The Prognostic Role of Pulmonary Arterial Elastance in Patients Undergoing Left Ventricular Assist Device Implantation: A Pilot Study.

: Pulmonary arterial elastance (Ea) is a helpful parameter to predict the risk of acute postoperative right ventricular failure (RVF) after left ventricular assist device (LVAD) implantation. A new method for calculating Ea, obtained by the ratio between transpulmonary gradient and stroke volume (Ea), has been proposed as a more accurate measure than the Ea obtained as the ratio between pulmonary artery systolic pressure and stroke volume (Ea). However, the role of Ea in predicting acute RVF post-LVAD implantation remains unclear.

Prenatal Attachment, Personality, and Depression in High-Risk Pregnancies During Pandemic Emergencies.

The observational study investigates how personality factors influence depression, prenatal attachment, and fear of COVID-19 in women with high-risk pregnancies. Women experiencing a high-risk pregnancy between the 20th and 24th weeks of gestation (N = 84) were selected. The Personality Inventory (PI), Beck Depression Inventory (BDI), Prenatal Attachment Inventory (PAI), and Fear of COVID (FCV-19S) were used for data collection.

Bortezomib for rituximab-refractory immune-mediated thrombotic thrombocytopenic purpura in the caplacizumab era: an Italian multicenter study.

Background: Immune-mediated thrombotic thrombocytopenic purpura (iTTP) patients are not responsive to standard rituximab in approximately 10% to 15% of cases, and oral immunosuppressants showed controversial results with significant toxicity. Targeting plasma cells with bortezomib appears promising, but the available evidence is scarce and stems only from isolated reports in the precaplacizumab era.

Objectives: To evaluate the safety and efficacy of bortezomib in rituximab-refractory iTTP patients.

Clinical, Laboratory, and Molecular Characteristics of Inherited Vitamin K-Dependent Coagulation Factors Deficiency.

Vitamin K-dependent coagulation factors deficiency (VKCFD) is a rare autosomal recessive genetic disease characterized by impaired levels of multiple coagulation factors (II, VII, IX, and X) and natural anticoagulants (proteins C and S). VKCFD is part of familial multiple coagulation factor deficiencies, reporting overall 50 affected families thus far. Disease manifestations are quite heterogeneous, bleeding symptoms may vary, and even, although generally mild, some patients may succumb to fatal outcomes.

Resilience in Adult Coeliac Patients on a Gluten-Free Diet: A Cross-Sectional Multicentre Italian Study.

Background: Data on resilience, the ability to recover from adversity, in coeliac disease (CeD) are lacking.

Aim: To assess the degree of resilience in patients with CeD on a gluten-free diet (GFD), and its association with clinical features, sociodemographic factors, psychological morbidity, and quality of life (QOL).

Methods: A cross-sectional multicentre Italian study was conducted on adult CeD patients between May 2022 and April 2023.

Personality Traits and Depression in Infertile Couples during the COVID-19.

: The study presented in this paper seeks to examine how personality traits and depressive symptoms, influenced by the fear of COVID-19, interact in infertile couples, who are on the verge of commencing treatments with assisted reproductive technology (ART). The purpose of this observational study was to explore the relationship between personality traits in infertile couples and the occurrence of depressive symptoms, taking into account the mediating effect of COVID-19 fear. : The study sample consisted of 108 women and 71 men (N = 179), all of whom had received an infertility diagnosis and expressed a desire to begin ART treatment at a Sicilian ART center; they were subsequently recruited.

Tissue- and liquid-biopsy based NGS profiling in advanced non-small-cell lung cancer in a real-world setting: the IMMINENT study.

Introduction: To date, for all non-small cell lung cancer (NSCLC) cases, it is recommended to test for driver alterations to identify actionable therapeutic targets. In this light, comprehensive genomic profiling (CGP) with next generation sequencing (NGS) has progressively gained increasing importance in clinical practice. Here, with the aim of assessing the distribution and the real-world frequency of gene alterations and their correlation with patient characteristics, we present the outcomes obtained using FoundationOne (F1CDx) and FoundationLiquid CDx (F1L/F1LCDx) NGS-based profiling in a nationwide initiative for advanced NSCLC patients.

Anemia and iron overload as prognostic markers of outcomes in β-thalassemia.

Introduction: Ineffective erythropoiesis and subsequent anemia as well as primary and secondary (transfusional) iron overload are key drivers for morbidity and mortality outcomes in patients with β-thalassemia.

Areas Covered: In this review, we highlight evidence from observational studies evaluating the association between measures of anemia and iron overload versus outcomes in both non-transfusion-dependent and transfusion-dependent forms of β-thalassemia.

Expert Opinion: Several prognostic thresholds have been identified with implications for patient management.

Hyperlysinemia, an ultrarare inborn error of metabolism: Review and update.

Unlabelled: Familial hyperlysinemia is a rare autosomal recessive disorder due to defects of the AASS (α-aminoadipate δ-semialdehyde synthase) gene, which encodes for a bifunctional enzyme. Two types of hyperlysinemia have been identified namely type 1, due to the deficit of the alfa-ketoglutarate activity, and type 2, due to the deficit of the saccharopine dehydrogenase activity.

Methods: To better characterize the phenotypic spectrum of familial hyperlysinemia type 1, we conducted a systematic review of cases in the literature following PRISMA guidelines.

Oxidative Stress as a Target for Non-Pharmacological Intervention in MAFLD: Could There Be a Role for EVOO?

Oxidative stress plays a central role in most chronic liver diseases and, in particular, in metabolic dysfunction-associated fatty liver disease (MAFLD), the new definition of an old condition known as non-alcoholic fatty liver disease (NAFLD). The mechanisms leading to hepatocellular fat accumulation in genetically predisposed individuals who adopt a sedentary lifestyle and consume an obesogenic diet progress through mitochondrial and endoplasmic reticulum dysfunction, which amplifies reactive oxygen species (ROS) production, lipid peroxidation, malondialdehyde (MDA) formation, and influence the release of chronic inflammation and liver damage biomarkers, such as pro-inflammatory cytokines. This close pathogenetic link has been a key stimulus in the search for therapeutic approaches targeting oxidative stress to treat steatosis, and a number of clinical trials have been conducted to date on subjects with NAFLD using drugs as well as supplements or nutraceutical products.

Iron chelation therapy for children with transfusion-dependent β-thalassemia: How young is too young?

In this review, we provide a summary of evidence on iron overload in young children with transfusion-dependent β-thalassemia (TDT) and explore the ideal timing for intervention. Key data from clinical trials and observational studies of the three available iron chelators deferoxamine, deferiprone, and deferasirox are also evaluated for inclusion of subsets of young children, especially those less than 6 years of age. Evidence on the efficacy and safety of iron chelation therapy for children ≥2 years of age with transfusional iron overload is widely available.

Endothelial function in healthy centenarians living in the Madonie's district (Italy).

Background: Endothelial function declines with age and plays a critical role in cardiovascular health. Therefore, investigating endothelial function in successful aging models, such as centenarians, is of interest. Flow-mediated dilation (FMD) of the brachial artery is the gold standard for measuring endothelial function in vivo in humans.

The role of Killer immunoglobulin-like receptors (KIRs) in the genetic susceptibility to non-celiac wheat sensitivity (NCWS).

Objectives: Non-celiac wheat sensitivity (NCWS) is an emerging clinical condition characterized by gastrointestinal and extraintestinal symptoms following the ingestion of gluten-containing foods in patients without celiac disease (CD) or wheat allergy. Despite the great interest for NCWS, the genetic risk factors still need to be fully clarified. In this study, we first assessed the possible contribution of genes and KIR haplotypes on the genetic predisposition to NCWS.

Fecal calprotectin levels in patients with non-celiac wheat sensitivity: a proof of concept.

Some data suggest the existence of intestinal inflammation in patients with non-celiac wheat sensitivity (NCWS). We aimed to verify whether fecal calprotectin (FCP), a marker of intestinal inflammation, could be used to confirm this inflammatory status and to test its diagnostic performance in differentiating NCWS from irritable bowel syndrome/functional dyspepsia (IBS/FD). We conducted a multicenter study, comparing NCWS patients, diagnosed by a double-blind placebo-controlled wheat challenge, with IBS/FD subjects.

Prognostic Role of Multiparametric Cardiac Magnetic Resonance in Neo Transfusion-Dependent Thalassemia.

Background: We prospectively evaluated the predictive value of multiparametric cardiac magnetic resonance (CMR) for cardiovascular complications in non-transfusion-dependent β-thalassemia (β-NTDT) patients who started regular transfusions in late childhood/adulthood (neo β-TDT).

Methods: We considered 180 patients (38.25 ± 11.

Distance follow-up by a remote medical care centre improves adherence to CPAP in patients with obstructive sleep apnoea over the short and long term.

Background: Adherence to continuous positive air pressure (CPAP) in patients with obstructive sleep apnoea (OSA) has remained invariably low over the last decades. Remote monitoring of the nocturnal CPAP treatment, within telemedicine (TM)-based follow-up programs, in these patients has been suggested as a potential tool to improve adherence and release the workload of sleep units. The aim of this study was therefore to assess whether a follow-up program carried out by a Remote Medical Care Centre (RMCC), outside the sleep unit, improves adherence to CPAP in the short and long term in patients with OSA.

Antithrombotic Treatment for Stroke Prevention in Cervical Artery Dissection: The STOP-CAD Study.

Background: Small, randomized trials of patients with cervical artery dissection showed conflicting results regarding optimal stroke prevention strategies. We aimed to compare outcomes in patients with cervical artery dissection treated with antiplatelets versus anticoagulation.

Methods: This is a multicenter observational retrospective international study (16 countries, 63 sites) that included patients with cervical artery dissection without major trauma.

Very Prolonged Treatment with Albendazole of a Case of Disseminated Abdominal Cystic Echinococcosis.

Cystic echinococcosis is a zoonosis caused by the ingestion of food or water contaminated by eggs. E. granulosus is the most common causative agent of cystic echinococcosis that still has a relevant incidence in Italy, especially on the islands of Sicily and Sardinia.

Global hemostasis assays in acute myeloid leukemia: results of an observational prospective study.

Background: Acute myeloid leukemia (AML) is characterized by a complex spectrum of coagulopathy ranging from hemorrhagic to thrombotic symptoms. To date, platelet count (PLT) and conventional coagulation tests (CCTs) cannot predict hemorrhagic events and thrombotic risk. Thromboelastography (TEG) measures the viscoelastic properties of the clot, thus providing information on the entire process of blood coagulation.

Impact of influenza vaccination on survival of patients with advanced cancer receiving immune checkpoint inhibitors (INVIDIa-2): final results of the multicentre, prospective, observational study.

Background: The prospective multicentre observational INVIDIa-2 study investigated the clinical effectiveness of influenza vaccination in patients with advanced cancer receiving immune checkpoint inhibitors (ICI). In this secondary analysis of the original trial, we aimed to assess the outcomes of patients to immunotherapy based on vaccine administration.

Methods: The original study enrolled patients with advanced solid tumours receiving ICI at 82 Italian Oncology Units from Oct 1, 2019, to Jan 31, 2020.

Adaptive responses to chronic intermittent hypoxia: contributions from the European Sleep Apnoea Database (ESADA) Cohort.

Obstructive sleep apnoea (OSA) is a common disease in the general population, and is associated with increased cardiovascular risk and several comorbidities. Obesity favours upper airway collapsibility, but other pathophysiological traits have been identified, i.e.