Standardized Response Assessment in Patients with Advanced Cholangiocarcinoma Treated with Personalized Therapy.

: Current guidelines recommend Cisplatin/Gemcitabine/Durvalumab as first-line treatment for inoperable or recurrent cholangiocarcinoma (CCA). Molecular tumor boards (MTB) have the expertise to support organ-specific tumor boards with evidence-based treatment recommendations for subsequent lines of treatment, based on genomic tumor data and scientific evidence. This study evaluates the adoption of an MTB at a comprehensive cancer center in Germany and whether actionable genetic alterations are associated with specific imaging phenotypes.

Multidisciplinary quantitative and qualitative assessment of IDH-mutant gliomas with full diagnostic deep learning image reconstruction.

Unlabelled: Diagnostic accuracy and therapeutic decision-making for IDH-mutant gliomas in tumor board reviews are based on MRI and multidisciplinary interactions.

Materials And Methods: This study explores the feasibility of deep learning-based reconstruction (DLR) in MRI for IDH-mutant gliomas. The research utilizes a multidisciplinary approach, engaging neuroradiologists, neurosurgeons, neuro-oncologists, and radiotherapists to evaluate qualitative aspects of DLR and conventional reconstructed (CR) sequences.

Adequate post-ischemic reperfusion of the mouse brain requires endothelial NFAT5.

Severity and outcome of strokes following cerebral hypoperfusion are significantly influenced by stress responses of the blood vessels. In this context, brain endothelial cells (BEC) regulate inflammation, angiogenesis and the vascular resistance to rapidly restore perfusion. Despite the relevance of these responses for infarct volume and tissue recovery, their transcriptional control in BEC is not well characterized.

Pediatric lymphomas: overview and diagnostic challenges.

Only 10% of new lymphoma diagnoses in the USA occur in children < 15 years. Although the same diagnostic criteria apply to both adult and pediatric lymphomas, there are important differences in some lymphoma subtypes. These differences are recognized by the World Health Organization (WHO) with the recent 2022 classification of pediatric tumors including pediatric hematopoietic tumors.

Platelet spleen tyrosine kinase is a key regulator of anti-PF4 antibody-induced immunothrombosis.

Vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare but serious prothrombotic adverse event following vaccination with adenovector-based COVID-19 vaccines. Laboratory findings indicate that anti-platelet factor 4 (PF4) immunoglobulin G antibodies are the causing factor for the onset of thromboembolic events in VITT. However, molecular mechanisms of cellular interactions, signaling pathways and involvement of different cell types in VITT antibody-mediated thrombosis are not fully understood.

Soft tissue tumor imaging in adults: European Society of Musculoskeletal Radiology-Guidelines 2024: imaging immediately after neoadjuvant therapy in soft tissue sarcoma, soft tissue tumor surveillance, and the role of interventional radiology.

Objectives: An update of the first European Society of Musculoskeletal Radiology (ESSR) consensus on soft tissue tumor imaging in 2015 became necessary due to technical advancements, further insights into specific entities, and the revised WHO classification (2020) and AJCC staging system (2017). The third part of the revised guidelines covers algorithms and techniques beyond initial imaging: (1) Imaging after neoadjuvant therapy in soft tissue sarcoma, (2) sarcoma surveillance, and (3) special aspects, including surveillance of non-malignant entities and the role of interventional radiology.

Materials And Methods: A validated Delphi method based on peer-reviewed literature was used to derive consensus among a panel of 46 specialized musculoskeletal radiologists from 12 European countries.

Discrepancies in Subjective Perceptions of Hydrocephalus Management and Self-Reported Outcomes.

: Despite surgical interventions with advances in endoscopic procedures as well as shunt technologies, the quality of life in patients with hydrocephalus can be poor. Clinical experience suggests discrepancies between objective measures of treatment success and subjective patient satisfaction. With this study, we retrospectively investigated patients' knowledge of their treatment as well as their satisfaction with received interventions.

Challenges and Choices in Breastfeeding Healthy, Sick and Preterm Babies: Review.

Although breastfeeding is associated with many health-related benefits for both mothers and children, the WHO recommendation for exclusive breastfeeding is not achieved by the majority in any WHO region. This paper aims to present the current state of research on challenges and choices in breastfeeding healthy, sick and preterm babies. The research was organized as a systematic search in PubMed and the study was performed as a narrative review after applying the PRISMA protocol.

Only Infant -Rearranged Leukemia Is Susceptible to an Inhibition of Polo-like Kinase 1 (PLK-1) by Volasertib.

-rearranged (r) leukemia is characterized by a poor prognosis. Depending on the cell of origin, it differs in the aggressiveness and therapy response. For instance, in adults, volasertib blocking Polo-like kinase 1 (PLK-1) exhibited limited success.

Coming out of the Integrative Oncology Comfort Zone: Addressing Healthcare Providers' Wartime-Related Concerns.

Study Objective: To assess the impact of a personalized integrative medicine (IM) intervention on healthcare providers (HCPs) expressing war-related emotional/spiritual and physical concerns.

Methods: Physicians, nurses, para-medical and other HCPs from 5 hospital departments in northern Israel underwent IM treatments provided by IM-trained practitioners working in integrative oncology (IO) care settings. The two main HCP-reported concerns were scored (from 0 to 6) before and following the intervention using the Measure Yourself Concerns and Wellbeing questionnaire.

Implementation of the new DGRh S2e guideline on diagnostics and treatment of adult-onset Still's disease in Germany : Implications for clinical practice in rheumatology.

Background: Adult-onset Still's disease (AOSD) is a rare autoinflammatory disease. Since it can lead to variable organ involvement, including life-threatening complications, and due to newly available therapeutic approaches, the German Society for Rheumatology and Clinical Immunology (Deutsche Gesellschaft für Rheumatologie und klinische Immunologie; DGRh) issued a newly developed S2e guideline in December 2022.

Objective: This study aims to investigate the influence of the new guideline on the diagnosis, management, and outcomes of AOSD.

Artificial intelligence-based rapid brain volumetry substantially improves differential diagnosis in dementia.

Introduction: This study evaluates the clinical value of a deep learning-based artificial intelligence (AI) system that performs rapid brain volumetry with automatic lobe segmentation and age- and sex-adjusted percentile comparisons.

Methods: Fifty-five patients-17 with Alzheimer's disease (AD), 18 with frontotemporal dementia (FTD), and 20 healthy controls-underwent cranial magnetic resonance imaging scans. Two board-certified neuroradiologists (BCNR), two board-certified radiologists (BCR), and three radiology residents (RR) assessed the scans twice: first without AI support and then with AI assistance.

Treatment of Pediatric, Adolescent, and Young Adult Patients With Fusion-Positive Alveolar Rhabdomyosarcoma Infiltrating Regional Lymph Nodes in the European CWS-2002P and RMS 2005 Studies and the Soft Tissue Sarcoma Registry.

Background: Patients with alveolar rhabdomyosarcoma (ARMS) with regional lymph node involvement (N1) are defined as "very-high-risk rhabdomyosarcoma" in Europe. Different chemotherapy regimens were used in European study protocols.

Methods: Patients with FOXO1 fusion-positive N1 ARMS registered in the CWS-2002P study, the EpSSG RMS 2005 study, and SoTiSaR were retrospectively investigated.

Flavopiridol restores granulopoiesis in experimental models of severe congenital neutropenia.

Severe congenital neutropenia (CN) patients require life-long treatment with recombinant human granulocyte colony-stimulating factor (rhG-CSF), but some show no response. We sought to establish a therapy for CN that targets signaling pathways causing maturation arrest of granulocytic progenitors. We developed an isogenic induced pluripotent stem cell (iPSC) in vitro model of CN associated with ELANE mutations (ELANE-CN) and performed an in silico drug repurposing analysis of the transcriptomics of iPSC-generated hematopoietic stem and progenitor cells.

Acute remote ischemic conditioning enhances (CD3+)- but not (FoxP3+)-T-cell invasion in the tumor center and increases IL 17 and TNF-alpha expression in a murine melanoma model.

Introduction: Hypoxia can drive tumor progression, suppress anti-tumor immunity, and reduce the effectiveness of radiotherapy and chemotherapy. This study aimed to assess the impact of remote ischemic conditioning (RIC) on tumor oxygenation (sO2) and the anti-tumor immune response.

Material And Methods: Fourteen B16-Ova tumor-bearing C57BL/6N mice received six 5-minute RIC cycles, while another fourteen underwent anesthesia only.

A New Severe Congenital Neutropenia Syndrome Associated with Autosomal Recessive COPZ1 Mutations.

We have identified a new inherited bone marrow (BM) failure syndrome with severe congenital neutropenia (CN) caused by autosomal recessive mutations in the coatomer protein complex I (COPI) subunit zeta 1 (COPZ1) gene. A stop-codon COPZ1 mutation and a missense mutation were found in three patients from two unrelated families. While two affected siblings with a stop-codon COPZ1 mutation suffered from congenital neutropenia (CN) that involves other hematological lineages, and non-hematological tissues, the patient with a missense COPZ1 mutation had isolated neutropenia.

Preserflo MicroShunt Implantation for Glaucoma Secondary to Viral and Juvenile Idiopathic Arthritis-Related Anterior Uveitis.

Purpose: To investigate the efficacy and safety of the Preserflo MicroShunt (PMS) in treating glaucoma secondary to different uveitic glaucoma (UG) types in a European population.

Methods: This study evaluated consecutive eyes with UG that received the PMS. The primary outcome measure was 12-month surgical success (≥20% IOP reduction, target IOP of 6-21 mmHg).

Does size matter? Center-specific characteristics and survival after allogeneic hematopoietic cell transplantation for acute myeloid leukemia: an analysis of the German Registry for Stem Cell Transplantation and Cell Therapy.

We investigated the effect of center-specific variables on overall survival (OS) after allogeneic hematopoietic cell transplantation (alloHCT) in acute myeloid leukemia (AML). Eligible were adult patients reported to DRST registry receiving first alloHCT for AML from a related or matched (>= 9/10 HLA-match) unrelated donor 2015-2021. Primary endpoint was OS at 12 months from alloHCT.

Longitudinal markers of cognitive procedural learning in fronto-striatal circuits and putative effects of a BDNF plasticity-related variant.

Procedural learning and automatization have widely been studied in behavioral psychology and typically involves a rapid improvement, followed by a plateau in performance throughout repeated training. More recently, brain imaging studies have implicated frontal-striatal brain circuits in skill learning. However, it is largely unknown whether frontal-striatal activation during skill learning and behavioral changes follow a similar learning curve pattern.

Mevalonate kinase deficiency: an updated clinical overview and revision of the SHARE recommendations.

Mevalonate kinase deficiency (MKD), a rare auto-inflammatory disorder, arises from mutations in the gene, disrupting isoprenoid biosynthesis, and affecting cellular processes. This comprehensive review provides an updated perspective on MKD, including its aetiology, pathogenesis, diagnostic modalities, and therapeutic strategies. Based on recent research and clinical advances, our objective is to bridge the knowledge gaps in the 2015 SHARE guidelines.

Non-invasive cerebral autoregulation monitoring during paediatric cardiac surgery without cardiopulmonary bypass requiring intraoperative cross-clamping of one of the carotid arteries.

Introduction: Neurologic complications remain one of the major risks after pediatric cardiac surgery. Cerebral autoregulation (CA) is a physiologic mechanism regulating cerebral perfusion. A dynamic intraoperative evaluation can possibly detect the impairment of the cerebral regulatory function during surgery.