IRIS
VOLPI, STEFANO
 Distribuzione geografica
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Continente #
EU - Europa 4.763
Totale 4.763
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Nazione #
IT - Italia 4.763
Totale 4.763
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Città #
Genova 3.012
Genoa 908
Rapallo 753
Vado Ligure 56
Bordighera 34
Totale 4.763
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Nome #
T cell defects in patients with ARPC1B germline mutations account for their combined immunodeficiency 139
CD70 deficiency due to a novel mutation in a patient with severe chronic EBV infection presenting as a periodic fever 125
Type I interferon pathway activation in COPA syndrome 121
Analysis of pulmonary features and treatment approaches in the COPA syndrome 121
Bone marrow-derived mesenchymal stem cells induce both polyclonal expansion and differentiation of B cells isolated from healthy donors and systemic lupus erythematosus patients 118
Enhancement of Muscle T Regulatory Cells and Improvement of Muscular Dystrophic Process in mdx Mice by Blockade of Extracellular ATP/P2X Axis. 117
Inborn Errors of RNA Lariat Metabolism in Humans with Brainstem Viral Infection 112
Circulating follicular helper and follicular regulatory T cells are severely compromised in human CD40 deficiency: A case report 110
Type i interferon-mediated autoinflammation due to DNase II deficiency 106
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 105
Novel genome-editing tools to model and correct primary immunodeficiencies 104
Life-threatening influenza and impaired interferon amplification in human IRF7 deficiency 103
A three-dimensional model of human lung development and disease from pluripotent stem cells 102
Disease-associated mutations identify a novel region in human STING necessary for the control of type I interferon signaling 101
The danger signal extracellular ATP is involved in the immunomediated damage of α-sarcoglycan deficient muscular dystrophy 101
Hyperactivated PI3Kδ promotes self and commensal reactivity at the expense of optimal humoral immunity 100
Deletion of WASp and N-WASp in B cells cripples the germinal center response and results in production of IgM autoantibodies 99
Dysregulation in B-cell responses and T follicular helper cell function in ADA2 deficiency patients 99
B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice 97
Next-Generation Sequencing Reveals Restriction and Clonotypic Expansion of Treg Cells in Juvenile Idiopathic Arthritis 96
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia 96
Efficacy and Adverse Events During Janus Kinase Inhibitor Treatment of SAVI Syndrome 96
Type I interferonopathies in pediatric rheumatology 90
N-WASP is required for B-cell-mediated autoimmunity in Wiskott-Aldrich syndrome 89
Human iPSC-derived trigeminal neurons lack constitutive TLR3-dependent immunity that protects cortical neurons from HSV-1 infection 89
A combined immunodeficiency with severe infections, inflammation, and allergy caused by ARPC1B deficiency 87
Dependence of immunoglobulin class switch recombination in B cells on vesicular release of ATP and CD73 ectonucleotidase activity. 85
Immunophenotype Anomalies Predict the Development of Autoimmune Cytopenia in 22q11.2 Deletion Syndrome 83
Powering the immune system: Mitochondria in immune function and deficiency 77
Predisposition to infection and SIRS in mitochondrial disorders: 8 years' experience in an academic center 75
Allele-specific regulation of primary cilia function by the von Hippel-Lindau tumor suppressor 73
When neonatal inflammation does not mean infection: an early-onset mevalonate kinase deficiency with interstitial lung disease 73
Overview of STING-Associated Vasculopathy with Onset in Infancy (SAVI) Among 21 Patients 64
Next generation sequencing panel in undifferentiated autoinflammatory diseases identify patients with colchicine-responder recurrent fevers 61
Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation 60
Pseudomonas aeruginosa severe skin infection in a toddler with X-linked agammaglobulinemia due to a novel BTK mutation 53
Safety and efficacy of early high-dose IV anakinra in severe COVID-19 lung disease 52
Consensus statement of the Italian society of pediatric allergy and immunology for the pragmatic management of children and adolescents with allergic or immunological diseases during the COVID-19 pandemic 52
Efficacy of early anti-inflammatory treatment with high doses of intravenous anakinra with or without glucocorticoids in patients with severe COVID-19 pneumonia 47
Serum IgG2 antibody multi-composition in systemic lupus erythematosus and in lupus nephritis (Part 2): prospective study 45
The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders 45
Neutrophil extracellular traps-dnase balance and autoimmunity 44
Neutrophil Extracellular Traps in Systemic Lupus Erythematosus Stimulate IgG2 Production From B Lymphocytes 43
Monogenetic causes of chilblains, panniculitis and vasculopathy: the Type I interferonopathies 42
Long-term follow-up of 168 patients with X-linked agammaglobulinemia reveals increased morbidity and mortality 41
Clinical characterization, long-term follow-up, and response to treatment of patients with syndrome of undifferentiated recurrent fever (SURF) 39
Activated PI3Kδ breaches multiple B cell tolerance checkpoints and causes autoantibody production 39
Plasma DNA Profile Associated with DNASE1L3 Gene Mutations: Clinical Observations, Relationships to Nuclease Substrate Preference, and In Vivo Correction 39
Next generation sequencing reveals skewing of the T and B cell receptor repertoires in patients with Wiskott-Aldrich syndrome 38
On the Alert for Cytokine Storm: Immunopathology in COVID-19 37
Spontaneous pregnancy after hematopoietic stem cell transplantation for chronic granulomatous disease 37
Immunological basis of virus-host interaction in COVID-19 36
A novel LC–MS/MS-based method for the diagnosis of ADA2 deficiency from dried plasma spot 36
Expanding the clinical and neuroimaging features of post-varicella arteriopathy of childhood 35
Recent insight into SARS-COV2 immunopathology and rationale for potential treatment and preventive strategies in COVID-19 34
Activated phosphoinositide 3-dinase delta syndrome (APDS): An update 34
Actin Remodeling Defects Leading to Autoinflammation and Immune Dysregulation 32
Syndrome of undifferentiated recurrent fever (Surf): An emerging group of autoinflammatory recurrent fevers 32
Type I interferon activation in RAS-associated autoimmune leukoproliferative disease (RALD) 31
Haploidentical α/β T-cell and B-cell depleted stem cell transplantation in severe mevalonate kinase deficiency 31
Second Wave Antibodies in Autoimmune Renal Diseases: The Case of Lupus Nephritis 30
Effect of anakinra on mortality in patients with COVID-19: a systematic review and patient-level meta-analysis 30
Progression of non-hematologic manifestations in SAMD9L-associated autoinflammatory disease (SAAD) after hematopoietic stem cell transplantation 30
Neutrophil extracellular traps profiles in patients with incident systemic lupus erythematosus and lupus nephritis 29
Erratum: Haploidentical α/β T-cell and B-cell depleted stem cell transplantation in severe mevalonate kinase deficiency (Rheumatology (2021) DOI: 10.1093/rheumatology/keaa912) 29
Inherited defects in the complement system 29
Autoantibody-mediated impairment of DNASE1L3 activity in sporadic systemic lupus erythematosus 29
Primary atopic disorders and chronic skin disease 29
Collapsing Glomerulopathy as a Complication of Type I Interferon–Mediated Glomerulopathy in a Patient With RNASEH2B-Related Aicardi-Goutières Syndrome 28
Spontaneous NLRP3 inflammasome-driven IL-1-β secretion is induced in severe COVID-19 patients and responds to anakinra treatment 26
Erratum: Next generation sequencing panel in undifferentiated autoinflammatory diseases identifies patients with colchicine-responder recurrent fevers (Rheumatology (2020) 59 (344-60) DOI: 10.1093/rheumatology/kez270) 26
LONG-TERM OUTCOME IN CEREBRAL ARTERIA ISCHEMIC STROKE (AIS) DUE TO VARICELLA ZOSTER VIRUS (VZV) IN CHILDREN 21
Targeted ngs yields plentiful ultra-rare variants in inborn errors of immunity patients 21
Adenosine Deaminase 2 Deficiency (DADA2): A Crosstalk Between Innate and Adaptive Immunity 17
Recurrence of previous chilblain lesions during the second wave of COVID-19: can we still doubt the correlation with SARS-CoV-2? 17
The Notch1/CD22 signaling axis disrupts Treg function in SARS-CoV-2-associated multisystem inflammatory syndrome in children 16
Pyrin Inflammasome Activation Defines Colchicine-Responsive SURF Patients from FMF and Other Recurrent Fevers 15
An atypical case of post-varicella stroke in a child presenting with hemichorea followed by late-onset inflammatory focal cerebral arteriopathy 15
Nuclease deficiencies alter plasma cell-free DNA methylation profiles 15
Inborn errors of immunity with atopic phenotypes: A practical guide for allergists 15
Neutrophil Extracellular Traps in the Autoimmunity Context 13
DADA-ism (2) 13
Proteomic Signatures of Monocytes in Hereditary Recurrent Fevers 12
Genotype-Phenotype Correlation and Functional Insights for Two Monoallelic TREX1 Missense Variants Affecting the Catalytic Core 11
New monogenic autoinflammatory diseases: 2021 year in review 10
A Report of 2 Cases of Kidney Involvement in ADA2 Deficiency: Different Disease Phenotypes and the Tissue Response to Type I Interferon 9
An Update on Familial Mediterranean Fever 9
Immune tolerance breakdown in inborn errors of immunity: Paving the way to novel therapeutic approaches 8
Remission of eczema and recovery of Th1 polarization following treatment with Dupilumab in STAT3 hyper IgE syndrome 8
Case Report: Susceptibility to viral infections and secondary hemophagocytic lymphohistiocytosis responsive to intravenous immunoglobulin as primary manifestations of adenosine deaminase 2 deficiency 8
COVID-19 and immune response: Weak defences and self-harms,Covid-19 e risposta immune: Tra debolezze in difesa ed errori in attacco 8
Serum IgG2 antibody multicomposition in systemic lupus erythematosus and lupus nephritis (Part 1): cross-sectional analysis 7
ARF1 prevents aberrant type I interferon induction by regulating STING activation and recycling 6
Activated PI3Kd breaches multiple B cell tolerance checkpoints and causes autoantibody production 6
Novel heterozygous TREX1 mutation in a juvenile systemic lupus erythematosus patient with severe cutaneous involvement treated successfully with Jak-inhibitors: a case report 6
The Inborn Errors of Immunity—Virtual Consultation System Platform in Service for the Italian Primary Immunodeficiency Network: Results from the Validation Phase 6
Hematopoietic Stem Cell Transplantation in ARPC1B Deficiency 6
Vasculitis and vasculopathy associated with inborn errors of immunity: an overview 5
A Novel LC-MS/MS Method for Therapeutic Drug Monitoring of Baricitinib in Plasma of Pediatric Patients 5
HSP90β controls NLRP3 autoactivation 5
Totale 4.996
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Categoria #
all - tutte 22.360
article - articoli 22.360
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 0
Totale 44.720
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2019/20201.249 0 24 21 84 95 131 241 150 162 192 123 26
2020/2021722 33 31 102 87 20 56 108 58 49 66 66 46
2021/2022802 18 11 50 64 35 76 22 163 69 118 68 108
2022/2023911 84 84 16 81 156 136 9 69 150 7 105 14
2023/2024747 16 58 35 87 52 106 69 59 62 39 66 98
2024/2025174 157 17 0 0 0 0 0 0 0 0 0 0
Totale 5.035