Andrea Finocchi
A5030333997- Immunodeficiency and Autoimmune Disorders
- Immune Cell Function and Interaction
- Blood disorders and treatments
- Neutrophil, Myeloperoxidase and Oxidative Mechanisms
- T-cell and B-cell Immunology
- Chronic Lymphocytic Leukemia Research
- Virus-based gene therapy research
- DNA Repair Mechanisms
- Parvovirus B19 Infection Studies
- Blood groups and transfusion
- CAR-T cell therapy research
- Hematopoietic Stem Cell Transplantation
- Diabetes and associated disorders
- Platelet Disorders and Treatments
- Inflammatory Bowel Disease
- Neutropenia and Cancer Infections
- Cystic Fibrosis Research Advances
- SARS-CoV-2 and COVID-19 Research
- Atherosclerosis and Cardiovascular Diseases
- Nitric Oxide and Endothelin Effects
- Neonatal Respiratory Health Research
- Immune Response and Inflammation
- Gastrointestinal disorders and treatments
- Genetic Neurodegenerative Diseases
- Pneumocystis jirovecii pneumonia detection and treatment
University of Rome Tor Vergata
2016-2025
Bambino Gesù Children's Hospital
2016-2025
Istituti di Ricovero e Cura a Carattere Scientifico
2010-2024
Federico II University Hospital
2022-2024
University of Brescia
2009-2022
University of Milan
2009-2022
Sapienza University of Rome
2006-2022
Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico
2015-2022
Weatherford College
2022
Azienda Ospedaliera Universitaria Pisana
2022
Wiskott-Aldrich syndrome (WAS) is an inherited immunodeficiency caused by mutations in the gene encoding WASP, a protein regulating cytoskeleton. Hematopoietic stem/progenitor cell (HSPC) transplants can be curative, but, when matched donors are unavailable, infusion of autologous HSPCs modified ex vivo therapy alternative approach. We used lentiviral vector functional WASP to genetically correct from three WAS patients and reinfused cells after reduced-intensity conditioning regimen. All...
Hemophagocytic lymphohistiocytosis (HLH) is characterized by immune dysregulation due to inadequate restraint of overactivated cells and associated with a variable clinical spectrum having overlap more common pathophysiologies. HLH difficult diagnose can be part inflammatory syndromes. Here, we identify novel hematological/autoinflammatory condition (NOCARH syndrome) in four unrelated patients superimposable features, including neonatal-onset cytopenia dyshematopoiesis, autoinflammation,...
STAT6 (signal transducer and activator of transcription 6) is a factor that plays central role in the pathophysiology allergic inflammation. We have identified 16 patients from 10 families spanning three continents with profound phenotype early-life onset immune dysregulation, widespread treatment-resistant atopic dermatitis, hypereosinophilia esosinophilic gastrointestinal disease, asthma, elevated serum IgE, IgE-mediated food allergies, anaphylaxis. The cases were either sporadic (seven...
Abstract Background Ataxia Teleangiectasia [AT] is a rare neurodegenerative disease characterized by early onset ataxia, oculocutaneous teleangiectasias, immunodeficiency, recurrent infections, radiosensitivity and proneness to cancer. No therapies are available for this devastating disease. Recent observational studies in few patients showed beneficial effects of short term treatment with betamethasone. To avoid the characteristic side long-term administration steroids we developed method...
Background— NADPH oxidase is believed to modulate arterial tone, but its role in humans still unclear. The objective of this study was evaluate whether involved flow-mediated dilation (FMD). Methods and Results— Twenty-five patients with hereditary deficiency gp91 phox , the catalytic core oxidase, (X-CGD), 25 healthy subjects, obese matched for sex age were recruited. FMD, platelet serum levels nitrite nitrate as markers nitric oxide generation, oxidized low-density lipoprotein, urinary...
Platelet isoprostane 8-ISO-prostaglandin F2α (8-iso-PGF2α), a proaggregating molecule, is believed to derive from nonenzymatic oxidation of arachidonic acid. We hypothesized that NADPH implicated in formation and platelet activation.We studied 8-iso-PGF2α platelets 8 male patients with hereditary deficiency gp91(phox), the catalytic subunit oxidase, controls. On stimulation, controls produced 8-iso-PGF2α, which was inhibited -8% by aspirin -58% specific inhibitor gp91(phox). Platelets...
Differences in B and T cell repertoires patients with RAG deficiency associate clinical severity.
Mass SARS-Cov-2 vaccination campaign represents the only strategy to defeat global pandemic we are facing. Immunocompromised patients represent a vulnerable population at high risk of developing severe COVID-19 and thus should be prioritized in programs study vaccine efficacy. Nevertheless, most data on efficacy safety available vaccines derive from trials conducted healthy individuals; hence, studies immunogenicity SARS-CoV2 such populations deeply needed. Here, perform an observational...
Memory B cells (MBCs) epitomize the adaptation of immune system to environment. We identify two MBC subsets in peripheral blood, CD27dull and CD27bright MBCs, whose frequency changes with age. Heavy chain variable region (VH) usage, somatic mutation replacement-to-silent ratio, CDR3 property changes, reflecting consecutive selection highly antigen-specific, low cross-reactive antibody variants, all demonstrate that MBCs represent sequential developmental stages, stringent antigen-driven...
CD40 ligand (CD40L) expression on platelets is mediated by agonists, but the underlying mechanism still unclear.CD40L was measured in from healthy subjects both with and without addition of antioxidants or a phospholipase A2 (PLA2) inhibitor 2 patients an inherited deficiency gp91phox. Immunoprecipitation analysis also performed to determine whether normal showed gp91phox expression. Unlike catalase mannitol, superoxide dismutase inhibited agonist-induced platelet CD40L subjects. that...
Abstract No controlled studies exist regarding the pharmaceutical reduction of ataxia symptoms in telangiectasia (A‐T). In a multicenter, double‐blind, randomized, placebo‐controlled crossover trial, oral betamethasone (BETA) and placebo were compared terms their as assessed with International Cooperative Ataxia Rating Scale (ICARS). this study 13 A‐T children, reduced ICARS total score by median points intent‐to‐treat population 16 per‐protocol (ie, percent decreases 28% 31%, respectively)....
Gene therapy with ex vivo-transduced hematopoietic stem/progenitor cells may represent a valid therapeutic option for monogenic immunohematological disorders such as Wiskott-Aldrich syndrome (WAS), primary immunodeficiency associated thrombocytopenia. We evaluated the preclinical safety and efficacy of human CD34+ transduced lentiviral vectors (LV) encoding WAS protein (WASp). first set up validated transduction protocol derived from bone marrow (BM) or mobilized peripheral blood (MPB) using...
Ataxia-telangiectasia (AT) is a rare, devastating neurodegenerative disease presenting with early-onset ataxia, oculocutaneous telangiectasia, immunodeficiency, radiosensitivity, and proneness to cancer. In previous phase 2 study, we showed that 6 monthly infusions of autologous erythrocytes loaded dexamethasone (EryDex; EryDel, Urbino, Italy) were effective in improving neurologic impairment young patients AT. The present article reports the results extension this study for an additional...
As the global COVID-19 pandemic progresses, it is paramount to gain knowledge on adaptive immunity SARS-CoV-2 in children define immune correlates of protection upon immunization or infection. We analyzed anti-SARS-CoV-2 antibodies and their neutralizing activity (PRNT) 66 COVID-19-infected at 7 (±2) days after symptom onset. Individuals with specific humoral responses presented faster virus clearance lower viral load associated a reduced vitro infectivity. demonstrated that frequencies...