IRIS
VOLPI, STEFANO
 Distribuzione geografica
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Continente #
EU - Europa 5.735
Totale 5.735
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Nazione #
IT - Italia 5.735
Totale 5.735
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Città #
Genova 3.012
Vado Ligure 994
Genoa 942
Rapallo 753
Bordighera 34
Totale 5.735
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Nome #
CD70 deficiency due to a novel mutation in a patient with severe chronic EBV infection presenting as a periodic fever 160
T cell defects in patients with ARPC1B germline mutations account for their combined immunodeficiency 153
Analysis of pulmonary features and treatment approaches in the COPA syndrome 142
Type I interferon pathway activation in COPA syndrome 127
Enhancement of Muscle T Regulatory Cells and Improvement of Muscular Dystrophic Process in mdx Mice by Blockade of Extracellular ATP/P2X Axis. 126
Bone marrow-derived mesenchymal stem cells induce both polyclonal expansion and differentiation of B cells isolated from healthy donors and systemic lupus erythematosus patients 125
Inborn Errors of RNA Lariat Metabolism in Humans with Brainstem Viral Infection 123
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 118
Type i interferon-mediated autoinflammation due to DNase II deficiency 116
Circulating follicular helper and follicular regulatory T cells are severely compromised in human CD40 deficiency: A case report 116
Novel genome-editing tools to model and correct primary immunodeficiencies 115
Next-Generation Sequencing Reveals Restriction and Clonotypic Expansion of Treg Cells in Juvenile Idiopathic Arthritis 114
Life-threatening influenza and impaired interferon amplification in human IRF7 deficiency 112
null 111
The danger signal extracellular ATP is involved in the immunomediated damage of α-sarcoglycan deficient muscular dystrophy 109
Deletion of WASp and N-WASp in B cells cripples the germinal center response and results in production of IgM autoantibodies 108
A three-dimensional model of human lung development and disease from pluripotent stem cells 108
Hyperactivated PI3Kδ promotes self and commensal reactivity at the expense of optimal humoral immunity 108
Disease-associated mutations identify a novel region in human STING necessary for the control of type I interferon signaling 107
B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice 105
Efficacy and Adverse Events During Janus Kinase Inhibitor Treatment of SAVI Syndrome 105
N-WASP is required for B-cell-mediated autoimmunity in Wiskott-Aldrich syndrome 102
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia 102
A combined immunodeficiency with severe infections, inflammation, and allergy caused by ARPC1B deficiency 99
Type I interferonopathies in pediatric rheumatology 97
Human iPSC-derived trigeminal neurons lack constitutive TLR3-dependent immunity that protects cortical neurons from HSV-1 infection 96
Dependence of immunoglobulin class switch recombination in B cells on vesicular release of ATP and CD73 ectonucleotidase activity. 90
Immunophenotype Anomalies Predict the Development of Autoimmune Cytopenia in 22q11.2 Deletion Syndrome 88
Powering the immune system: Mitochondria in immune function and deficiency 82
When neonatal inflammation does not mean infection: an early-onset mevalonate kinase deficiency with interstitial lung disease 82
Allele-specific regulation of primary cilia function by the von Hippel-Lindau tumor suppressor 79
Predisposition to infection and SIRS in mitochondrial disorders: 8 years' experience in an academic center 78
Overview of STING-Associated Vasculopathy with Onset in Infancy (SAVI) Among 21 Patients 73
Next generation sequencing panel in undifferentiated autoinflammatory diseases identify patients with colchicine-responder recurrent fevers 70
Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation 66
Pseudomonas aeruginosa severe skin infection in a toddler with X-linked agammaglobulinemia due to a novel BTK mutation 65
Efficacy of early anti-inflammatory treatment with high doses of intravenous anakinra with or without glucocorticoids in patients with severe COVID-19 pneumonia 58
Safety and efficacy of early high-dose IV anakinra in severe COVID-19 lung disease 57
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 56
Neutrophil extracellular traps-dnase balance and autoimmunity 55
Serum IgG2 antibody multi-composition in systemic lupus erythematosus and in lupus nephritis (Part 2): prospective study 52
The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders 51
Monogenetic causes of chilblains, panniculitis and vasculopathy: the Type I interferonopathies 51
Neutrophil Extracellular Traps in Systemic Lupus Erythematosus Stimulate IgG2 Production From B Lymphocytes 50
Clinical characterization, long-term follow-up, and response to treatment of patients with syndrome of undifferentiated recurrent fever (SURF) 49
Activated PI3Kδ breaches multiple B cell tolerance checkpoints and causes autoantibody production 47
Spontaneous pregnancy after hematopoietic stem cell transplantation for chronic granulomatous disease 47
Long-term follow-up of 168 patients with X-linked agammaglobulinemia reveals increased morbidity and mortality 46
Recent insight into SARS-COV2 immunopathology and rationale for potential treatment and preventive strategies in COVID-19 46
Immunological basis of virus-host interaction in COVID-19 46
Progression of non-hematologic manifestations in SAMD9L-associated autoinflammatory disease (SAAD) after hematopoietic stem cell transplantation 45
Next generation sequencing reveals skewing of the T and B cell receptor repertoires in patients with Wiskott-Aldrich syndrome 44
On the Alert for Cytokine Storm: Immunopathology in COVID-19 44
Expanding the clinical and neuroimaging features of post-varicella arteriopathy of childhood 44
Type I interferon activation in RAS-associated autoimmune leukoproliferative disease (RALD) 44
A novel LC–MS/MS-based method for the diagnosis of ADA2 deficiency from dried plasma spot 44
Plasma DNA Profile Associated with DNASE1L3 Gene Mutations: Clinical Observations, Relationships to Nuclease Substrate Preference, and In Vivo Correction 43
Actin Remodeling Defects Leading to Autoinflammation and Immune Dysregulation 43
Activated phosphoinositide 3-dinase delta syndrome (APDS): An update 42
Collapsing Glomerulopathy as a Complication of Type I Interferon–Mediated Glomerulopathy in a Patient With RNASEH2B-Related Aicardi-Goutières Syndrome 40
Syndrome of undifferentiated recurrent fever (Surf): An emerging group of autoinflammatory recurrent fevers 40
Effect of anakinra on mortality in patients with COVID-19: a systematic review and patient-level meta-analysis 39
Inherited defects in the complement system 39
Haploidentical α/β T-cell and B-cell depleted stem cell transplantation in severe mevalonate kinase deficiency 38
Autoantibody-mediated impairment of DNASE1L3 activity in sporadic systemic lupus erythematosus 37
Second Wave Antibodies in Autoimmune Renal Diseases: The Case of Lupus Nephritis 36
Neutrophil extracellular traps profiles in patients with incident systemic lupus erythematosus and lupus nephritis 36
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) 36
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) 35
Primary atopic disorders and chronic skin disease 35
Spontaneous NLRP3 inflammasome-driven IL-1-β secretion is induced in severe COVID-19 patients and responds to anakinra treatment 34
Pyrin Inflammasome Activation Defines Colchicine-Responsive SURF Patients from FMF and Other Recurrent Fevers 31
Targeted ngs yields plentiful ultra-rare variants in inborn errors of immunity patients 29
LONG-TERM OUTCOME IN CEREBRAL ARTERIA ISCHEMIC STROKE (AIS) DUE TO VARICELLA ZOSTER VIRUS (VZV) IN CHILDREN 28
An atypical case of post-varicella stroke in a child presenting with hemichorea followed by late-onset inflammatory focal cerebral arteriopathy 25
Recurrence of previous chilblain lesions during the second wave of COVID-19: can we still doubt the correlation with SARS-CoV-2? 25
Adenosine Deaminase 2 Deficiency (DADA2): A Crosstalk Between Innate and Adaptive Immunity 24
Neutrophil Extracellular Traps in the Autoimmunity Context 23
The Notch1/CD22 signaling axis disrupts Treg function in SARS-CoV-2-associated multisystem inflammatory syndrome in children 21
Nuclease deficiencies alter plasma cell-free DNA methylation profiles 21
Inborn errors of immunity with atopic phenotypes: A practical guide for allergists 21
Vasculitis and vasculopathy associated with inborn errors of immunity: an overview 20
DADA-ism (2) 19
A critical view on autoantibodies in lupus nephritis: Concrete knowledge based on evidence 19
The Inborn Errors of Immunity—Virtual Consultation System Platform in Service for the Italian Primary Immunodeficiency Network: Results from the Validation Phase 18
Proteomic Signatures of Monocytes in Hereditary Recurrent Fevers 17
Hematopoietic Stem Cell Transplantation in ARPC1B Deficiency 17
A Report of 2 Cases of Kidney Involvement in ADA2 Deficiency: Different Disease Phenotypes and the Tissue Response to Type I Interferon 17
Genotype-Phenotype Correlation and Functional Insights for Two Monoallelic TREX1 Missense Variants Affecting the Catalytic Core 17
A Novel LC-MS/MS Method for Therapeutic Drug Monitoring of Baricitinib in Plasma of Pediatric Patients 16
New monogenic autoinflammatory diseases: 2021 year in review 16
Remission of eczema and recovery of Th1 polarization following treatment with Dupilumab in STAT3 hyper IgE syndrome 16
An Update on Familial Mediterranean Fever 16
Immune tolerance breakdown in inborn errors of immunity: Paving the way to novel therapeutic approaches 15
Case Report: Susceptibility to viral infections and secondary hemophagocytic lymphohistiocytosis responsive to intravenous immunoglobulin as primary manifestations of adenosine deaminase 2 deficiency 15
Baricitinib treatment in children with COPA syndrome 14
ARF1 prevents aberrant type I interferon induction by regulating STING activation and recycling 12
Activated PI3Kd breaches multiple B cell tolerance checkpoints and causes autoantibody production 12
Novel heterozygous TREX1 mutation in a juvenile systemic lupus erythematosus patient with severe cutaneous involvement treated successfully with Jak-inhibitors: a case report 12
HSP90β controls NLRP3 autoactivation 12
Totale 5.874
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Categoria #
all - tutte 27.834
article - articoli 27.834
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 0
Totale 55.668
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2019/2020894 0 0 0 0 0 0 241 150 162 192 123 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
2024/20251.181 157 178 124 166 299 257 0 0 0 0 0 0
Totale 6.042