The clinical assessment of lung involvement in patients with Still's disease, results from the multicentre international AIDA Network Still's Disease Registry.

Objectives: To assess the lung involvement in patients with Still's disease, an inflammatory disease assessing both children and adults. To exploit possible associated factors for parenchymal lung involvement in these patients.

Methods: A multicentre observational study was arranged assessing consecutive patients with Still's disease characterized by the lung involvement among those included in the AIDA (AutoInflammatory Disease Alliance) Network Still's Disease Registry.

Longitudinal Assessment of Abnormal Cortical Folding in Fetuses and Neonates With Isolated Non-Severe Ventriculomegaly.

Purpose: The impact of ventriculomegaly (VM) on cortical development and brain functionality has been extensively explored in existing literature. VM has been associated with higher risks of attention-deficit and hyperactivity disorders, as well as cognitive, language, and behavior deficits. Some studies have also shown a relationship between VM and cortical overgrowth, along with reduced cortical folding, both in fetuses and neonates.

Fetal Corpus Callosum Anomalies: A Review of Underlying Genetic Disorders and Prenatal Testing Options.

Anomalies of the corpus callosum (CC) are amongst the most common fetal Central Nervous System (CNS) anomalies detectable on ultrasound. Underlying genetic disease plays an important part in defining prognosis. Associations with aneuploidy and submicroscopic chromosomal deletions or duplications have been well demonstrated using chromosomal microarray analysis.

Idecabtagene vicleucel or ciltacabtagene autoleucel for relapsed or refractory multiple myeloma: An international multicenter study.

Idecabtagene vicleucel (ide-cel) and ciltacabtagene autoleucel (cilta-cel) have revolutionized the treatment of relapsed/refractory multiple myeloma (RRMM), but direct comparisons are lacking. Leveraging an international multicenter RRMM cohort, we compared the outcome of ide-cel ( = 162) versus cilta-cel ( = 42). Co-primary efficacy endpoints of the study were overall response rate (ORR) and progression-free survival (PFS).

Germline structural variant as the cause of Lynch Syndrome in a family from Ecuador.

Colorectal cancer (CRC) is one of the most common cancers worldwide. Lynch Syndrome (LS) is the most common form of hereditary CRC and it is caused by germline defects in the DNA-mismatch repair (MMR) pathway. It is of extreme importance for affected LS patients and their relatives to identify the germline causative alteration to provide intensified surveillance to those at risk and allow early diagnosis and cancer prevention.

Deep cascaded registration and weakly-supervised segmentation of fetal brain MRI.

Deformable image registration is a cornerstone of many medical image analysis applications, particularly in the context of fetal brain magnetic resonance imaging (MRI), where precise registration is essential for studying the rapidly evolving fetal brain during pregnancy and potentially identifying neurodevelopmental abnormalities. While deep learning has become the leading approach for medical image registration, traditional convolutional neural networks (CNNs) often fall short in capturing fine image details due to their bias toward low spatial frequencies. To address this challenge, we introduce a deep learning registration framework comprising multiple cascaded convolutional networks.

Effectiveness of positive allosteric modulators of metabotropic glutamate receptor 2/3 (mGluR2/3) in animal models of schizophrenia.

Schizophrenia (SZ) is a deleterious brain disorder characterised by its heterogeneity and complex symptomatology consisting of positive, negative and cognitive deficits. Current antipsychotic drugs ameliorate the positive symptomatology, but are inefficient in treating the negative symptomatology and cognitive deficits. The neurodevelopmental glutamate hypothesis of SZ has opened new avenues in the development of drugs targeting the glutamatergic system.

Risk Factors and Outcomes Associated With Polymicrobial Infection in Community-Acquired Pneumonia.

Background: Polymicrobial pneumonia is a concern for clinicians due to its association with increased disease severity. Determining the prevalence of polymicrobial pneumonia and identifying patients who have an increased risk of this aetiology is important for the management of CAP patients. Here we describe the clinical characteristics and outcomes of adult hospitalized patients with CAP, and identify the risk factors related to polymicrobial pneumonia and specifically to 30-day mortality.

Endothelial Damage in JAK2V617F Myeloproliferative Neoplasms with Splanchnic Vein Thrombosis.

Background:  V617F-mutated myeloproliferative neoplasms (MPN) exhibit abnormal proliferation of bone marrow progenitors and increased risk of thrombosis, specifically in splanchnic veins (SVT). The contribution of the endothelium to the development of the prothrombotic phenotype was explored.

Material And Methods:  Plasma and serum samples from V617F MPN patients with (n=26) or without (n=7) thrombotic debut and different treatments, were obtained (n=33).

Humoral and cellular immune responses after 6 months of a heterologous SARS-CoV-2 booster with the protein-based PHH-1V vaccine in a phase IIb trial.

The HIPRA-HH-2 was a multicentre, randomized, active-controlled, double-blind, non-inferiority phase IIb clinical trial comparing the immunogenicity and safety of the PHH-1V adjuvanted recombinant vaccine as a heterologous booster against homologous booster with BNT162b2. Interim results demonstrated strong humoral and cellular immune response against the SARS-CoV-2 Wuhan-Hu-1 strain and the Beta, Delta, and Omicron BA.1 variants up to day 98 post-dosing.

Searching for the minimum required dose of daratumumab to induce effective plasma cell depletion in antibody-mediated rejection of the kidney graft: a case report.

There is no established treatment for late or chronic antibody-mediated rejection of a kidney graft. Rituximab-based treatment is not effective, since long-lived high-affinity plasma cells do not express CD20 and do not depend on previous maturation steps to generate donor-specific antibodies. Conversely, daratumumab, an anti-CD38 monoclonal antibody, directly targets plasma cells, with proven efficacy in multiple myeloma.

Heterogeneity of reduced FEV in early adulthood: A looking forward, looking backwards analysis.

Background: Some individuals never achieve normal peak FEV in early adulthood. It is unknown if this is due to airflow limitation and/or lung restriction.

Methods: To investigate this, we: (1) looked forward in 19,791 participants in the Dutch Lifelines general population cohort aged 25-35 years with 5-year follow-up; and (2) looked backwards in 2032 participants in the Swedish BAMSE birth cohort with spirometry at 24 years of age but also at 16 and/or 8 years.

Study Protocol for a Prospective, Unicentric, Double-Blind, Randomized, and Placebo-Controlled Trial on the Efficacy of a Low-Histamine Diet and DAO Enzyme Supplementation in Patients with Histamine Intolerance.

Background/objectives: Histamine intolerance is primarily caused by a deficiency in the diamine oxidase (DAO) enzyme at the intestinal level. The reduced histamine degradation in the gut leads to its accumulation in plasma, thereby causing multiple clinical manifestations, such as urticaria, diarrhea, headache, dyspnea, or tachycardia, among others. The dietary management of this food intolerance consists of the follow-up of a low-histamine diet, often combined with DAO supplementation.

Transorbital laser surgery for epilepsy: Anatomic-radiological feasibility of transorbital magnetic resonance imaging-guided laser interstitial thermal therapy (MRIgLITT) for amygdalohippocampectomy in refractory epilepsy.

Objective: to study the anatomical feasibility of laser fiber insertion for interstitial thermal therapy via transorbital approach to the temporo-mesial structures (amygdala-hippocampus-parahippocampus complex).

Methods: Anatomical dissections were performed bilaterally on two human cadaveric heads via a transorbital approach, in which screws and laser fibers were used for magnetic resonance imaging-guided laser interstitial thermal therapy (MRIgLITT) assisted by neuronavigation. In addition, eight transorbital trajectories were simulated using the transorbital entry points obtained from a cadaveric radiological study of four patients previously operated on for mesial temporal lobe epilepsy.

Safety of steroids in severe community-acquired pneumonia.

The systemic use of corticosteroids for patients with severe community-acquired pneumonia (sCAP) remains controversial in clinical practice, particularly in terms of the safety profile of these drugs. This narrative review aims to analyse the available literature data concerning the safety of short-term steroid use in the treatment of sCAP, while also highlighting potential future research directions. Several trials and meta-analyses have evaluated corticosteroid therapy as an adjuvant treatment for sCAP, yielding heterogeneous results regarding its efficacy and safety.

Survival benefit associated with liver transplantation for hepatocellular carcinoma based on tumor burden scores at listing.

Introduction: Liver transplantation (LT) provides significant survival benefits to patients with unresectable HCC. In the United States, organ allocation policies for HCCs within the United Network for Organ Sharing criteria do not prioritize patients based on their differences in oncological characteristics. This study assessed whether transplant-associated survival benefits (TASBs) vary among patients with different tumor burden scores (TBS) measured at the time of listing.