IRIS
VOLPI, STEFANO
 Distribuzione geografica
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Continente #
EU - Europa 8.390
AS - Asia 38
NA - Nord America 2
Totale 8.430
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Nazione #
IT - Italia 8.390
CN - Cina 32
SG - Singapore 4
HK - Hong Kong 2
US - Stati Uniti d'America 2
Totale 8.430
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Città #
Genoa 3.604
Genova 3.012
Vado Ligure 988
Rapallo 753
Bordighera 33
Beijing 9
Singapore 3
Ashburn 2
Hong Kong 2
Totale 8.406
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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 200
Analysis of pulmonary features and treatment approaches in the COPA syndrome 189
T cell defects in patients with ARPC1B germline mutations account for their combined immunodeficiency 180
Type I interferon pathway activation in COPA syndrome 149
Bone marrow-derived mesenchymal stem cells induce both polyclonal expansion and differentiation of B cells isolated from healthy donors and systemic lupus erythematosus patients 144
Enhancement of Muscle T Regulatory Cells and Improvement of Muscular Dystrophic Process in mdx Mice by Blockade of Extracellular ATP/P2X Axis. 143
B cell-intrinsic deficiency of the Wiskott-Aldrich syndrome protein (WASp) causes severe abnormalities of the peripheral B-cell compartment in mice 140
A three-dimensional model of human lung development and disease from pluripotent stem cells 139
Inborn Errors of RNA Lariat Metabolism in Humans with Brainstem Viral Infection 137
EXTL3 mutations cause skeletal dysplasia, immune deficiency, and developmental delay 134
Novel genome-editing tools to model and correct primary immunodeficiencies 132
Type i interferon-mediated autoinflammation due to DNase II deficiency 132
Life-threatening influenza and impaired interferon amplification in human IRF7 deficiency 131
A combined immunodeficiency with severe infections, inflammation, and allergy caused by ARPC1B deficiency 131
Circulating follicular helper and follicular regulatory T cells are severely compromised in human CD40 deficiency: A case report 130
Next-Generation Sequencing Reveals Restriction and Clonotypic Expansion of Treg Cells in Juvenile Idiopathic Arthritis 126
The danger signal extracellular ATP is involved in the immunomediated damage of α-sarcoglycan deficient muscular dystrophy 126
Efficacy and Adverse Events During Janus Kinase Inhibitor Treatment of SAVI Syndrome 122
Deletion of WASp and N-WASp in B cells cripples the germinal center response and results in production of IgM autoantibodies 121
Hyperactivated PI3Kδ promotes self and commensal reactivity at the expense of optimal humoral immunity 119
N-WASP is required for B-cell-mediated autoimmunity in Wiskott-Aldrich syndrome 116
Type I interferonopathies in pediatric rheumatology 116
Disease-associated mutations identify a novel region in human STING necessary for the control of type I interferon signaling 116
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Dependence of immunoglobulin class switch recombination in B cells on vesicular release of ATP and CD73 ectonucleotidase activity. 106
Broad spectrum of autoantibodies in patients with Wiskott-Aldrich syndrome and X-linked thrombocytopenia 105
Immunophenotype Anomalies Predict the Development of Autoimmune Cytopenia in 22q11.2 Deletion Syndrome 105
A critical view on autoantibodies in lupus nephritis: Concrete knowledge based on evidence 100
Allele-specific regulation of primary cilia function by the von Hippel-Lindau tumor suppressor 100
Human iPSC-derived trigeminal neurons lack constitutive TLR3-dependent immunity that protects cortical neurons from HSV-1 infection 99
Efficacy of early anti-inflammatory treatment with high doses of intravenous anakinra with or without glucocorticoids in patients with severe COVID-19 pneumonia 96
Powering the immune system: Mitochondria in immune function and deficiency 95
When neonatal inflammation does not mean infection: an early-onset mevalonate kinase deficiency with interstitial lung disease 94
A novel LC–MS/MS-based method for the diagnosis of ADA2 deficiency from dried plasma spot 93
Pseudomonas aeruginosa severe skin infection in a toddler with X-linked agammaglobulinemia due to a novel BTK mutation 90
Spontaneous NLRP3 inflammasome-driven IL-1-β secretion is induced in severe COVID-19 patients and responds to anakinra treatment 89
Predisposition to infection and SIRS in mitochondrial disorders: 8 years' experience in an academic center 89
Overview of STING-Associated Vasculopathy with Onset in Infancy (SAVI) Among 21 Patients 89
Serum IgG2 antibody multi-composition in systemic lupus erythematosus and in lupus nephritis (Part 2): prospective study 85
Clinical characterization, long-term follow-up, and response to treatment of patients with syndrome of undifferentiated recurrent fever (SURF) 84
Neutrophil Extracellular Traps in Systemic Lupus Erythematosus Stimulate IgG2 Production From B Lymphocytes 83
Safety and efficacy of early high-dose IV anakinra in severe COVID-19 lung disease 82
Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation 79
Neutrophil extracellular traps-dnase balance and autoimmunity 79
Expanding the clinical and neuroimaging features of post-varicella arteriopathy of childhood 78
Plasma DNA Profile Associated with DNASE1L3 Gene Mutations: Clinical Observations, Relationships to Nuclease Substrate Preference, and In Vivo Correction 77
LONG-TERM OUTCOME IN CEREBRAL ARTERIA ISCHEMIC STROKE (AIS) DUE TO VARICELLA ZOSTER VIRUS (VZV) IN CHILDREN 76
Actin Remodeling Defects Leading to Autoinflammation and Immune Dysregulation 76
Long-term follow-up of 168 patients with X-linked agammaglobulinemia reveals increased morbidity and mortality 75
Activated PI3Kδ breaches multiple B cell tolerance checkpoints and causes autoantibody production 75
Autoantibody-mediated impairment of DNASE1L3 activity in sporadic systemic lupus erythematosus 75
The challenge of early diagnosis of autoimmune lymphoproliferative syndrome in children with suspected autoinflammatory/autoimmune disorders 74
Progression of non-hematologic manifestations in SAMD9L-associated autoinflammatory disease (SAAD) after hematopoietic stem cell transplantation 74
Type I interferon activation in RAS-associated autoimmune leukoproliferative disease (RALD) 74
A Novel LC-MS/MS Method for Therapeutic Drug Monitoring of Baricitinib in Plasma of Pediatric Patients 68
Monogenetic causes of chilblains, panniculitis and vasculopathy: the Type I interferonopathies 66
Activated phosphoinositide 3-dinase delta syndrome (APDS): An update 66
Syndrome of undifferentiated recurrent fever (Surf): An emerging group of autoinflammatory recurrent fevers 63
Pyrin Inflammasome Activation Defines Colchicine-Responsive SURF Patients from FMF and Other Recurrent Fevers 62
Second Wave Antibodies in Autoimmune Renal Diseases: The Case of Lupus Nephritis 62
Adenosine Deaminase 2 Deficiency (DADA2): A Crosstalk Between Innate and Adaptive Immunity 61
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 61
Neutrophil Extracellular Traps in the Autoimmunity Context 59
On the Alert for Cytokine Storm: Immunopathology in COVID-19 59
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) 59
Spontaneous pregnancy after hematopoietic stem cell transplantation for chronic granulomatous disease 58
Haploidentical α/β T-cell and B-cell depleted stem cell transplantation in severe mevalonate kinase deficiency 58
Blood Lymphocyte Subsets and Proinflammatory Cytokine Profile in ROHHAD(NET) and non-ROHHAD(NET) Obese Individuals 57
Recent insight into SARS-COV2 immunopathology and rationale for potential treatment and preventive strategies in COVID-19 57
Collapsing Glomerulopathy as a Complication of Type I Interferon–Mediated Glomerulopathy in a Patient With RNASEH2B-Related Aicardi-Goutières Syndrome 56
An atypical case of post-varicella stroke in a child presenting with hemichorea followed by late-onset inflammatory focal cerebral arteriopathy 55
Effect of anakinra on mortality in patients with COVID-19: a systematic review and patient-level meta-analysis 55
Targeted ngs yields plentiful ultra-rare variants in inborn errors of immunity patients 55
Primary atopic disorders and chronic skin disease 55
Next generation sequencing reveals skewing of the T and B cell receptor repertoires in patients with Wiskott-Aldrich syndrome 54
A Report of 2 Cases of Kidney Involvement in ADA2 Deficiency: Different Disease Phenotypes and the Tissue Response to Type I Interferon 53
Case Report: Multisystem inflammatory syndrome in children with associated proximal tubular injury 53
Inherited defects in the complement system 53
Immunological basis of virus-host interaction in COVID-19 52
Neutrophil extracellular traps profiles in patients with incident systemic lupus erythematosus and lupus nephritis 52
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) 52
An Update on Familial Mediterranean Fever 50
Genotype-Phenotype Correlation and Functional Insights for Two Monoallelic TREX1 Missense Variants Affecting the Catalytic Core 48
Baricitinib treatment in children with COPA syndrome 47
ARF1 prevents aberrant type I interferon induction by regulating STING activation and recycling 46
Dysregulation in B-cell responses and T follicular helper cell function in ADA2 deficiency patients 44
The Notch1/CD22 signaling axis disrupts Treg function in SARS-CoV-2-associated multisystem inflammatory syndrome in children 43
Case Report: Susceptibility to viral infections and secondary hemophagocytic lymphohistiocytosis responsive to intravenous immunoglobulin as primary manifestations of adenosine deaminase 2 deficiency 43
Recurrence of previous chilblain lesions during the second wave of COVID-19: can we still doubt the correlation with SARS-CoV-2? 43
Proteomic Signatures of Monocytes in Hereditary Recurrent Fevers 42
Nuclease deficiencies alter plasma cell-free DNA methylation profiles 42
The Inborn Errors of Immunity—Virtual Consultation System Platform in Service for the Italian Primary Immunodeficiency Network: Results from the Validation Phase 40
Inborn errors of immunity with atopic phenotypes: A practical guide for allergists 39
DADA-ism (2) 38
Vasculitis and vasculopathy associated with inborn errors of immunity: an overview 37
Hematopoietic stem cell transplantation for inborn errors of immunity: 30-year single-center experience 36
Remission of eczema and recovery of Th1 polarization following treatment with Dupilumab in STAT3 hyper IgE syndrome 34
Efficacy of High-Dose Intravenous Anakinra in Pediatric TAFRO Syndrome: Report of Two Cases and Literature Review 34
Interleukin-1 blockade in patients with Wiskott-Aldrich syndrome: a retrospective multinational case series 31
Totale 8.291
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Categoria #
all - tutte 38.191
article - articoli 38.191
book - libri 0
conference - conferenze 0
curatela - curatele 0
other - altro 0
patent - brevetti 0
selected - selezionate 0
volume - volumi 0
Totale 76.382
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Totale Lug Ago Sett Ott Nov Dic Gen Feb Mar Apr Mag Giu
2020/2021556 0 0 0 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
2023/2024743 16 58 34 86 52 106 69 58 62 39 65 98
2024/20252.618 155 177 122 165 298 256 293 442 120 132 249 209
2025/20261.294 599 146 271 278 0 0 0 0 0 0 0 0
Totale 8.769