Analysis and prediction of condylar resorption following orthognathic surgery.

Condylar resorption is a feared complication of orthognathic surgery. This study investigated condylar resorption in a cohort of 200 patients This allowed for a powerful update on incidence and risk factors. 9.

EDENT1FI Master Protocol for screening of presymptomatic early-stage type 1 diabetes in children and adolescents.

Introduction: The identification of type 1 diabetes at an early presymptomatic stage has clinical benefits. These include a reduced risk of diabetic ketoacidosis (DKA) at the clinical manifestation of the disease and a significant reduction in clinical symptoms. The European action for the Diagnosis of Early Non-clinical Type 1 diabetes For disease Interception (EDENT1FI) represents a pioneering effort to advance early detection of type 1 diabetes through public health screening.

The Delicate Balancing of Pros and Cons in the Surgical Management of Hyperparathyroidism in a Young Female with Germline Variant in the CDC73 Gene.

Hyperparathyroidism-jaw tumor syndrome is a rare form of syndromic primary hyperparathyroidism. We describe a young female with a history of common precursor B acute lymphoblastic leukaemia who was diagnosed with overt primary hyperparathyroidism due to a pathogenic CDC73 variant (c.25C > T).

Basic Science and Pathogenesis.

Background: Limbic-predominant age-related TDP-43 encephalopathy (LATE) has been recently recognized as a cause of dementia in the elderly. LATE and Alzheimer's disease (AD) share similar clinical presentations, and their neuropathological changes-LATE-NC and ADNC-commonly co-occur in the brains of individuals with dementia. Frontotemporal degeneration (FTLD-TDP) represents another group of TDP-43-associated neurodegenerative diseases.

Basic Science and Pathogenesis.

Background: Published data have highlighted associations between Alzheimer's disease (AD) susceptibility loci and AD-related brain changes. The amyloid imaging to prevent AD (AMYPAD) consortium is a European collaboration consisting of several parent cohorts, four of which had raw genotype array data available. We sought to integrate and harmonise the genetic data, calculate AD polygenic risk scores (PRS), and investigate their association with global amyloid deposition.

Fight or Flight: Emergency Health Care Workers' Willingness to Work during Crises and Disasters. A Cross-Sectional Multicenter Study in the Netherlands.

Objective: Expanding staff levels is a strategy for hospitals to increase their surge capacity. The aim of this study was to evaluate whether emergency health care workers (HCWs) are willing to work during crises or disasters, and which working conditions influence their decisions.

Methods: HCWs in the emergency departments (EDs) and intensive care units (ICUs) of 5 Dutch hospitals were surveyed about various disaster scenarios.

Anti-TNF Non-Response in Ulcerative Colitis: Correcting for Mucosal Drug Exposure Reveals Distinct Cytokine Profiles.

Introduction: It remains unclear why up to 30% of ulcerative colitis patients (UC) do not respond to tumor necrosis factor inhibitors (TNFi). Validated biomarkers for (non-)response ((N)R) are lacking. Most studies investigating underlying mechanisms do not differentiate between pharmacokinetic and inflammatory mechanisms.

Prediction of antimicrobial susceptibility of pneumococci based on whole-genome sequencing data: a direct comparison of two genomic tools to conventional antimicrobial susceptibility testing.

Determination of antimicrobial resistance (AMR) in pneumococcal isolates is important for surveillance purposes and in a clinical context. Antimicrobial susceptibility testing (AST) of pneumococci is complicated by the need for exact minimal inhibitory concentrations (MICs) of beta-lactam antibiotics. Two next-generation sequencing (NGS) analysis tools have implemented the prediction of AMR in their analysis workflow, including the prediction of MICs: Pathogenwatch (https://pathogen.

Successful reversal of intestinal obstruction by PRRT: two case reports and literature review of PRRT in Small Intestinal Neuroendocrine Tumors with mesenteric fibrosis.

Small intestinal neuroendocrine tumors (SI-NETs) typically follow an indolent disease course and are often accompanied by mesenteric lymph node metastases upon diagnosis. These tumors can incite a fibroblastic reaction within the mesenteric root. Here, we present two cases of patients with symptomatic small bowel obstruction due to such mesenteric involvement.

Treatment patterns and outcomes of patients with complex Crohn's perianal fistula in five European countries: the PREFACE study.

Background And Study Aims: Crohn's disease (CD) is often complicated by perianal fistulas or abscesses that worsen patient's quality of life, with 52-88% of CD-related perianal fistulas (CPFs) being complex. This retrospective study describes sociodemographic characteristics, treatment patterns and outcomes of patients with complex CPFs in five European countries.

Patients And Methods: PREFACE is a multi-national medical chart review study of CD patients who started medical or surgical treatment for a new episode of complex CPFs between September 2011 and September 2014 (index date).

Neuromonitoring modalities predicting neurological impairment in pediatric congenital heart disease: a systematic review.

Unlabelled: The increased risk of neurodevelopmental impairment in children with congenital heart disease (CHD) has been established, but the search for targeted neurological predictors of adverse outcome is ongoing. This systematic review reports on the utility of three functional neuromonitoring modalities, Near-infrared Spectroscopy (NIRS), electroencephalography (EEG) and biochemical biomarkers, in predicting either clinical neurodevelopmental outcome or structural brain abnormalities after pediatric CHD surgery. Medline, Embase, CENTRAL, Web of Science, clinicaltrials.

Overlap Syndrome (COPD and OSA): A Treatable Trait for Triple Treatment?

The coexistence of chronic obstructive pulmonary disease (COPD) and obstructive sleep apnea (OSA) in the same patient is referred to as overlap syndrome (OS). Patients with OS suffer more frequently from cardiovascular disease (CVD) and carry a higher risk of COPD-related exacerbations than patients with COPD alone, especially when OSA is left untreated. Based on recent evidence, triple therapy, namely inhaled corticosteroid/long-acting muscarinic antagonist/long-acting beta-agonist (ICS-LABA-LAMA), is a treatment strategy in COPD patients with a history of exacerbations and/or CVD comorbidity.

Validation of a novel tool for automated tooth modelling by fusion of CBCT-derived roots with the respective IOS-derived crowns.

Objectives: To validate a novel artificial intelligence (AI)-based tool for automated tooth modelling by fusing cone beam computed tomography (CBCT)-derived roots with corresponding intraoral scanner (IOS)-derived crowns.

Methods: A retrospective dataset of 30 patients, comprising 30 CBCT scans and 55 IOS dental arches, was used to evaluate the fusion model at full arch and single tooth levels. AI-fused models were compared with CBCT tooth segmentation using point-to-point surface distances-reported as median surface distance (MSD), root mean square distance (RMSD), and Hausdorff distance (HD)- alongside visual assessments.

Hemophilia B Leyden: Characteristics and natural history in the PedgNet Registry.

Background: A unique form of Hemophilia B (HB) is HB Leyden. We evaluated the International PedNet Registry database to explore the natural history of HB Leyden, investigate genotype-phenotype associations and guide clinical decision-making.

Objectives: To assess the association between genetic variants, endogenous factor (FIX) levels over time, treatment and bleeding phenotype in children with HB Leyden.

Assessment of the efficacy of palliative sedation in advanced cancer patients by evaluating discomfort levels: a prospective, international, multicenter observational study.

Background: Palliative sedation involves the intentional proportional lowering of the level of consciousness in patients with life-limiting disease who are experiencing refractory suffering. The efficacy of palliative sedation needs to be monitored to ensure patient comfort. The aim of this study was to evaluate the efficacy using discomfort levels combined with sedation/agitation levels.

Fenfluramine treatment for Dravet syndrome: Long term real-world analysis demonstrates safety and reduced health care burden.

Objective: Fenfluramine (FFA), stiripentol (STP), and cannabidiol (CBD) are approved add-on therapies for seizures in Dravet syndrome (DS). We report on the long-term safety and health care resource utilization (HCRU) of patients with DS treated with FFA under an expanded access program (EAP).

Methods: A cohort of 124 patients received FFA for a median of 2.

Polygenic score distribution differences across European ancestry populations: implications for breast cancer risk prediction.

Background: The 313-variant polygenic risk score (PRS) provides a promising tool for clinical breast cancer risk prediction. However, evaluation of the PRS across different European populations which could influence risk estimation has not been performed.

Methods: We explored the distribution of PRS across European populations using genotype data from 94,072 females without breast cancer diagnosis, of European-ancestry from 21 countries participating in the Breast Cancer Association Consortium (BCAC) and 223,316 females without breast cancer diagnosis from the UK Biobank.

Inhibiting CFTR through inh-172 in primary neutrophils reveals CFTR-specific functional defects.

The lungs of people with cystic fibrosis (PwCF) are characterized by recurrent bacterial infections and inflammation. Infections in cystic fibrosis (CF) are left unresolved despite excessive neutrophil infiltration. The role of CFTR in neutrophils is not fully understood.

Longitudinal synaptic loss versus tau Braak staging in amnestic mild cognitive impairment.

Introduction: The longitudinal progression of synaptic loss in Alzheimer's disease (AD) and how it is affected by tau pathology remains poorly understood.

Methods: Thirty patients with amnestic mild cognitive impairment (aMCI) and 26 healthy controls underwent cognitive evaluations and tau, synaptic vesicle protein 2A (SV2A), and amyloid positron emission tomography. Twenty-one aMCI underwent 2-year follow-up (FU) investigations.

Development of a New Score Based on Image Defined Risk Factors to Standardize Surgical Risk in Neuroblastoma Resection - A SIOPEN Collaborative Study.

Background And Aims: Image Defined Risk Factors (IDRFs) assess surgical risk in neuroblastoma (NB) and guide neoadjuvant therapy. Despite chemotherapy IDRFs may persist in 70 % of cases. Several studies have suggested that not all IDRFs hold equal significance and that the presence of an IDRF does not inherently signify unresectability.