New insights into the management of homozygous familial hypercholesterolemia patients treated with lomitapide: a single-center experience.

Familial hypercholesterolemia (FH) is a genetic disease, usually with onset during childhood, characterized by elevated blood LDL cholesterol levels and potentially associated with severe cardiovascular complications. Concerning mutated genes in FH, such as , a small subset of FH patients presents a homozygous genotype, resulting in homozygous FH (HoFH) disease with a generally aggressive phenotype. Besides statins, ezetimibe and PCSK9 inhibitors, lomitapide (an anti-ApoB therapy) was also approved in 2012-2013 as an adjunctive treatment for HoFH.

Cardiopulmonary exercise response at high altitude in patients with congenital heart disease: a systematic review and meta-analysis.

Background: An increasing number of patients with congenital heart disease (CHD) engage in physical activities and may exercise at high altitudes (HA). The physiological adaptations required at HA and their implications on individuals with CHD, especially during exercise, remain underexplored. This systematic review aims to investigate cardiopulmonary exercise responses to short-term HA exposure in individuals with CHD.

Absence of Viral Replication Is Associated With Improved Outcome in Anti-HCV-Positive Patients With Hepatocellular Carcinoma.

Background And Aims: Presence of active hepatitis C virus (HCV) infection may influence the outcome of patients treated for hepatocellular carcinoma (HCC), although this issue has never been adequately assessed in a large series of patients. The aim of this study was to evaluate whether the presence of active HCV affects the survival of patients treated for HCC.

Methods: This study assessed the outcome of 3123 anti-HCV-positive patients with HCC, subdivided according to the presence of active HCV infection or previous sustained virological response (SVR).

Critical Care Therapy After Cytoreductive Nephrectomy for Metastatic Kidney Cancer.

Objectives: To examine critical care therapy rates after cytoreductive nephrectomy in metastatic kidney cancer patients.

Design, Setting, And Patients: Relying on the National Inpatient Sample (2000-2019), we addressed critical care therapy use (total parenteral nutrition, invasive mechanical ventilation, renal replacement therapy, percutaneous endoscopic gastrostomy tube insertion, and tracheostomy) and in-hospital mortality in surgically treated metastatic kidney cancer patients. Estimated annual percentage changes and multivariable logistic regression models were fitted.

Association between recorded physical activity and cancer progression or mortality in individuals diagnosed with cancer in South Africa.

Objectives: This study aimed to determine the association between progression and mortality in individuals with stage 1 cancer and their recorded physical activity before the diagnosis of the cancer.

Methods: We included 28 248 members with stage 1 cancers enrolled in an oncology programme in South Africa. Physical activity was recorded using fitness devices, logged gym sessions and participation in organised fitness events.

Polymer-free versus biodegradable-polymer drug-eluting stent in patients undergoing percutaneous coronary intervention: an assessor-blind, non-inferiority, randomised controlled trial.

Background: Few data are available on polymer-free drug-eluting stents in patients undergoing percutaneous coronary intervention (PCI).

Aims: We aimed to determine the efficacy and safety of a polymer-free amphilimus-eluting stent (AES), using a reservoir-based technology for drug delivery, compared with a biodegradable-polymer everolimus-eluting stent (EES).

Methods: This was a randomised, investigator-initiated, assessor-blind, non-inferiority trial conducted at 14 hospitals in Italy (ClinicalTrials.

Differential expression of plasma proteins and pathway enrichments in pediatric diabetic ketoacidosis.

Background: In children with type 1 diabetes (T1D), diabetic ketoacidosis (DKA) triggers a significant inflammatory response; however, the specific effector proteins and signaling pathways involved remain largely unexplored. This pediatric case-control study utilized plasma proteomics to explore protein alterations associated with severe DKA and to identify signaling pathways that associate with clinical variables.

Methods: We conducted a proteome analysis of plasma samples from 17 matched pairs of pediatric patients with T1D; one cohort with severe DKA and another with insulin-controlled diabetes.

Carbohydrate conversion in spent coffee grounds: pretreatment strategies and novel enzymatic cocktail to produce value-added saccharides and prebiotic mannooligosaccharides.

Background: Spent coffee grounds (SCG) are the most abundant waste byproducts generated from coffee beverage production worldwide. Typically, these grounds are seen as waste and end up in landfills. However, SCG contain valuable compounds that can be valorized and used in different applications.

Evaluating the impact of changing inhaler color on perception of symptoms and disease burden in patients with asthma: The FEEL study.

ObjectivesPatient perception of treatment effectiveness is key to optimizing adherence. This is potentially impacted by color, yet no such studies have been conducted in asthma. This study assessed the influence of pink vs.

Pemphigus and Bullous Pemphigoid Following COVID-19 Vaccination: A Systematic Review.

The COVID-19 pandemic has encouraged the rapid development and licensing of vaccines against SARS-CoV-2. Currently, numerous vaccines are available on a global scale and are based on different mechanisms of action, including mRNA technology, viral vectors, inactive viruses, and subunit particles. Mass vaccination conducted worldwide has highlighted the potential development of side effects, including ones with skin involvement.

Morphological and Photosynthetic Pigment Screening of Four Microgreens Species Exposed to Heavy Ions.

Numerous challenges are posed by the extra-terrestrial environment for space farming and various technological growth systems are being developed to allow for microgreens' cultivation in space. Microgreens, with their unique nutrient profiles, may well integrate the diet of crew members, being a natural substitute for chemical food supplements. However, the space radiation environment may alter plant properties, and there is still a knowledge gap concerning the effects of various types of radiation on plants and specifically on the application of efficient and rapid methods for selecting new species for space farming, based on their radio-resistance.

Essential Oils Obtained from Sicilian Blanco By-Products: Antibacterial and Allelopathic Activity.

Mandarin, one of the winter fruits commonly used in the preparation of foods and juices, is a fruit native to China and Southeast Asia. In this work, essential oils (EOs) obtained from by-products of the Blanco flavedo of five cultivars present and cultivated within the Botanical Garden of Palermo were chemically and biologically studied: 'Avana' (), 'Tardivo di Ciaculli' (), 'Bombajensis' (), 'Aurantifolia' (), and 'Padre Bernardino' (). The GC and GC-MS analysis performed on all the extracted samples clearly highlighted the notable presence of limonene, a characteristic hydrocarbon monoterpene of EOs of the genus.

Unveiling the Bioleaching Versatility of .

is a Gram-negative bacterium that thrives in extreme acidic conditions. It has emerged as a key player in biomining and bioleaching technologies thanks to its unique ability to mobilize a wide spectrum of elements, such as Li, P, V, Cr, Fe, Ni, Cu, Zn, Ga, As, Mo, W, Pb, U, and its role in ferrous iron oxidation and reduction. catalyzes the extraction of elements by generating iron (III) ions in oxic conditions, which are able to react with metal sulfides.

Analysis of Polyphenols During Alcoholic Fermentation of Red Grape Aglianico ( L.): Potential Winemaking Optimization and Pomace Valorization.

The polyphenol extraction and evolution during a traditional 14-day fermentation of the Aglianico red grape, a variety widely cultivated across Southern Italy, was for the first time investigated, with the purpose of optimizing the phenolic profile in finished wines. Anthocyanins, BSA-reactive tannins, iron-reactive phenols, and vanillin-reactive flavans (VRFs) were analyzed in the free-run must, pressed pomace liquid, and in pomace extracts at different maceration times. Experimental evidence suggested that, instead of the typical 14-day maceration of Aglianico grapes, it is recommendable to choose an 11-day maceration in order to prevent the over-extraction of polyphenols that may detrimentally affect the sensory characteristics of wines.

Sphaeropsidin A Loaded in Liposomes to Reduce Its Cytotoxicity and Preserve Antifungal Activity Against .

species constitute the most common cause of fungal infections in humans; the emergence of resistance and biofilm formation by species further threaten the limited availability of antifungal agents. Over the past decade, . has caused significant outbreaks worldwide and has emerged as a human pathogenic fungus that causes diseases ranging from superficial to life-threatening disseminated infections.

Kanamycin and G-Quadruplexes: An Exploration of Binding Interactions.

G-quadruplexes (G4s) are distinctive four-stranded nucleic acid structures formed by guanine-rich sequences, making them attractive targets for drug repurposing efforts. Modulating their stability and function holds promise for treating diseases like cancer. To identify potential drug candidates capable of interacting with these complex DNA formations, docking studies and molecular dynamics (MDs) simulations were conducted.

Oleanolic Acid Modulates DNA Damage Response to Camptothecin Increasing Cancer Cell Death.

Targeting DNA damage response (DDR) pathways represents one of the principal approaches in cancer therapy. However, defects in DDR mechanisms, exhibited by various tumors, can also promote tumor progression and resistance to therapy, negatively impacting patient survival. Therefore, identifying new molecules from natural extracts could provide a powerful source of novel compounds for cancer treatment strategies.

Exploring the Link Between Psoriasis and Adipose Tissue: One Amplifies the Other.

Psoriasis and obesity, while distinct, are inter-related inflammatory conditions. Adipose tissue (AT)-derived mediators could be pathogenically active in triggering and/or amplifying psoriatic skin inflammation and, vice versa, skin inflammation could drive increased adiposity that triggers the development of several chronic conditions. Gaining insight into their intricate relationship could be essential for effective management and treatment.

P2 Receptor Antagonists Rescue Defective Heme Content in an In Vitro SLC25A38-Associated Congenital Sideroblastic Anemia Cell Model.

Mutations in the SLC25A38 gene are responsible for the second most common form of congenital sideroblastic anemia (CSA), a severe condition for which no effective treatment exists. We developed and characterized a K562 erythroleukemia cell line with markedly reduced expression of the SLC25A38 protein (A38-low cells). This model successfully recapitulated the main features of CSA, including reduced heme content and mitochondrial respiration, increase in mitochondrial iron, ROS levels and sensitivity to oxidative stress.

"To Be or Not to Be" a Conscientious Objector to Voluntary Abortion: An Italian Web-Survey of Healthcare Workers.

: Conscientious objection to voluntary abortion remains a hot debate topic. This could affect the accessibility to pregnancy termination. Our aim is to evaluate the possible aspects related to an operators' choice about objection for voluntary abortion, such as the following: the abolition of the time limit, the instruction of a multi-collegiate commission, the introduction of pharmacological rather than surgical procedures, the fetal/maternal illness and the case of sexual violence.

Real-World Effectiveness and Safety of Selective JAK Inhibitors in Ulcerative Colitis and Crohn's Disease: A Retrospective, Multicentre Study.

: Data on the real-world effectiveness and safety of selective JAK inhibitors (JAKis) in ulcerative colitis (UC) and Crohn's disease (CD) are limited. : We conducted a multicentre, retrospective study to assess clinical, biochemical, and endoscopic outcomes of selective JAKis in bio-experienced UC and CD. : A total of 246 patients (mean age: 40.

The Role of Therapeutic Endoscopic Ultrasound in Management of Malignant Double Obstruction (Biliary and Gastric Outlet): A Comprehensive Review with Clinical Scenarios.

Endoscopic ultrasound (EUS)-guided interventions have revolutionized the management of malignant biliary obstruction (MBO) and gastric outlet obstruction (GOO), providing minimally invasive alternatives with improved outcomes. These procedures have significantly reduced the need for high-risk surgical interventions or percutaneous alternatives and have provided effective palliative care for patients with advanced gastrointestinal and bilio-pancreatic malignancies. EUS-guided biliary drainage (EUS-BD) techniques, including hepaticogastrostomy (EUS-HGS), choledochoduodenostomy (EUS-CDS), and antegrade stenting (EUS-AS), offer high technical and clinical success rates, with a good safety profile particularly when Endoscopic Retrograde Cholangiopancreatography (ERCP) is not feasible.

Stoma Acceptance Mediates Body Image Distress and Mental Health-Related Quality of Life: A Single-Center Study on Radical Cystectomy Patients with Ureterostomy.

Muscle-invasive bladder cancer and subsequent radical cystectomy with ureterocutaneostomy significantly impact patients' body image and quality of life, potentially increasing the risk of adverse mental health outcomes. Acceptance may represent a psychosocial resource to buffer the effects of body image impairment on health, thereby supporting stoma adjustment and preserving quality of life. This study aimed to investigate the mediating role of stoma acceptance in the relationship between body image distress and mental health.

The Zebrafish Retina and the Evolution of the Onecut-Mediated Pathway in Cell Type Differentiation.

() genes play an important role in the proper formation of retinal cells in vertebrates, in particular horizontal, retinal ganglion and amacrine cells. However, it is not fully known how the unique and combined action of multiple gene copies leads to the induction and differentiation of specific retinal cell types. To gain new insights on how genes influence retina formation, we have examined the developmental role of , and genes during eye formation in the non-mammalian vertebrate zebrafish .

Food Allergy-Related Bullying in Pediatric Patients: A Systematic Review.

Background: Food allergy (FA)-related bullying is a significant public health concern affecting pediatric patients. This systematic review investigates the prevalence, characteristics, and psychosocial impact of FA-related bullying, as well as current intervention strategies within educational and healthcare settings.

Methods: A systematic literature search was conducted across the PubMed, Web of Science, and CINAHL databases, covering publications up to February 2024.