew nut OIT. This real-life study investigated the safety and feasibility of cashew nut OIT, comparing it with peanut OIT, with a focus on the up-dosing process.

Methods: We analysed cashew nut (n = 24) and peanut (n = 38) OIT cases with treatment initiated between 2018 and 2022 at the University Children's Hospital Basel.

Vaccine safety in children with genetically confirmed mitochondrial disease.

We here explore adverse events following immunization (AEFI) in children with mitochondrial disease (MD) recruited from two expertise centers in Austria (SALK) and The Netherlands (RUMC). Parents completed a questionnaire on the type of immunizations received and AEFI in a post-vaccination exposure period of seven days. 95 individuals were invited to this study, of whom 30 (median age 13.

Effectiveness of Game-Based Training of Selective Voluntary Motor Control in Children With Upper Motor Neuron Lesions: Randomized Multiple Baseline Design Study.

Background: Selective voluntary motor control (SVMC) is the ability to control joint movements independently. Impairments in SVMC can affect functional activities, but only a few interventions directly target SVMC. Therefore, we developed a game-based intervention for children with upper motor neuron lesions to improve SVMC.