Expert insights on Hodgkin's lymphoma development in an activated PI3K delta syndrome patient undergoing leniolisib treatment.

Activated PI3K delta syndrome (APDS) is a primary immunodeficiency that is caused by mutations in the PI3K signalling pathway resulting in either gain-of-function or loss-of-function phenotypes of APDS 1 and 2. Malignancy is one of the most serious complications associated with APDS patients, with the most commonly occurring of these being lymphoma, and is the most common cause of death in APDS patients. Management of APDS is complex and variable due to the heterogeneous nature of the disease and ranges from antimicrobial and immunosuppressant agents to haematopoetic stem cell transplantation.

Retinal Protection of New Nutraceutical Formulation.

Retinal ganglion cell (RGC) protection represents an unmet need in glaucoma. This study assessed the neuroprotective, antioxidant, and anti-inflammatory effect of a new nutraceutical formulation named Epicolin, based on citicoline, homotaurine, epigallocatechin-3-gallate, forskolin, and vitamins, through in vitro and in vivo studies. The neuroprotective effect of Epicolin or its single components, and Epicolin compared to an untreated control and two marketed formulations [Formulation G (FG) and N (FN)], was evaluated in neuroblastoma cells (SH-SY5Y) challenged with staurosporine.

Adherence to vaccination against SARS-CoV-2 and vaccine safety in patients with immunoglobulin G4-related disease.

Objective: To assess the adherence to the vaccination campaign against SARS-CoV-2 in patients with immunoglobulin-G4-related disease (IgG4-RD) and to evaluate the development of local and systemic adverse events (AEs) following vaccination. Additionally, to investigate the rate and outcome of SARS-CoV-2 infection in IgG4-RD patients.

Methods: Patients with IgG4-RD in follow-up before the onset of the SARS-CoV-2 pandemic were contacted by telephone and asked to answer an ad hoc questionnaire regarding their vaccination status against SARS-CoV-2 and related AEs following vaccination.

Interleukin-32 positive immune and resident cells in kidney samples from lupus patients: a pilot study.

Introduction: Lupus nephritis (LN), caused by immune complexes produced or deposited from the bloodstream, is one of the most severe features of Systemic Lupus Erythematosus (SLE) leading to an increased morbidity and mortality. Toll like receptors (TLRs), such as TLR3, TLR7 and TLR9, may play a key role in its pathogenesis. Interleukin-32 (IL-32), a cytokine involved in both innate and adaptive immune responses, has been widely considered in autoimmune-inflammatory rheumatic diseases.

Baricitinib dose reduction in patients with rheumatoid arthritis achieving sustained disease control: Final results from the RA-BEYOND study.

Objective: This study examines the impact of dose step-down in patients with rheumatoid arthritis (RA) who achieved sustained disease control with baricitinib 4-mg once-daily up to 96-weeks.

Methods: Patients who completed a baricitinib phase 3 study could enter a long-term extension (LTE). In the LTE, patients who received baricitinib 4-mg for ≥15 months and maintained clinical disease activity index (CDAI) low disease activity (LDA) or remission (REM) were blindly randomized to continue 4-mg or taper to 2-mg.

New Insights in Microplastic Cellular Uptake Through a Cell-Based Organotypic Rainbow-Trout () Intestinal Platform.

Microplastics (MPs) in fish can cross the intestinal barrier and are often bioaccumulated in several tissues, causing adverse effects. While the impacts of MPs on fish are well documented, the mechanisms of their cellular internalization remain unclear. A rainbow-trout () intestinal platform, comprising proximal and distal intestinal epithelial cells cultured on an Alvetex scaffold, was exposed to 50 mg/L of MPs (size 1-5 µm) for 2, 4, and 6 h.

Bridging trauma and eating disorders: the role of loneliness.

Introduction: Eating disorders (EDs) are complex and often linked to traumatic childhood experiences. While childhood trauma is known to increase the risk of EDs, the role of loneliness remains underexplored. This study investigates whether loneliness mediates the relationship between childhood trauma and ED symptoms.

Report of the Italian Cohort with Activated Phosphoinositide 3-Kinase δ Syndrome in the Target Therapy Era.

Background: Activated Phosphoinositide 3-Kinase (PI3K) δ Syndrome (APDS), an inborn error of immunity due to upregulation of the PI3K pathway, leads to recurrent infections and immune dysregulation (lymphoproliferation and autoimmunity).

Methods: Clinical and genetic data of 28 APDS patients from 25 unrelated families were collected from fifteen Italian centers.

Results: Patients were genetically confirmed with APDS-1 (n = 20) or APDS-2 (n = 8), with pathogenic mutations in the PIK3CD or PIK3R1 genes.

An Automated Low-Cost Solution for Creating a Multiple-Step Thermal Gradient to Record Daily Fish Thermoregulatory Behavior.

Photoperiod and temperature are two of the most powerful environmental cues that entrain circadian clocks. Being ectothermic, fish must keep their body temperature within a physiological range to optimize biological processes mainly applying behavioral strategies. Here, we developed a low-cost, automated system that allows to create a horizontal multiple-step thermal gradient and video record fish behavior for long-term periods.

Aseptic abscess syndrome: a case report of a patient achieving remission with both infliximab originator and biosimilar administered at varied intervals.

Aseptic abscesses syndrome is a rare but increasingly recognized disease that falls within the spectrum of autoinflammatory disorders. Here, we describe the case of a patient who presented with abdominal pain and fever, along with multiple abdominal and extra-abdominal abscesses, in the absence of underlying hematologic, autoimmune, infectious, or neoplastic conditions. Initially, the patient responded to glucocorticoids, but experienced several flares upon discontinuation, leading to the initiation of treatment with a TNFα inhibitor.

Are Tattoos Safe in Patients with Systemic Lupus Erythematosus? Results From a Single-Center Study.

Introduction: Systemic Lupus Erythematosus is a pleiotropic autoimmune disease with common skin involvement. To date, only one study has investigated tattoos safety in SLE patients.

Objective: We performed a single-center study to evaluate the development of local and systemic complications after tattooing in a cohort of systemic lupus erythematosus (SLE) patients.

Early decrease of T-bet B cells during subcutaneous belimumab predicts response to therapy in systemic lupus erythematosus patients.

Systemic lupus erythematosus (SLE) is characterized by B cell dysregulation and expansion of atypical B cells that may correlate with disease manifestations and activity. This study investigated the impact of subcutaneous (sc) Belimumab (BLM) on the peripheral B cell compartment and on the functional properties of CD21, T-bet and CD11c atypical B cells, in 21 active SLE patients over a 12-month period. At baseline, active SLE patients displayed reduced unswitched IgM memory B cells and expansion of atypical B cells, compared to healthy donors and to SLE patients in remission.

Consensus of the Italian Primary Immunodeficiency Network on the use and interpretation of genetic testing for the diagnosis of inborn errors of immunity (IEI).

Background: Inborn errors of immunity (IEIs) are more than 500 different rare congenital disorders of the immune system characterized by susceptibility to infections and immune dysregulation. The significant overlap of the clinical features among the different forms may lead to diagnostic delay. High throughput sequencing techniques may allow a timely genetic definition.

The Role of Dietary Fatty Acids in Modulating Blue Crab () Physiology, Reproduction, and Quality Traits in Captivity.

The invasive blue crab is challenging the Mediterranean basin, progressively declining local populations. This reflects a lower prey availability and suitability of dietary nutrients (mainly n-3 polyunsaturated fatty acids, PUFA). The present study aimed to challenge blue crab males and females with a feed source low in n-3 PUFA with respect to one showing a proper fatty acid profile and to investigate the responses in terms of growth, welfare, lipid characterization of target tissues, and reproductive status.

HLA evolutionary divergence effect on bacterial infection risk in cirrhotic liver transplant candidates.

Bacterial infections are common in cirrhosis patients, increasing the risk of decompensation and death. The impact of HLA evolutionary divergence (HED) on infection risk hasn't been studied in humans before. We conducted a retrospective study on cirrhosis patients awaiting liver transplantation (LT) from January 2019 to February 2022, examining class I and II-HED effects on bacterial infections and cirrhosis decompensation.

The holistic management of peripheral spondyloarthritis: focus on articular involvement in patients with inflammatory bowel disease.

Objective: To provide a comprehensive overview of peripheral spondyloarthritis (pSpA), focusing specifically on its occurrence and management in patients with inflammatory bowel disease (IBD).

Methods: An exhaustive literature search was conducted in PubMed, Embase, Cochrane Database of Systematic Reviews, and Google Scholar to identify relevant studies on pSpA in IBD patients. Titles, abstracts, and full-text articles were screened for relevance.

From Preformulative Design to Tests: A Complex Path of Requisites and Studies for Nanoparticle Ocular Application. Part 2: , , and Studies.

The incidence of ocular pathologies is constantly increasing, as is the interest of the researchers in developing new strategies to ameliorate the treatment of these conditions. Nowadays, drug delivery systems are considered among the most relevant approaches due to their applicability in the treatment of a great variety of inner and outer eye pathologies through painless topical administrations. The design of such nanocarriers requires a deep study of many aspects related to the administration route but also a consideration of the authorities and pharmacopeial requirements, in order to achieve a clinical outcome.

Daily rhythms of locomotor activity and transcript levels of non-visual opsins in the brain of the blind Mexican cavefish (Astyanax mexicanus).

Most organisms possess endogenous circadian clocks that synchronise their physiology and behaviour with environmental cycles, with the light-dark (LD) cycle being the most potent synchronising signal. Consequently, it can be hypothesised that animals that have evolved in the dark, as in caves or deep sea, may no longer possess a functional light-entrained biological clock. In this research, the blind cavefish Astyanax mexicanus was selected as a model organism to investigate the potential effects of daily light conditions on the circadian timekeeping mechanisms.

PASSing to the patient side: early achieving of an acceptable symptom state in patients with rheumatoid arthritis treated with Janus kinase inhibitors.

Objective: Patients Acceptable Symptom State (PASS) is a single dichotomized question assessing health satisfaction. We aimed to investigate PASS achievement within 4 weeks of treatment with Janus kinase (JAK) inhibitors (Jakinibs) and its association with treatment response after 4 and 12 weeks in rheumatoid arthritis (RA) patients.

Methods: We recruited consecutive RA patients starting baricitinib or tofacitinib.

Auto-Abs neutralizing type I IFNs in patients with severe Powassan, Usutu, or Ross River virus disease.

Arboviral diseases are a growing global health concern. Pre-existing autoantibodies (auto-Abs) neutralizing type I interferons (IFNs) can underlie encephalitis due to West Nile virus (WNV) (∼40% of patients) and tick-borne encephalitis (TBE, due to TBE virus [TBEV]) (∼10%). We report here that these auto-Abs can also underlie severe forms of rarer arboviral infections.

The Identification of New Pharmacological Targets for the Treatment of Glaucoma: A Network Pharmacology Approach.

Glaucoma is a progressive optic neuropathy characterized by the neurodegeneration and death of retinal ganglion cells (RGCs), leading to blindness. Current glaucoma interventions reduce intraocular pressure but do not address retinal neurodegeneration. In this effort, to identify new pharmacological targets for glaucoma management, we employed a network pharmacology approach.

Hepatitis E Virus Infection in Patients with Systemic and Cutaneous Lupus Erythematosus.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by a multifactorial etiology in which genetic and environmental factors interplay. An exclusively cutaneous condition has been described and defined as cutaneous lupus erythematosus (CLE). In Italy, a nationwide blood donor survey found an overall HEV prevalence of 8.