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102. Lymphedema Surgical Treatment Using BioBridgeTM: A Preliminary Experience.

Author

Cè, Maurizio, Menozzi, Andrea, Soresina, Massimo, Giardini, Denisa, Martinenghi, Carlo, and Cellina, Michaela

Subjects
LYMPHEDEMA, MAGNETIC resonance imaging, MAGNETIC resonance, PATIENT experience, LYMPHANGIOGRAPHY, ARACHNOID cysts, SURGICAL meshes, ARTIFICIAL implants
Abstract

Lymphedema treatment has traditionally been based on conservative therapies. In recent years, the introduction of two microsurgical techniques—venolymphatic anastomoses and lymph-node transplantation—have revolutionized the therapeutic approach to this disease. However, these surgical techniques only provide a partial volume reduction, and thus, they require further improvement. A new device called BioBridgeTM, an implantable biocompatible and biodegradable mesh, made up of aligned nanofibrillar collagen scaffolds that mimic the collagen matrix, could significantly improve the benefits of these surgical strategies, favoring lymphatic regeneration. In this article, we describe our experience with three patients affected by primary lymphedema, two treated with a lymph-node transplant, and one with venolymphatic anastomosis. All interventions involved the placement of nanocollagen scaffolds. With each patient, non-contrast magnetic resonance lymphography was performed for surgical planning and there was an imaging and a clinical follow-up at 1 year. All patients showed a significant reduction in the affected areas with the demonstration of new lymphatic vessels at MR imaging follow-up. This was a preliminary experience, and it showed the potential benefit of positioning nonfibrillar scaffolds in combination with other microsurgical therapies for lymphedema. [ABSTRACT FROM AUTHOR]

Published
2023
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103. Clinical and immunological data of nine patients with chronic mucocutaneous candidiasis disease

Author

Laura Dotta, Omar Scomodon, Rita Padoan, Silviana Timpano, Alessandro Plebani, Annarosa Soresina, Vassilios Lougaris, Daniela Concolino, Angela Nicoletti, Giuliana Giardino, Amelia Licari, Gianluigi Marseglia, Claudio Pignata, Nicola Tamassia, Fabio Facchetti, Donatella Vairo, and Raffaele Badolato

Subjects
Computer applications to medicine. Medical informatics, R858-859.7, Science (General), Q1-390
Abstract

This paper describes the heterogeneous clinical phenotype of a cohort of nine patients diagnosed with heterozygous mutations in STAT1. We report data of extended immunophenotyping over time and we show lung damage in four patients. The increased phosphorylation of STAT1 in response to IFNγ and IFNα stimulation proves the gain-of-function nature of the defects. The data are supplemental to our original article concurrently published “Clinical heterogeneity of dominant chronic mucocutaneous candidiasis disease: presenting as treatment-resistant candidiasis and chronic lung disease” [1], where additional results and interpretation of our research can be found.

Published
2016
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105. Milan riots of 1853: history and remembrance

Author

MARCO SORESINA

Subjects
Cultural Studies, 6 February 1853, History, Milan, Mazzini, public history, remembering the Risorgimento, Sociology and Political Science, Political Science and International Relations, Settore M-STO/04 - Storia Contemporanea
Published
2023

108. Circulating Follicular Helper and Follicular Regulatory T Cells Are Severely Compromised in Human CD40 Deficiency: A Case Report

Author

Maria Pia Cicalese, Jolanda Gerosa, Manuela Baronio, Davide Montin, Francesco Licciardi, Annarosa Soresina, Rosa Maria Dellepiane, Maurizio Miano, Lucia Augusta Baselli, Stefano Volpi, Carlo Dufour, Alessandro Plebani, Alessandro Aiuti, Vassilios Lougaris, and Georgia Fousteri

Subjects
hyper-IgM syndrome, follicular helper T cells, follicular regulatory T cells, class switch recombination, somatic hypermutation, AICDA, Immunologic diseases. Allergy, RC581-607
Abstract

Mutations in genes that control class switch recombination and somatic hypermutation during the germinal center (GC) response can cause diverse immune dysfunctions. In particular, mutations in CD40LG, CD40, AICDA, or UNG cause hyper-IgM (HIGM) syndrome, a heterogeneous group of primary immunodeficiencies. Follicular helper (Tfh) and follicular regulatory (Tfr) T cells play a key role in the formation and regulation of GCs, but their role in HIGM pathogenesis is still limited. Here, we found that compared to CD40 ligand (CD40L)- and activation-induced cytidine deaminase (AICDA)-deficient patients, circulating Tfh and Tfr cells were severely compromised in terms of frequency and activation phenotype in a child with CD40 deficiency. These findings offer useful insight for human Tfh biology, with potential implications for understanding the molecular basis of HIGM syndrome caused by mutations in CD40.

Published
2018
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110. Progressive Depletion of B and T Lymphocytes in Patients with Ataxia Telangiectasia: Results of the Italian Primary Immunodeficiency Network

Author

Emilia Cirillo, Agata Polizzi, Annarosa Soresina, Rosaria Prencipe, Giuliana Giardino, Caterina Cancrini, Andrea Finocchi, Beatrice Rivalta, Rosa M. Dellepiane, Lucia A. Baselli, Davide Montin, Antonino Trizzino, Rita Consolini, Chiara Azzari, Silvia Ricci, Lorenzo Lodi, Isabella Quinti, Cinzia Milito, Lucia Leonardi, Marzia Duse, Maria Carrabba, Giovanna Fabio, Patrizia Bertolini, Paola Coccia, Irene D’Alba, Andrea Pession, Francesca Conti, Marco Zecca, Claudio Lunardi, Manuela Lo Bianco, Santiago Presti, Laura Sciuto, Roberto Micheli, Dario Bruzzese, Vassilios Lougaris, Raffaele Badolato, Alessandro Plebani, Luciana Chessa, Claudio Pignata, Cirillo, E., Polizzi, A., Soresina, A., Prencipe, R., Giardino, G., Cancrini, C., Finocchi, A., Rivalta, B., Dellepiane, R. M., Baselli, L. A., Montin, D., Trizzino, A., Consolini, R., Azzari, C., Ricci, S., Lodi, L., Quinti, I., Milito, C., Leonardi, L., Duse, M., Carrabba, M., Fabio, G., Bertolini, P., Coccia, P., D'Alba, I., Pession, A., Conti, F., Zecca, M., Lunardi, C., Bianco, M. L., Presti, S., Sciuto, L., Micheli, R., Bruzzese, D., Lougaris, V., Badolato, R., Plebani, A., Chessa, L., and Pignata, C.

Subjects
B lymphocyte, T-Lymphocytes, genotype, Immunology, T lymphocytes, Ataxia Telangiectasia Mutated Proteins, lymphopenia, primary immunodeficiency, Settore MED/02, Mutation, Humans, Ataxia telangiectasia, B lymphocytes, Immunology and Allergy, Retrospective Studies
Abstract

Ataxia telangiectasia (AT) is a rare neurodegenerative genetic disorder due to bi-allelic mutations in the Ataxia Telangiectasia Mutated (ATM) gene. The aim of this paper is to better define the immunological profile over time, the clinical immune-related manifestations at diagnosis and during follow-up, and to attempt a genotype–phenotype correlation of an Italian cohort of AT patients. Retrospective data of 69 AT patients diagnosed between December 1984 and November 2019 were collected from the database of the Italian Primary Immunodeficiency Network. Patients were classified at diagnosis as lymphopenic (Group A) or non-lymphopenic (Group B). Fifty eight out of 69 AT patients (84%) were genetically characterized and distinguished according to the type of mutations in truncating/truncating (TT; 27 patients), non-truncating (NT)/T (28 patients), and NT/NT (5 patients). In 3 patients, only one mutation was detected. Data on age at onset and at diagnosis, cellular and humoral compartment at diagnosis and follow-up, infectious diseases, signs of immune dysregulation, cancer, and survival were analyzed and compared to the genotype. Lymphopenia at diagnosis was related per se to earlier age at onset. Progressive reduction of cellular compartment occurred during the follow-up with a gradual reduction of T and B cell number. Most patients of Group A carried bi-allelic truncating mutations, had a more severe B cell lymphopenia, and a reduced life expectancy. A trend to higher frequency of interstitial lung disease, immune dysregulation, and malignancy was noted in Group B patients. Lymphopenia at the onset and the T/T genotype are associated with a worst clinical course. Several mechanisms may underlie the premature and progressive immune decline in AT subjects.

Published
2022

111. The Impact of SARS-CoV-2 Infection in Patients with Inborn Errors of Immunity: the Experience of the Italian Primary Immunodeficiencies Network (IPINet)

Author

Giuliana Giardino, Cinzia Milito, Vassilios Lougaris, Alessandra Punziano, Maria Carrabba, Francesco Cinetto, Riccardo Scarpa, Rosa Maria Dellepiane, Silvia Ricci, Beatrice Rivalta, Francesca Conti, Antonio Marzollo, Davide Firinu, Emilia Cirillo, Gianluca Lagnese, Caterina Cancrini, Baldassare Martire, Maria Giovanna Danieli, Andrea Pession, Angelo Vacca, Chiara Azzari, Giovanna Fabio, Annarosa Soresina, Carlo Agostini, Giuseppe Spadaro, Raffaele Badolato, Maria Pia Cicalese, Alessandro Aiuti, Alessandro Plebani, Isabella Quinti, Claudio Pignata, Giardino, Giuliana, Milito, Cinzia, Lougaris, Vassilio, Punziano, Alessandra, Carrabba, Maria, Cinetto, Francesco, Scarpa, Riccardo, Dellepiane, Rosa Maria, Ricci, Silvia, Rivalta, Beatrice, Conti, Francesca, Marzollo, Antonio, Firinu, Davide, Cirillo, Emilia, Lagnese, Gianluca, Cancrini, Caterina, Martire, Baldassare, Danieli, Maria Giovanna, Pession, Andrea, Vacca, Angelo, Azzari, Chiara, Fabio, Giovanna, Soresina, Annarosa, Agostini, Carlo, Spadaro, Giuseppe, Badolato, Raffaele, Cicalese, Maria Pia, Aiuti, Alessandro, Plebani, Alessandro, Quinti, Isabella, and Pignata, Claudio

Subjects
Adult, Male, SARS-CoV-2, COVID-19, Inborn errors of immunity, Outcome, Seroconversion, Viral shedding, Immunology, Hospitalization, Common Variable Immunodeficiency, Post-Acute COVID-19 Syndrome, Settore MED/02, Immunology and Allergy, Humans, Female, Child, Retrospective Studies
Abstract

COVID-19 manifestations range from asymptomatic to life-threatening infections. The outcome in different inborn errors of immunity (IEI) is still a matter of debate. In this retrospective study, we describe the experience of the of the Italian Primary Immunodeficiencies Network (IPINet). Sixteen reference centers for adult or pediatric IEI were involved. One hundred fourteen patients were enrolled including 35 pediatric and 79 adult patients. Median age was 32 years, and male-to-female ratio was 1.5:1. The most common IEI were 22q11.2 deletion syndrome in children (26%) and common variable immunodeficiency (CVID) in adults (65%). Ninety-one patients did not require hospital admission, and among these, 33 were asymptomatic. Hospitalization rate was 20.17%. Older age (p 0.004) and chronic lung disease (p 0.0008) represented risk factors for hospitalization. Hospitalized patients mainly included adults suffering from humoral immunodeficiencies requiring immunoglobulin replacement therapy and as expected had lower B cell counts compared to non-hospitalized patients. Infection fatality rate in the whole cohort was 3.5%. Seroconversion was observed is 86.6% of the patients evaluated and in 83.3% of CVID patients. 16.85% of the patients reported long-lasting COVID symptoms. All but one patient with prolonged symptoms were under IgRT. The fatality rate observed in IEI was slightly similar to the general population. The age of the patients who did not survive was lower compared to the general population, and the age stratified mortality in the 50–60 age range considerable exceeded the mortality from 50 to 60 age group of the Italian population (14.3 vs 0.6%; p

Published
2022

112. The topological line of ABJ(M) theory

Author

Gorini, N, Griguolo, L, Guerrini, L, Penati, S, Seminara, D, Soresina, P, Gorini N., Griguolo L., Guerrini L., Penati S., Seminara D., Soresina P., Gorini, N, Griguolo, L, Guerrini, L, Penati, S, Seminara, D, Soresina, P, Gorini N., Griguolo L., Guerrini L., Penati S., Seminara D., and Soresina P.

Abstract

We construct the one-dimensional topological sector of N = 6 ABJ(M) theory and study its relation with the mass-deformed partition function on S3. Supersymmetric localization provides an exact representation of this partition function as a matrix integral, which interpolates between weak and strong coupling regimes. It has been proposed that correlation functions of dimension-one topological operators should be computed through suitable derivatives with respect to the masses, but a precise proof is still lacking. We present non-trivial evidence for this relation by computing the two-point function at two-loop, successfully matching the matrix model expansion at weak coupling and finite ranks. As a by-product we obtain the two-loop explicit expression for the central charge cT of ABJ(M) theory. Three- and four-point functions up to one-loop confirm the relation as well. Our result points towards the possibility to localize the one-dimensional topological sector of ABJ(M) and may also be useful in the bootstrap program for 3d SCFTs.

Published
2021

115. Noncontrast Magnetic Resonance Lymphography in Secondary Lymphedema Due to Prostate Cancer

Author

Daniele Gibelli, Giancarlo Oliva, Massimo Soresina, Andrea Menozzi, Denisa Giardini, Carlo Martinenghi, and Michaela Cellina

Subjects
Male, medicine.medical_specialty, Magnetic Resonance Spectroscopy, medicine.diagnostic_test, Secondary lymphedema, business.industry, Lymphography, Prostatic Neoplasms, Cancer, Magnetic resonance imaging, medicine.disease, body regions, Prostate cancer, Lymphedema, Lower Extremity, hemic and lymphatic diseases, Quality of Life, medicine, Humans, Radiology, medicine.symptom, Cardiology and Cardiovascular Medicine, business, Retrospective Studies
Abstract

Background: As survival from malignancies continues to improve, a greater emphasis is being placed on the quality of life after cancer treatments. Lymphedema (LE) represents a common and devastatin...

Published
2021

116. Clinical outcome, incidence, and SARS-CoV-2 infection-fatality rates in Italian patients with inborn errors of immunity

Author

Silvia Ricci, Isabella Quinti, Andrea Matucci, Maria Carrabba, Cinzia Milito, Francesco Cinetto, Gianluca Lagnese, Rosa Maria Delle Piane, Angelo Vacca, Alessandro Plebani, Francesca Conti, Alessandro Aiuti, Andrea Pession, Anna Rosa Soresina, Alessandra Vultaggio, Caterina Cancrini, Riccardo Scarpa, Emanuele Vivarelli, Chiara Azzari, Beatrice Rivalta, Maria Pia Cicalese, Davide Firinu, Raffaele Badolato, Maria Giovanna Danieli, Carlo Agostini, Vassilios Lougaris, Antonio Marzollo, Giovanna Fabio, Claudio Pignata, Federica Pulvirenti, Carolina Marasco, Alessandra Punziano, Giuseppe Spadaro, Baldassare Martire, Giuliana Giardino, Lucia Augusta Baselli, Milito, C., Lougaris, V., Giardino, G., Punziano, A., Vultaggio, A., Carrabba, M., Cinetto, F., Scarpa, R., Delle Piane, R. M., Baselli, L., Ricci, S., Rivalta, B., Conti, F., Marasco, C., Marzollo, A., Firinu, D., Pulvirenti, F., Lagnese, G., Vivarelli, E., Cancrini, C., Martire, B., Danieli, M. G., Pession, A., Vacca, A., Azzari, C., Fabio, G., Matucci, A., Soresina, A. R., Agostini, C., Spadaro, G., Badolato, R., Cicalese, M. P., Aiuti, A., Plebani, A., Pignata, C., Quinti, I., Milito, Cinzia, Lougaris, Vassilio, Giardino, Giuliana, Punziano, Alessandra, Vultaggio, Alessandra, Carrabba, Maria, Cinetto, Francesco, Scarpa, Riccardo, Delle Piane, Rosa Maria, Baselli, Lucia, Ricci, Silvia, Rivalta, Beatrice, Conti, Francesca, Marasco, Carolina, Marzollo, Antonio, Firinu, Davide, Pulvirenti, Federica, Lagnese, Gianluca, Vivarelli, Emanuele, Cancrini, Caterina, Martire, Baldassare, Danieli, Maria Giovanna, Pession, Andrea, Vacca, Angelo, Azzari, Chiara, Fabio, Giovanna, Matucci, Andrea, Soresina, Anna Rosa, Agostini, Carlo, Spadaro, Giuseppe, Badolato, Raffaele, Cicalese, Maria Pia, Aiuti, Alessandro, Plebani, Alessandro, Pignata, Claudio, and Quinti, Isabella

Subjects
medicine.medical_specialty, 2019-20 coronavirus outbreak, Humans, Incidence, Italy, COVID-19, SARS-CoV-2, Coronavirus disease 2019 (COVID-19), business.industry, Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2), Incidence (epidemiology), MEDLINE, Clinical Communications, inborn errors of immunity (IEI), Settore MED/02, Immunity, Internal medicine, medicine, Immunology and Allergy, business, Human
Abstract

not available

Published
2021

118. Long term longitudinal follow up of AD-HIES cohort: impact of early diagnosis and enrolment to IPINet centres on natural history of Job's Syndrome

Author

Maria Carrabba, Rosa Maria Dellepiane, Manuela Cortesi, Lucia Augusta Baselli, Annarosa Soresina, Emilia Cirillo, Giuliana Giardino, Francesca Conti, Laura Dotta, Andrea Finocchi, Caterina Cancrini, Cinzia Milito, Lucia Pacillo, Bianca Laura Cinicola, Fausto Cossu, Rita Consolini, Davide Montin, Isabella Quinti, Andrea Pession, Giovanna Fabio, Claudio Pignata, Maria Cristina Pietrogrande, and Raffaele Badolato

Abstract

BackgroundJob’s Syndrome or Autosomal Dominant Hyper Immunoglobulin E Syndrome (AD-HIES) is a very rare inborn error of immunity disorder with multi-organ involvement and long-life post-infective damages. Longitudinal registries are of main importance to improve knowledge on natural history and management of these rare disorders. This study aims to describe the natural history of 30 Italian patients recorded in the IPINet registry with dominat negative AD-HIES. MethodsThe study shows the incidence of manifestations presented at the time of diagnosis versus the ones arose during follow up at a referral centre for inborn errors of immunity (IEI). ResultsThe mean time of diagnostic delay was 13.7 years, while age at disease onset was Antifungal prophylaxis decreased the mucocutaneous candidiasis occurrence from 70% to 56.7%. In the course of SARS-CoV-2 pandemic, seven patients developed COVID-19. Survival analyses showed that 27 out of 30 patients are still alive, while three patients died at age of 28, 39 and 46 as consequence of lungs bleeding, lymphoma and sepsis, respectively. ConclusionsAnalysis of a cumulative follow-up of 278.7 patient-years has shown that early diagnosis, adequate management at expertise centres for IEI, prophylactic antibiotic and antifungal therapy improve outcome and can positively influence patients’ life expectancy.

Published
2022

119. Coronavirus disease 2019 in patients with inborn errors of immunity: An international study

Author

Liliana Bezrodnik, Vijay G. Sankaran, Silvia Sánchez-Ramón, Peter Mustillo, Michael A. Keller, Isabelle Meyts, Giorgia Bucciol, Yesim Yilmaz Demirdag, Luis Ignacio Gonzalez-Granado, Andrew R. Gennery, Alexandra F. Freeman, Raffaele Badolato, Alain Fischer, Safa Baris, Federica Barzaghi, Sudhir Gupta, Carlo Agostini, Gulbu Uzel, Kissy Guevara-Hoyer, Isabella Quinti, M. Cecilia Poli, Charlotte Cunningham-Rundles, Stephen Jolles, Elif Karakoc-Aydiner, Alessandro Aiuti, Cinzia Milito, Fabian Hauck, Angel Robles-Marhuenda, Stuart G. Tangye, Marco Yamazaki-Nakashimada, Elena Seoane, Sara Elva Espinosa-Padilla, Pierre Yves Jeandel, Kathleen E. Sullivan, Klaus Warnatz, Claire Fieschi, Cedric Bosteels, Alessandro Plebani, Leonardo Oliveira Mendonça, Carla Gianelli, François Vermeulen, Bart N. Lambrecht, Annarosa Soresina, Virgil A. S. H. Dalm, Selma Scheffler-Mendoza, Catherine Paillard, Eduardo López-Granados, Vassilios Lougaris, Ahmet Ozen, Grant Hayman, Nizar Mahlaoui, Yazmin Espinosa, Bénédicte Neven, Giuseppe Spadaro, Roshini S. Abraham, Meyts, Isabelle, Bucciol, Giorgia, Quinti, Isabella, Neven, Bénédicte, Fischer, Alain, Seoane, Elena, Lopez-Granados, Eduardo, Gianelli, Carla, Robles-Marhuenda, Angel, Jeandel, Pierre-Yve, Paillard, Catherine, Sankaran, Vijay G, Demirdag, Yesim Yilmaz, Lougaris, Vassilio, Aiuti, Alessandro, Plebani, Alessandro, Milito, Cinzia, Dalm, Virgil Ash, Guevara-Hoyer, Kissy, Sánchez-Ramón, Silvia, Bezrodnik, Liliana, Barzaghi, Federica, Gonzalez-Granado, Luis Ignacio, Hayman, Grant R, Uzel, Gulbu, Mendonça, Leonardo Oliveira, Agostini, Carlo, Spadaro, Giuseppe, Badolato, Raffaele, Soresina, Annarosa, Vermeulen, Françoi, Bosteels, Cedric, Lambrecht, Bart N, Keller, Michael, Mustillo, Peter J, Abraham, Roshini S, Gupta, Sudhir, Ozen, Ahmet, Karakoc-Aydiner, Elif, Baris, Safa, Freeman, Alexandra F, Yamazaki-Nakashimada, Marco, Scheffler-Mendoza, Selma, Espinosa-Padilla, Sara, Gennery, Andrew R, Jolles, Stephen, Espinosa, Yazmin, Poli, M Cecilia, Fieschi, Claire, Hauck, Fabian, Cunningham-Rundles, Charlotte, Mahlaoui, Nizar, Warnatz, Klau, Sullivan, Kathleen E, Tangye, Stuart G, Meyts, I., Bucciol, G., Quinti, I., Neven, B., Fischer, A., Seoane, E., Lopez-Granados, E., Gianelli, C., Robles-Marhuenda, A., Jeandel, P. -Y., Paillard, C., Sankaran, V. G., Demirdag, Y. Y., Lougaris, V., Aiuti, A., Plebani, A., Milito, C., Dalm, V. A., Guevara-Hoyer, K., Sanchez-Ramon, S., Bezrodnik, L., Barzaghi, F., Gonzalez-Granado, L. I., Hayman, G. R., Uzel, G., Mendonca, L. O., Agostini, C., Spadaro, G., Badolato, R., Soresina, A., Vermeulen, F., Bosteels, C., Lambrecht, B. N., Keller, M., Mustillo, P. J., Abraham, R. S., Gupta, S., Ozen, A., Karakoc-Aydiner, E., Baris, S., Freeman, A. F., Yamazaki-Nakashimada, M., Scheffler-Mendoza, S., Espinosa-Padilla, S., Gennery, A. R., Jolles, S., Espinosa, Y., Poli, M. C., Fieschi, C., Hauck, F., Cunningham-Rundles, C., Mahlaoui, N., Warnatz, K., Sullivan, K. E., Tangye, S. G., Internal Medicine, Neven, Benedicte, Lopez-Grandos, Eduardo, Jeandel, Pierre-Yves, Sankaran, Vijay G., Lougaris, Vassilios, Dalm, Virgil A. S. H., Sanchez-Ramon, Silvia, Ignacio Gonzalez-Granado, Luis, Hayman, Grant R., Mendonca, Leonardo Oliveira, Vermeulen, Francois, Lambrecht, Bart N., Mustillo, Peter J., Abraham, Roshini S., Freeman, Alexandra F., Gennery, Andrew R., Poli, M. Cecilia, Warnatz, Klaus, Sullivan, Kathleen E., and Tangye, Stuart G.

Subjects
0301 basic medicine, Male, inborn errors of immunity, X-CGD, X-linked chronic granulomatous disease, CGD, Chronic granulomatous disease, X-SCID, X-linked severe combined immunodeficiency, medicine.disease_cause, Severity of Illness Index, law.invention, HSCT, Hematopoietic stem cell transplantation, 0302 clinical medicine, law, Risk Factors, PID, Primary immunodeficiency, AIHA, Autoimmune hemolytic anemia, Medicine and Health Sciences, Immunology and Allergy, Child, Immunodeficiency, education.field_of_study, COVID-19, Coronavirus disease 2019, Middle Aged, Intensive care unit, ICU, Intensive care unit, Child, Preschool, ALPS, Autoimmune lymphoproliferative syndrome, Female, CVID, Common variable immune deficiency, primary immunodeficiencies, Adult, medicine.medical_specialty, IEI, Inborn errors of immunity, Adolescent, hypogammaglobulinemia, Population, Immunology, P, Patient, 03 medical and health sciences, Young Adult, immune dysregulation, Internal medicine, Intensive care, Severity of illness, medicine, Humans, education, Aged, Retrospective Studies, AR, Autosomal-recessive, SARS-CoV-2, Severe acute respiratory syndrome coronavirus 2, business.industry, SARS-CoV-2, AGS, Aicardi-Goutieres syndrome, Genetic Diseases, Inborn, Immunologic Deficiency Syndromes, Infant, Newborn, Infant, COVID-19, Retrospective cohort study, Immune dysregulation, medicine.disease, HLH, Hemophagocytic lymphohistiocytosis, 030104 developmental biology, Primary immunodeficiency, CID, Combined immunodeficiency, business, 030215 immunology
Abstract

BACKGROUND: There is uncertainty about the impact of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection in individuals with rare inborn errors of immunity (IEI), a population at risk of developing severe coronavirus disease 2019. This is relevant not only for these patients but also for the general population, because studies of IEIs can unveil key requirements for host defense. OBJECTIVE: We sought to describe the presentation, manifestations, and outcome of SARS-CoV-2 infection in IEI to inform physicians and enhance understanding of host defense against SARS-CoV-2. METHODS: An invitation to participate in a retrospective study was distributed globally to scientific, medical, and patient societies involved in the care and advocacy for patients with IEI. RESULTS: We gathered information on 94 patients with IEI with SARS-CoV-2 infection. Their median age was 25 to 34 years. Fifty-three patients (56%) suffered from primary antibody deficiency, 9 (9.6%) had immune dysregulation syndrome, 6 (6.4%) a phagocyte defect, 7 (7.4%) an autoinflammatory disorder, 14 (15%) a combined immunodeficiency, 3 (3%) an innate immune defect, and 2 (2%) bone marrow failure. Ten were asymptomatic, 25 were treated as outpatients, 28 required admission without intensive care or ventilation, 13 required noninvasive ventilation or oxygen administration, 18 were admitted to intensive care units, 12 required invasive ventilation, and 3 required extracorporeal membrane oxygenation. Nine patients (7 adults and 2 children) died. CONCLUSIONS: This study demonstrates that (1) more than 30% of patients with IEI had mild coronavirus disease 2019 (COVID-19) and (2) risk factors predisposing to severe disease/mortality in the general population also seemed to affect patients with IEI, including more younger patients. Further studies will identify pathways that are associated with increased risk of severe disease and are nonredundant or redundant for protection against SARS-CoV-2. ispartof: JOURNAL OF ALLERGY AND CLINICAL IMMUNOLOGY vol:147 issue:2 pages:520-531 ispartof: location:United States status: published

Published
2021

120. La sindrome di Chediak-Higashi a esordio tardivo

Author

Silvia Ciancia, Maria del Carmen Cano Garcinuno, Maria Francesca Dalla Porta, Annarosa Soresina, Raffaele Badolato, I. Mariotti, Monica Cellini, Greta Cingolani, and Lorenzo Iughetti

Subjects
business.industry, hemic and lymphatic diseases, Geography, Planning and Development, Medicine, Management, Monitoring, Policy and Law, business
Abstract

Chediak-Higashi syndrome (CHS) is a rare autosomal recessive disorder caused by mutations in the CHS1/LYST gene, encoding for LYST protein, involved in lysosomal trafficking. It is characterized by recurrent bacterial infections, oculocutaneous albinism, silver hair, haematological and neurological alterations and a possible evolution towards the socalled accelerated phase (haemophagocytic lymphohistiocytosis). It is classified in a classic form, with infantile onset, lethal if bone marrow transplantation is not promptly performed, and in an atypical form, with adolescent/adult onset, for which a more conservative approach may be possible. The paper describes a case of hypertrophic-hyperplastic gingivopathy associated with leuko-neutropenia. The evaluation of the bone marrow smear raised the suspicion of CHS and the analysis of the clinical history highlighted the presence of suggestive criteria for atypical CHS. The genetic investigation confirmed the diagnosis.

Published
2021

121. Experiences of political mobilization and popular participation in Milan's working-class neighbourhoods: 1945–1967

Author

Marco Soresina

Subjects
History, media_common.quotation_subject, 05 social sciences, Geography, Planning and Development, 0211 other engineering and technologies, 0507 social and economic geography, 021107 urban & regional planning, Gender studies, 02 engineering and technology, Political mobilization, Urban Studies, Arts and Humanities (miscellaneous), Working class, Political science, 050703 geography, media_common
Abstract

The years 1945–55 were a period of reconstruction for Italy; the following decade was one of economic growth. An aspect of this transition is analysed here, in relation to the forms of social integration created in working-class neighbourhoods. The case-study focuses on Milan, and the two organizations studied are the consulte popolari (the ‘people's councils’), created by the left in the immediate post-war period, and the ‘social centres’ created in the mid-1950s by the IACP (the Autonomous Institute of Public Housing). Both were attempts to involve the new, outlying suburbs in the city's political life, each of them trying to adapt to different political phases. Both, I would like to suggest, succeeded in achieving certain results.

Published
2021

122. White-collar workers in Milan: c. 1880–1915

Author

Marco Soresina

Subjects
060104 history, History, White (horse), Middle class, media_common.quotation_subject, education, 0601 history and archaeology, 06 humanities and the arts, humanities, Demography, Collar, media_common
Abstract

The article examines the milieu of white-collar workers and their structures of association through a case study of late nineteenth- and early twentieth-century Milan. The city had the largest numb...

Published
2021

123. Capsule Networks for Object Detection in UAV Imagery

Author

Mohamed Lamine Mekhalfi, Mesay Belete Bejiga, Davide Soresina, Farid Melgani, and Begüm Demir

Subjects
unmanned aerial vehicles, object detection, convolutional neural networks, capsule networks, dynamic routing, Science
Abstract

Recent advances in Convolutional Neural Networks (CNNs) have attracted great attention in remote sensing due to their high capability to model high-level semantic content of Remote Sensing (RS) images. However, CNNs do not explicitly retain the relative position of objects in an image and, thus, the effectiveness of the obtained features is limited in the framework of the complex object detection problems. To address this problem, in this paper we introduce Capsule Networks (CapsNets) for object detection in Unmanned Aerial Vehicle-acquired images. Unlike CNNs, CapsNets extract and exploit the information content about objects’ relative position across several layers, which enables parsing crowded scenes with overlapping objects. Experimental results obtained on two datasets for car and solar panel detection problems show that CapsNets provide similar object detection accuracies when compared to state-of-the-art deep models with significantly reduced computational time. This is due to the fact that CapsNets emphasize dynamic routine instead of the depth.

Published
2019
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124. Long term longitudinal follow up of AD-HIES cohort: impact of early diagnosis and enrolment to IPINet centres on natural history of Job's Syndrome

Author

Carrabba, Maria, primary, Dellepiane, Rosa Maria, additional, Cortesi, Manuela, additional, Baselli, Lucia Augusta, additional, Soresina, Annarosa, additional, Cirillo, Emilia, additional, Giardino, Giuliana, additional, Conti, Francesca, additional, Dotta, Laura, additional, Finocchi, Andrea, additional, Cancrini, Caterina, additional, Milito, Cinzia, additional, Pacillo, Lucia, additional, Cinicola, Bianca Laura, additional, Cossu, Fausto, additional, Consolini, Rita, additional, Montin, Davide, additional, Quinti, Isabella, additional, Pession, Andrea, additional, Fabio, Giovanna, additional, Pignata, Claudio, additional, Pietrogrande, Maria Cristina, additional, and Badolato, Raffaele, additional

Published
2022
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127. The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet): Twenty Years of Experience (1999–2019)

Author

Lougaris, V, Pession, A, Baronio, M, Soresina, A, Rondelli, R, Gazzurelli, L, Benvenuto, A, Martino, S, Gattorno, M, Biondi, A, Zecca, M, Marinoni, M, Fabio, G, Aiuti, A, Marseglia, G, Putti, M, Agostini, C, Lunardi, C, Tommasini, A, Bertolini, P, Gambineri, E, Consolini, R, Matucci, A, Azzari, C, Danieli, M, Paganelli, R, Duse, M, Cancrini, C, Moschese, V, Chessa, L, Spadaro, G, Civino, A, Vacca, A, Cardinale, F, Martire, B, Carpino, L, Trizzino, A, Russo, G, Cossu, F, Badolato, R, Pietrogrande, M, Quinti, I, Rossi, P, Ugazio, A, Pignata, C, Plebani, A, Lougaris V., Pession A., Baronio M., Soresina A., Rondelli R., Gazzurelli L., Benvenuto A., Martino S., Gattorno M., Biondi A., Zecca M., Marinoni M., Fabio G., Aiuti A., Marseglia G., Putti M. C., Agostini C., Lunardi C., Tommasini A., Bertolini P., Gambineri E., Consolini R., Matucci A., Azzari C., Danieli M. G., Paganelli R., Duse M., Cancrini C., Moschese V., Chessa L., Spadaro G., Civino A., Vacca A., Cardinale F., Martire B., Carpino L., Trizzino A., Russo G., Cossu F., Badolato R., Pietrogrande M. C., Quinti I., Rossi P., Ugazio A., Pignata C., Plebani A., Lougaris, V, Pession, A, Baronio, M, Soresina, A, Rondelli, R, Gazzurelli, L, Benvenuto, A, Martino, S, Gattorno, M, Biondi, A, Zecca, M, Marinoni, M, Fabio, G, Aiuti, A, Marseglia, G, Putti, M, Agostini, C, Lunardi, C, Tommasini, A, Bertolini, P, Gambineri, E, Consolini, R, Matucci, A, Azzari, C, Danieli, M, Paganelli, R, Duse, M, Cancrini, C, Moschese, V, Chessa, L, Spadaro, G, Civino, A, Vacca, A, Cardinale, F, Martire, B, Carpino, L, Trizzino, A, Russo, G, Cossu, F, Badolato, R, Pietrogrande, M, Quinti, I, Rossi, P, Ugazio, A, Pignata, C, Plebani, A, Lougaris V., Pession A., Baronio M., Soresina A., Rondelli R., Gazzurelli L., Benvenuto A., Martino S., Gattorno M., Biondi A., Zecca M., Marinoni M., Fabio G., Aiuti A., Marseglia G., Putti M. C., Agostini C., Lunardi C., Tommasini A., Bertolini P., Gambineri E., Consolini R., Matucci A., Azzari C., Danieli M. G., Paganelli R., Duse M., Cancrini C., Moschese V., Chessa L., Spadaro G., Civino A., Vacca A., Cardinale F., Martire B., Carpino L., Trizzino A., Russo G., Cossu F., Badolato R., Pietrogrande M. C., Quinti I., Rossi P., Ugazio A., Pignata C., and Plebani A.

Abstract

Primary immunodeficiencies (PIDs) are heterogeneous disorders, characterized by variable clinical and immunological features. National PID registries offer useful insights on the epidemiology, diagnosis, and natural history of these disorders. In 1999, the Italian network for primary immunodeficiencies (IPINet) was established. We report on data collected from the IPINet registry after 20 years of activity. A total of 3352 pediatric and adult patients affected with PIDs are registered in the database. In Italy, a regional distribution trend of PID diagnosis was observed. Based on the updated IUIS classification of 2019, PID distribution in Italy showed that predominantly antibody deficiencies account for the majority of cases (63%), followed by combined immunodeficiencies with associated or syndromic features (22.5%). The overall age at diagnosis was younger for male patients. The minimal prevalence of PIDs in Italy resulted in 5.1 per 100.000 habitants. Mortality was similar to other European registries (4.2%). Immunoglobulin replacement treatment was prescribed to less than one third of the patient cohort. Collectively, this is the first comprehensive description of the PID epidemiology in Italy.

Published
2020

129. Consensus of the Italian Primary Immunodeficiency Network on transition management from pediatric to adult care in patients affected with childhood-onset inborn errors of immunity

Author

Baldassarre Martire, Luciana Chessa, Alessandro Plebani, Francesca Conti, Andrea Finocchi, Francesca Ferrua, Laura Dotta, Clementina Canessa, Vassilios Lougaris, Donato Amodio, Emilia Cirillo, Davide Montin, Federica Barzaghi, Franco Locatelli, Alberto Tommasini, Viviana Moschese, Maria Pia Cicalese, Rosa Maria Dellepiane, Andrea Pession, Chiara Azzari, Lucia Augusta Baselli, Caterina Cancrini, Maria Caterina Putti, Raffaele Badolato, Alessandro Aiuti, Claudio Pignata, Annarosa Soresina, Roberta Romano, Silvia Ricci, Agata Polizzi, Antonio Marzollo, Giuliana Giardino, Cirillo, E., Giardino, G., Ricci, S., Moschese, V., Lougaris, V., Conti, F., Azzari, C., Barzaghi, F., Canessa, C., Martire, B., Badolato, R., Dotta, L., Soresina, A., Cancrini, C., Finocchi, A., Montin, D., Romano, R., Amodio, D., Ferrua, F., Tommasini, A., Baselli, L. A., Dellepiane, R. M., Polizzi, A., Chessa, L., Marzollo, A., Cicalese, M. P., Putti, M. C., Pession, A., Aiuti, A., Locatelli, F., Plebani, A., Pignata, C., Cirillo, Emilia, Giardino, Giuliana, Ricci, Silvia, Moschese, Viviana, Lougaris, Vassilio, Conti, Francesca, Azzari, Chiara, Barzaghi, Federica, Canessa, Clementina, Martire, Baldassarre, Badolato, Raffaele, Dotta, Laura, Soresina, Annarosa, Cancrini, Caterina, Finocchi, Andrea, Montin, Davide, Romano, Roberta, Amodio, Antonio, Ferrua, Francesca, Tommasini, Alberto, Baselli, Lucia Augusta, Dellepiane, Rosa Maria, Polizzi, Agata, Chessa, Luciana, Marzollo, Antonio, Cicalese, Mariapia, Putti, Maria Caterina, Pession, Andrea, Aiuti, Alessandro, Locatelli, Franco, Plebani, Alessandro, and Pignata, Claudio

Subjects
0301 basic medicine, Stress management, DiGeorge syndrome, Italian Network of Primary Immunodeficiencies, Transitional care, combined immunodeficiency, dna-repai syndromes, humoral immune defects, inborn errors of immunity, innate immune defects, primary immunodeficiency, DNA repair syndromes, 0302 clinical medicine, Italian Network of Primary Immunodeficiencie, Intellectual disability, Immunology and Allergy, Medicine, dna-repai syndrome, Age of Onset, Child, Settore MED/38, Italy, Settore MED/38 - PEDIATRIA GENERALE E SPECIALISTICA, Practice Guidelines as Topic, Adult, medicine.medical_specialty, Transition to Adult Care, Consensus, Genetic counseling, Primary Immunodeficiency Diseases, Immunology, humoral immune defect, 03 medical and health sciences, Quality of life (healthcare), Humans, Information Services, business.industry, Common variable immunodeficiency, medicine.disease, DNA repair syndrome, 030104 developmental biology, Family medicine, Primary immunodeficiency, Age of onset, business, innate immune defect, 030217 neurology & neurosurgery
Abstract

Medical advances have dramatically improved the long-term prognosis of children and adolescents with inborn errors of immunity (IEIs). Transfer of the medical care of individuals with pediatric IEIs to adult facilities is also a complex task because of the large number of distinct disorders, which requires involvement of patients and both pediatric and adult care providers. To date, there is no consensus on the optimal pathway of the transitional care process and no specific data are available in the literature regarding patients with IEIs. We aimed to develop a consensus statement on the transition process to adult health care services for patients with IEIs. Physicians from major Italian Primary Immunodeficiency Network centers formulated and answered questions after examining the currently published literature on the transition from childhood to adulthood. The authors voted on each recommendation. The most frequent IEIs sharing common main clinical problems requiring full attention during the transitional phase were categorized into different groups of clinically related disorders. For each group of clinically related disorders, physicians from major Italian Primary Immunodeficiency Network institutions focused on selected clinical issues representing the clinical hallmark during early adulthood.

Published
2020

130. The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet): Twenty Years of Experience (1999–2019)

Author

A G Ugazio, Giovanna Russo, Maria Giovanna Danieli, Alberto Tommasini, Maria Cristina Pietrogrande, Raffaele Badolato, Andrea Pession, Carlo Agostini, Fabio Cardinale, Eleonora Gambineri, Baldassare Martire, Adele Civino, Manuela Baronio, Marco Gattorno, Marco Zecca, Viviana Moschese, Chiara Azzari, Alessio Benvenuto, Marzia Duse, Antonino Trizzino, Luisa Gazzurelli, Isabella Quinti, Vassilios Lougaris, Andrea Matucci, Giuseppe Spadaro, Claudio Lunardi, Angelo Vacca, Roberto Rondelli, Maria Caterina Putti, Luciana Chessa, Giovanna Fabio, Andrea Biondi, Fausto Cossu, Roberto Paganelli, Paolo Rossi, Rita Consolini, Alessandro Aiuti, Gian Luigi Marseglia, Luigi Carpino, Caterina Cancrini, Maddalena Marinoni, Silvana Martino, Claudio Pignata, Annarosa Soresina, Patrizia Bertolini, Alessandro Plebani, Lougaris, V., Pession, A., Baronio, M., Soresina, A., Rondelli, R., Gazzurelli, L., Benvenuto, A., Martino, S., Gattorno, M., Biondi, A., Zecca, M., Marinoni, M., Fabio, G., Aiuti, A., Marseglia, G., Putti, M. C., Agostini, C., Lunardi, C., Tommasini, A., Bertolini, P., Gambineri, E., Consolini, R., Matucci, A., Azzari, C., Danieli, M. G., Paganelli, R., Duse, M., Cancrini, C., Moschese, V., Chessa, L., Spadaro, G., Civino, A., Vacca, A., Cardinale, F., Martire, B., Carpino, L., Trizzino, A., Russo, G., Cossu, F., Badolato, R., Pietrogrande, M. C., Quinti, I., Rossi, P., Ugazio, A., Pignata, C., Plebani, A., Lougaris, V, Pession, A, Baronio, M, Soresina, A, Rondelli, R, Gazzurelli, L, Benvenuto, A, Martino, S, Gattorno, M, Biondi, A, Zecca, M, Marinoni, M, Fabio, G, Aiuti, A, Marseglia, G, Putti, M, Agostini, C, Lunardi, C, Tommasini, A, Bertolini, P, Gambineri, E, Consolini, R, Matucci, A, Azzari, C, Danieli, M, Paganelli, R, Duse, M, Cancrini, C, Moschese, V, Chessa, L, Spadaro, G, Civino, A, Vacca, A, Cardinale, F, Martire, B, Carpino, L, Trizzino, A, Russo, G, Cossu, F, Badolato, R, Pietrogrande, M, Quinti, I, Rossi, P, Ugazio, A, Pignata, C, and Plebani, A

Subjects
Adult, Male, medicine.medical_specialty, Pediatrics, Primary immunodeficiencies, Adolescent, Databases, Factual, Primary Immunodeficiency Diseases, Immunology, Age at diagnosis, History, 21st Century, Combined immunodeficiencies, Young Adult, Medical microbiology, patient registry, Epidemiology, medicine, Prevalence, Immunology and Allergy, Humans, Registries, Geography, Medical, Child, Adult patients, business.industry, Infant, Newborn, Infant, History, 20th Century, medicine.disease, Prognosis, Settore MED/38, Natural history, Italy, Child, Preschool, Population Surveillance, Cohort, Primary immunodeficiency, Original Article, Female, business
Abstract

Primary immunodeficiencies (PIDs) are heterogeneous disorders, characterized by variable clinical and immunological features. National PID registries offer useful insights on the epidemiology, diagnosis, and natural history of these disorders. In 1999, the Italian network for primary immunodeficiencies (IPINet) was established. We report on data collected from the IPINet registry after 20 years of activity. A total of 3352 pediatric and adult patients affected with PIDs are registered in the database. In Italy, a regional distribution trend of PID diagnosis was observed. Based on the updated IUIS classification of 2019, PID distribution in Italy showed that predominantly antibody deficiencies account for the majority of cases (63%), followed by combined immunodeficiencies with associated or syndromic features (22.5%). The overall age at diagnosis was younger for male patients. The minimal prevalence of PIDs in Italy resulted in 5.1 per 100.000 habitants. Mortality was similar to other European registries (4.2%). Immunoglobulin replacement treatment was prescribed to less than one third of the patient cohort. Collectively, this is the first comprehensive description of the PID epidemiology in Italy.

Published
2020

131. 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

Author

Fabio Cardinale, Giorgio Ciprandi, Salvatore Barberi, Roberto Bernardini, Carlo Caffarelli, Mauro Calvani, Giovanni Cavagni, Elena Galli, Domenico Minasi, Michele Miraglia Del Giudice, Viviana Moschese, Elio Novembre, Francesco Paravati, Diego G Peroni, Maria Angela Tosca, Giovanni Traina, Salvatore Tripodi, Gian Luigi Marseglia, Doriana Amato, Caterina Anania, Elisa Anastasio, Rachele Antignani, Stefania Arasi, Martire Baldassarre, Ermanno Baldo, Andrea Barbalace, Simona Barni, Federica Betti, Annamaria Bianchi, Ezio Bolzacchini, Maira Bonini, Paolo Bottau, Sara Bozzetto, Maria Antonia Brighetti, Davide Caimmi, Silvia Caimmi, Luigi Calzone, Caterina Cancrini, Lucia Caminiti, Giulia Capata, Lucetta Capra, Carlo Capristo, Elena Carboni, Francesco Carella, Riccardo Castagnoli, Elena Chiappini, Fernanda Chiera, Iolanda Chinellato, Loredana Chini, Francesca Cipriani, Flavio Civitelli, Pasquale Comberiati, Daniele Contini, Stefania Corrente, Claudio Cravidi, Giuseppe Crisafulli, Barbara Cuomo, Enza D'Auria, Sofia D'Elios, Fabio Decimo, Auro Della Giustina, Rosa Maria Delle Piane, Maria De Filippo, Valentina De Vittori, Lucia Diaferio, Maria Elisa Di Cicco, Dora Di Mauro, Marzia Duse, Silvia Federici, Giuseppe Felice, Maria Grazia Fenu, Giuliana Ferrante, Tiziana Foti, Fabrizio Franceschini, Daniele Ghiglioni, Giuliana Giardino, Mattia Giovannini, Giovanni Cosimo Indirli, Cristiana Indolfi, Massimo Landi, Francesco La Torre, Lucia Maddalena Leone, Amelia Licari, Lucia Liotti, Vassilios Lougaris, Nunzia Maiello, Paride Mantecca, Sara Manti, Marco Maria Mariani, Alberto Martelli, Carla Mastrorilli, Violetta Mastrorilli, Davide Montin, Francesca Mori, Roberta Olcese, Giorgio Ottaviano, Claudia Paglialunga, Giovanni Pajno, Giuseppe Parisi, Stefano Pattini, Luca Pecoraro, Umberto Pelosi, Claudio Pignata, Giampaolo Ricci, Silvia Ricci, Stefano Rizzi, Caterina Rizzo, Sara Rosati, Paolo Rosso, Maria Sangerardi, Angelica Santoro, Francesca Saretta, Lucrezia Sarti, Marco Sartorio, Majla Sgruletti, Annarosa Soresina, Ifigenia Sfika, Mayla Sgrulletti, Nuccia Tesse, Valentina Tranchino, Alessandro Travaglini, Malizia Velia, Elvira Verduci, Mario Vernich, Elisabetta Veronelli, Stefano Volpi, Martina Votto, Anna Maria Zicari, Cardinale, F., Ciprandi, G., Barberi, S., Bernardini, R., Caffarelli, C., Calvani, M., Cavagni, G., Galli, E., Minasi, D., Del Giudice, M. M., Moschese, V., Novembre, E., Paravati, F., Peroni, D. G., Tosca, M. A., Traina, G., Tripodi, S., Marseglia, G. L., Amato, D., Anania, C., Anastasio, E., Antignani, R., Arasi, S., Baldassarre, M., Baldo, E., Barbalace, A., Barni, S., Betti, F., Bianchi, A., Bolzacchini, E., Bonini, M., Bottau, P., Bozzetto, S., Brighetti, M. A., Caimmi, D., Caimmi, S., Calzone, L., Cancrini, C., Caminiti, L., Capata, G., Capra, L., Capristo, C., Carboni, E., Carella, F., Castagnoli, R., Chiappini, E., Chiera, F., Chinellato, I., Chini, L., Cipriani, F., Civitelli, F., Comberiati, P., Contini, D., Corrente, S., Cravidi, C., Crisafulli, G., Cuomo, B., D'Auria, E., D'Elios, S., Decimo, F., Giustina, A. D., Piane, R. M. D., De Filippo, M., De Vittori, V., Diaferio, L., Di Mauro, M. E., Duse, M., Federici, S., Felice, G., Fenu, G., Ferrante, G., Foti, T., Franceschini, F., Ghiglioni, D., Giardino, G., Giovannini, M., Indirli, G. C., Indolfi, C., Landi, M., La Torre, F., Leone, L. M., Licari, A., Liotti, L., Lougaris, V., Maiello, N., Mantecca, P., Manti, S., Mariani, M. M., Martelli, A., Mastrorilli, C., Mastrorilli, V., Montin, D., Mori, F., Olcese, R., Ottaviano, G., Paglialunga, C., Pajno, G., Parisi, G., Pattini, S., Pecoraro, L., Pelosi, U., Pignata, C., Ricci, G., Ricci, S., Rizzi, S., Rizzo, C., Rosati, S., Rosso, P., Sangerardi, M., Santoro, A., Saretta, F., Sarti, L., Sartorio, M., Sgruletti, M., Soresina, A., Sfika, I., Sgrulletti, M., Tesse, N., Tranchino, V., Travaglini, A., Velia, M., Verduci, E., Vernich, M., Veronelli, E., Volpi, S., Votto, M., Zicari, A. M., Cardinale, Fabio, Ciprandi, Giorgio, Barberi, Salvatore, Bernardini, Roberto, Caffarelli, Carlo, Calvani, Mauro, Cavagni, Giovanni, Galli, Elena, Minasi, Domenico, Del Giudice, Michele Miraglia, Moschese, Viviana, Novembre, Elio, Paravati, Francesco, Peroni, Diego G, Tosca, Maria Angela, Traina, Giovanni, Tripodi, Salvatore, Marseglia, Gian Luigi, SIAIP task force Pignata, Claudio, Cardinale, F, Ciprandi, G, Barberi, S, Bernardini, R, Caffarelli, C, Calvani, M, Cavagni, G, Galli, E, Minasi, D, Del Giudice, M, Moschese, V, Novembre, E, Paravati, F, Peroni, D, Tosca, M, Traina, G, Tripodi, S, Marseglia, G, Amato, D, Anania, C, Anastasio, E, Antignani, R, Arasi, S, Baldassarre, M, Baldo, E, Barbalace, A, Barni, S, Betti, F, Bianchi, A, Bolzacchini, E, Bonini, M, Bottau, P, Bozzetto, S, Brighetti, M, Caimmi, D, Caimmi, S, Calzone, L, Cancrini, C, Caminiti, L, Capata, G, Capra, L, Capristo, C, Carboni, E, Carella, F, Castagnoli, R, Chiappini, E, Chiera, F, Chinellato, I, Chini, L, Cipriani, F, Civitelli, F, Comberiati, P, Contini, D, Corrente, S, Cravidi, C, Crisafulli, G, Cuomo, B, D'Auria, E, D'Elios, S, Decimo, F, Giustina, A, Piane, R, De Filippo, M, De Vittori, V, Diaferio, L, Di Mauro, M, Duse, M, Federici, S, Felice, G, Fenu, G, Ferrante, G, Foti, T, Franceschini, F, Ghiglioni, D, Giardino, G, Giovannini, M, Indirli, G, Indolfi, C, Landi, M, La Torre, F, Leone, L, Licari, A, Liotti, L, Lougaris, V, Maiello, N, Mantecca, P, Manti, S, Mariani, M, Martelli, A, Mastrorilli, C, Mastrorilli, V, Montin, D, Mori, F, Olcese, R, Ottaviano, G, Paglialunga, C, Pajno, G, Parisi, G, Pattini, S, Pecoraro, L, Pelosi, U, Pignata, C, Ricci, G, Ricci, S, Rizzi, S, Rizzo, C, Rosati, S, Rosso, P, Sangerardi, M, Santoro, A, Saretta, F, Sarti, L, Sartorio, M, Sgruletti, M, Soresina, A, Sfika, I, Sgrulletti, M, Tesse, N, Tranchino, V, Travaglini, A, Velia, M, Verduci, E, Vernich, M, Veronelli, E, Volpi, S, Votto, M, Zicari, A, and Fabio Cardinale, Giorgio Ciprandi, Salvatore Barberi, Roberto Bernardini, Carlo Caffarelli, Mauro Calvani, Giovanni Cavagni, Elena Galli, Domenico Minasi, Michele Miraglia Del Giudice, Viviana Moschese, Elio Novembre, Francesco Paravati, Diego G Peroni, Maria Angela Tosca, Giovanni Traina, Salvatore Tripodi, Gian Luigi Marseglia, Doriana Amato, Caterina Anania, Elisa Anastasio, Rachele Antignani, Stefania Arasi, Martire Baldassarre, Ermanno Baldo, Andrea Barbalace, Simona Barni, Federica Betti, Annamaria Bianchi, Ezio Bolzacchini, Maira Bonini, Paolo Bottau, Sara Bozzetto, Maria Antonia Brighetti, Davide Caimmi, Silvia Caimmi, Luigi Calzone, Caterina Cancrini, Lucia Caminiti, Giulia Capata, Lucetta Capra, Carlo Capristo, Elena Carboni, Francesco Carella, Riccardo Castagnoli, Elena Chiappini, Fernanda Chiera, Iolanda Chinellato, Loredana Chini, Francesca Cipriani, Flavio Civitelli, Pasquale Comberiati, Daniele Contini, Stefania Corrente, Claudio Cravidi, Giuseppe Crisafulli, Barbara Cuomo, Enza D'Auria, Sofia D'Elios, Fabio Decimo, Auro Della Giustina, Rosa Maria Delle Piane, Maria De Filippo, Valentina De Vittori, Lucia Diaferio, Maria Elisa Di Cicco, Dora Di Mauro, Marzia Duse, Silvia Federici, Giuseppe Felice, Maria Grazia Fenu, Giuliana Ferrante, Tiziana Foti, Fabrizio Franceschini, Daniele Ghiglioni, Giuliana Giardino, Mattia Giovannini, Giovanni Cosimo Indirli, Cristiana Indolfi, Massimo Landi, Francesco La Torre, Lucia Maddalena Leone, Amelia Licari, Lucia Liotti, Vassilios Lougaris, Nunzia Maiello, Paride Mantecca, Sara Manti, Marco Maria Mariani, Alberto Martelli, Carla Mastrorilli, Violetta Mastrorilli, Davide Montin, Francesca Mori, Roberta Olcese, Giorgio Ottaviano, Claudia Paglialunga, Giovanni Pajno, Giuseppe Parisi, Stefano Pattini, Luca Pecoraro, Umberto Pelosi, Claudio Pignata, Giampaolo Ricci, Silvia Ricci, Stefano Rizzi, Caterina Rizzo, Sara Rosati, Paolo Rosso, Maria Sangerardi, Angelica Santoro, Francesca Saretta, Lucrezia Sarti, Marco Sartorio, Majla Sgruletti, Annarosa Soresina, Ifigenia Sfika, Mayla Sgrulletti, Nuccia Tesse, Valentina Tranchino, Alessandro Travaglini, Malizia Velia, Elvira Verduci, Mario Vernich, Elisabetta Veronelli, Stefano Volpi, Martina Votto, Anna Maria Zicari

Subjects
Allergy, Review, 030207 dermatology & venereal diseases, Settore MED/38 - Pediatria Generale E Specialistica, 0302 clinical medicine, COVID-19, Child, Pandemic, Immunologic disease, Asthma, Adolescent, Viral, 030212 general & internal medicine, Disease management (health), Societies, Medical, pandemic, child, adolescent, allergy, asthma, immunologic disease, Incidence (epidemiology), lcsh:RJ1-570, Disease Management, General Medicine, Atopic dermatitis, Settore MED/38, Coronavirus Infections, Decision Making, Humans, Italy, Pneumonia, Viral, Pragmatic Clinical Trials as Topic, SARS-CoV-2, Allergy and Immunology, Betacoronavirus, Consensus, Pandemics, Latex allergy, Human, Telemedicine, Consensu, 03 medical and health sciences, Medical, medicine, Risk factor, Betacoronaviru, business.industry, Coronavirus Infection, lcsh:Pediatrics, Pneumonia, medicine.disease, Immunology, Societies, business, Rare disease
Abstract

The COVID-19 pandemic has surprised the entire population. The world has had to face an unprecedented pandemic. Only, Spanish flu had similar disastrous consequences. As a result, drastic measures (lockdown) have been adopted worldwide. Healthcare service has been overwhelmed by the extraordinary influx of patients, often requiring high intensity of care. Mortality has been associated with severe comorbidities, including chronic diseases. Patients with frailty were, therefore, the victim of the SARS-COV-2 infection. Allergy and asthma are the most prevalent chronic disorders in children and adolescents, so they need careful attention and, if necessary, an adaptation of their regular treatment plans. Fortunately, at present, young people are less suffering from COVID-19, both as incidence and severity. However, any age, including infancy, could be affected by the pandemic.Based on this background, the Italian Society of Pediatric Allergy and Immunology has felt it necessary to provide a Consensus Statement. This expert panel consensus document offers a rationale to help guide decision-making in the management of children and adolescents with allergic or immunologic diseases.

Published
2020

132. Diagnosis and Treatment of Post-Prostatectomy Lymphedema: What's New?

Author

Bianchi, Lorenzo Maria Giuseppe, Irmici, Giovanni, Cè, Maurizio, D'Ascoli, Elisa, Della Pepa, Gianmarco, Di Vita, Filippo, Casati, Omar, Soresina, Massimo, Menozzi, Andrea, Khenkina, Natallia, and Cellina, Michaela

Subjects
LYMPHEDEMA, MAGNETIC resonance imaging, DIAGNOSIS, INDOCYANINE green, DISEASE management, POLYPOIDAL choroidal vasculopathy, PROSTATE cancer
Abstract

Lymphedema is a chronic progressive disorder that significantly compromises patients' quality of life. In Western countries, it often results from cancer treatment, as in the case of post-radical prostatectomy lymphedema, where it can affect up to 20% of patients, with a significant disease burden. Traditionally, diagnosis, assessment of severity, and management of disease have relied on clinical assessment. In this landscape, physical and conservative treatments, including bandages and lymphatic drainage have shown limited results. Recent advances in imaging technology are revolutionizing the approach to this disorder: magnetic resonance imaging has shown satisfactory results in differential diagnosis, quantitative classification of severity, and most appropriate treatment planning. Further innovations in microsurgical techniques, based on the use of indocyanine green to map lymphatic vessels during surgery, have improved the efficacy of secondary LE treatment and led to the development of new surgical approaches. Physiologic surgical interventions, including lymphovenous anastomosis (LVA) and vascularized lymph node transplant (VLNT), are going to face widespread diffusion. A combined approach to microsurgical treatment provides the best results: LVA is effective in promoting lymphatic drainage, bridging VLNT delayed lymphangiogenic and immunological effects in the lymphatic impairment site. Simultaneous VLNT and LVA are safe and effective for patients with both early and advanced stages of post-prostatectomy LE. A new perspective is now represented by the combination of microsurgical treatments with the positioning of nano fibrillar collagen scaffolds (BioBridgeTM) to favor restoring the lymphatic function, allowing for improved and sustained volume reduction. In this narrative review, we proposed an overview of new strategies for diagnosing and treating post-prostatectomy lymphedema to get the most appropriate and successful patient treatment with an overview of the main artificial intelligence applications in the prevention, diagnosis, and management of lymphedema. [ABSTRACT FROM AUTHOR]

Published
2023
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135. Imaging of bronchial pathology in antibody deficiency

Author

Cinzia Milito, Maria Pia Bondioni, Scott Hackett, Esther de Vries, Alessandro Plebani, Vassilios Lougaris, Nicholas Screaton, Isabelle Meyts, Goffredo Serra, Nermeen Galal, Jamanda Haddock, M Lucas, Arpan K. Banerjee, Michael R. Loebinger, Vojtech Thon, Katharina Schütz, Peter M. van Hagen, Alison M. Condliffe, Robert G Stirling, Bodo Grimbacher, Annarosa Soresina, Luis Ignacio Gonzalez-Granado, John R. Hurst, Gertjan J. Driessen, Klaus Warnatz, Vera Postranecka, Peter Kelleher, Jiri Litzman, Dinakantha S. Kumararatne, Andrew Exley, Isabella Quinti, Judith Babar, Cesare Sirignano, Smita Y. Patel, Ieneke Hartmann, Ulrich Baumann, Giuseppe Spadaro, Francesco Fraioli, Sabine Dettmer, Alison Jones, Annemarie Bruining, Helen Chapel, Milica Mitrevski, Claire-Michèle Farber, Benjamin Gathmann, Diana Alecsandru, Schütz, Katharina, Alecsandru, Diana, Grimbacher, Bodo, Haddock, Jamanda, Bruining, Annemarie, Driessen, Gertjan, de Vries, Esther, van Hagen, Peter M., Hartmann, Ieneke, Fraioli, Francesco, Milito, Cinzia, Mitrevski, Milica, Quinti, Isabella, Serra, Goffredo, Kelleher, Peter, Loebinger, Michael, Litzman, Jiri, Postranecka, Vera, Thon, Vojtech, Babar, Judith, Condliffe, Alison M., Exley, Andrew, Kumararatne, Dinakantha, Screaton, Nick, Jones, Alison, Bondioni, Maria P., Lougaris, Vassilio, Plebani, Alessandro, Soresina, Annarosa, Sirignano, Cesare, Spadaro, Giuseppe, Galal, Nermeen, Gonzalez-Granado, Luis I., Dettmer, Sabine, Stirling, Robert, Chapel, Helen, Lucas, Mary, Patel, Smita, Farber, Claire-Michele, Meyts, Isabelle, Banerjee, Arpan K., Hackett, Scott, Hurst, John R., Warnatz, Klau, Gathmann, Benjamin, Baumann, Ulrich, Pediatrics, Public Health, Immunology, Internal Medicine, Radiology & Nuclear Medicine, Tranzo, Scientific center for care and wellbeing, and Geestelijke Gezondheidszorg

Subjects
Male, 0301 basic medicine, Pathology, bronchiectasis, X-linked agammaglobulinemia, LARGE COHORT, Disease, Pulmonary function testing, bronchiectasi, 0302 clinical medicine, Medical microbiology, Immunology and Allergy, Child, Immunodeficiency, medicine.diagnostic_test, CVID, X-LINKED AGAMMAGLOBULINEMIA, 3. Good health, LUNG-FUNCTION, 1107 Immunology, Child, Preschool, DISEASES, Female, Life Sciences & Biomedicine, Adult, Spirometry, medicine.medical_specialty, Adolescent, Immunology, CYSTIC FIBROSIS BRONCHIECTASIS, Bronchi, COMMON VARIABLE IMMUNODEFICIENCY, Chest CT in Antibody Deficiency Group, Young Adult, 03 medical and health sciences, Chest CT, bronchial pathology, primary antibody deficiency, medicine, MANAGEMENT, Humans, COMPUTED-TOMOGRAPHY, Thoracic Wall, Aged, Science & Technology, Bronchiectasis, business.industry, Common variable immunodeficiency, Immunologic Deficiency Syndromes, Infant, PULMONARY-FUNCTION, medicine.disease, 030104 developmental biology, Tomography, X-Ray Computed, business, SCAN, 030215 immunology
Abstract

Studies of chest computed tomography (CT) in patients with primary antibody deficiency syndromes (ADS) suggest a broad range of bronchial pathology. However, there are as yet no multicentre studies to assess the variety of bronchial pathology in this patient group. One of the underlying reasons is the lack of a consensus methodology, a prerequisite to jointly document chest CT findings. We aimed to establish an international platform for the evaluation of bronchial pathology as assessed by chest CT and to describe the range of bronchial pathologies in patients with antibody deficiency. Ffteen immunodeficiency centres from 9 countries evaluated chest CT scans of patients with ADS using a predefined list of potential findings including an extent score for bronchiectasis. Data of 282 patients with ADS were collected. Patients with common variable immunodeficiency disorders (CVID) comprised the largest subgroup (232 patients, 82.3%). Eighty percent of CVID patients had radiological evidence of bronchial pathology including bronchiectasis in 61%, bronchial wall thickening in 44% and mucus plugging in 29%. Bronchiectasis was detected in 44% of CVID patients aged less than 20 years. Cough was a better predictor for bronchiectasis than spirometry values. Delay of diagnosis as well as duration of disease correlated positively with presence of bronchiectasis. The use of consensus diagnostic criteria and a pre-defined list of bronchial pathologies allows for comparison of chest CT data in multicentre studies. Our data suggest a high prevalence of bronchial pathology in CVID due to late diagnosis or duration of disease.

Published
2019

138. Secondary Lymphedema Following Radical Prostatectomy

Author

Giancarlo Oliva, Dario Boccanera, Andrea Menozzi, Massimo Soresina, Michaela Cellina, Denisa Giardini, Daniele Gibelli, and Carlo Martinenghi

Subjects
Male, medicine.medical_specialty, Magnetic Resonance Spectroscopy, Secondary lymphedema, medicine.medical_treatment, 030230 surgery, Malignancy, 03 medical and health sciences, 0302 clinical medicine, Manual lymphatic drainage, medicine, Humans, Lymphedema, Lymph node, Prostatectomy, medicine.diagnostic_test, business.industry, Lymphography, Magnetic resonance imaging, medicine.disease, medicine.anatomical_structure, Lymphatic system, 030220 oncology & carcinogenesis, Quality of Life, Surgery, Lymph Nodes, Radiology, medicine.symptom, business
Abstract

Lymphedema (LE) is a progressive chronic disorder, frequently related to malignancies and their treatment in Western countries. It markedly affected patients' quality of life, and its management is challenging, mainly based on conservative therapy, as bandages and manual lymphatic drainage, with limited results.Recent advances in imaging technology and microsurgical techniques have changed the approach to this disorder, particularly the recent application of magnetic resonance for the study of LE and the increasingly widespread use of surgical interventions, such as vascularized lymph node transfer. As secondary LE due to radical prostatectomy and/or radiation treatment is a frequent cause of chronic disability in the male population, and few data are available in literature about which imaging technique can be applied to diagnose it and about the surgical modalities to treat this condition, we want to propose an overview on the lymphatic vessels anatomy and function, on the applications of noncontrast magnetic resonance lymphangiography and about the pieces of information this examination can provide, and on the technique of vascularized lymph node transfer and the rationale of this surgical procedure in secondary LE related to prostatic malignancy treatment.

Published
2020

139. Hopf bifurcations in the full SKT model and where to find them

Author

CINZIA SORESINA

Subjects
Nonlinear Sciences::Chaotic Dynamics, Mathematics - Analysis of PDEs, 35B32, 65P30, 35K59, 35B36, 35Q92, 92D25, Applied Mathematics, FOS: Mathematics, Discrete Mathematics and Combinatorics, Nonlinear Sciences::Pattern Formation and Solitons, Analysis, Analysis of PDEs (math.AP)
Abstract

In this paper, we consider the Shigesada–Kawasaki–Teramoto (SKT) model, which presents cross-diffusion terms describing competition pressure effects. Even though the reaction part does not present the activator–inhibitor structure, cross-diffusion can destabilise the homogeneous equilibrium. However, in the full cross-diffusion system and weak competition regime, the cross-diffusion terms have an opposite effect and the bifurcation structure of the system modifies as the interspecific competition pressure increases. The major changes in the bifurcation structure, the type of pitchfork bifurcations on the homogeneous branch, as well as the presence of Hopf bifurcation points are here investigated. Through weakly nonlinear analysis, we can predict the type of pitchfork bifurcation. Increasing the additional cross-diffusion coefficients, the first two pitchfork bifurcation points from super-critical become sub-critical, leading to the appearance of a multi-stability region. The interspecific competition pressure also influences the possible appearance of stable time-period spatial patterns appearing through a Hopf bifurcation point.

Published
2022

140. Constant primary operators and where to find them: The strange case of BPS defects in ABJ(M) theory

Author

Nicola Gorini, Luca Griguolo, Luigi Guerrini, Silvia Penati, Domenico Seminara, and Paolo Soresina

Subjects
High Energy Physics - Theory, Nuclear and High Energy Physics, High Energy Physics - Theory (hep-th), hep-th, FOS: Physical sciences, Particle Physics - Theory
Abstract

We investigate the one-dimensional defect SCFT defined on the $1/2$ BPS Wilson line/loop in ABJ(M) theory. We show that the supermatrix structure of the defect imposes a covariant supermatrix representation of the supercharges. Exploiting this covariant formulation, we prove the existence of a long multiplet whose highest weight state is a constant supermatrix operator. At weak coupling, we study this operator in perturbation theory and confirm that it acquires a non-trivial anomalous dimension. At strong coupling, we conjecture that this operator is dual to the lowest bound state of fluctuations of the fundamental open string in AdS$_4\times \mathbb{CP}_3$ around the classical $1/2$ BPS solution. Quite unexpectedly, this operator also arises in the cohomological equivalence between bosonic and fermionic Wilson loops. We also discuss some regularization subtleties arising in perturbative calculations on the infinite Wilson line., Comment: 36 pages plus appendices, 5 figures, 5 tables

Published
2022
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141. When a Nontuberculous Mycobacterial Infection Reveals an Error of Immunity: A Single Center's Experience

Author

Alessia Morreale, Laura Dotta, Donatella Vairo, Tullia Bazzana, Vassilios Lougaris, Annarosa Soresina, Alessandro Plebani, Silvia Clara Giliani, Fulvio Porta, Alberto Matteelli, Luca Oscar Redaelli De Zinis, and Raffaele Badolato

Subjects
Microbiology (medical), Infectious Diseases, Pediatrics, Perinatology and Child Health, Humans, Mycobacterium Infections, Nontuberculous, Nontuberculous Mycobacteria, Child
Abstract

We present an algorithm that may be applied in case of a diagnosis of pediatric nontuberculous mycobacterial disease to identify the patients who may require an immunologic assessment to discover a possible underlying immune system defect predisposing to their nontuberculous mycobacterial infections.

Published
2022

142. Omenn Syndrome due to RAG1 Mutation Presenting With Nonimmune Hydrops Fetalis in Two Siblings

Author

Lara Valeri, Licia Lugli, Lorenzo Iughetti, Annarosa Soresina, Silvia Giliani, Fulvio Porta, and Alberto Berardi

Subjects
Homeodomain Proteins, Male, Hydrops Fetalis, Siblings, Homozygote, Infant, Newborn, Alopecia, Pedigree, Morocco, Pediatrics, Perinatology and Child Health, Humans, Severe Combined Immunodeficiency, Frameshift Mutation, Dermatitis, Exfoliative
Abstract

Omenn syndrome (OS) is a rare variant of severe combined immunodeficiency characterized by susceptibility to severe opportunistic infections and peculiar manifestations, such as protein-losing erythroderma, alopecia, hepatosplenomegaly, lymphadenopathies, and severe diarrhea. The typical form of the disease is caused by hypomorphic mutation of the recombination-activating genes (RAG1 and RAG2), which are critical in initiating the molecular processes leading to lymphocyte and immunoglobulin receptor formation. Affected patients lack B cells, whereas autoreactive oligoclonal T cells infiltrate the skin, gut, spleen, and liver. In the absence of hematopoietic stem cell transplantation, patients with OS usually succumb early in life because of opportunistic infections. The incidence of OS is estimated to be

Published
2022

143. Inherited defects in the complement system

Author

Leonardi, L, La Torre, F, Soresina, A, Federici, S, Cancrini, C, Castagnoli, R, Cinicola, B, Corrente, S, Giardino, G, Lougaris, V, Volpi, S, Marseglia, G, Cardinale, F, and Immunology Task Force of the Italian Society of Pediatric Allergy and Immunology (SIAIP)

Subjects
Immunology, pathways, alternative, classical, complement, deficiency, inherited, lectin, system, Complement System Proteins, Settore MED/02, Pediatrics, Perinatology and Child Health, Humans, Immunology and Allergy
Abstract

The complement system plays an essential role in both innate and adaptive immune responses. Any dysregulation in this system can disturb normal host defense and alter inflammatory response leading to both infections and autoimmune diseases. The complement system can be activated through three different pathways. Inherited complement deficiencies have been described for all complement components and their regulators. Despite being rare diseases, complement deficiencies are often severe, with a frequent onset during childhood. We provide an overview of clinical disorders related to these disorders and describe current diagnostic strategies required for their comprehensive characterization and management.

Published
2022

144. The topological line of ABJ(M) theory

Author

Domenico Seminara, Luigi Guerrini, Silvia Penati, Luca Griguolo, Nicola Gorini, Paolo Soresina, Gorini, N, Griguolo, L, Guerrini, L, Penati, S, Seminara, D, and Soresina, P

Subjects
High Energy Physics - Theory, Nuclear and High Energy Physics, Chern-Simons Theories, FOS: Physical sciences, QC770-798, Chern-Simons Theorie, Topology, 01 natural sciences, Conformal Field Theory, Extended Supersymmetry, Matrix Models, Matrix (mathematics), Nuclear and particle physics. Atomic energy. Radioactivity, 0103 physical sciences, 010306 general physics, Representation (mathematics), M-theory, Physics, Partition function (quantum field theory), 010308 nuclear & particles physics, Conformal field theory, Function (mathematics), Coupling (probability), Matrix Model, High Energy Physics - Theory (hep-th), Central charge
Abstract

We construct the one-dimensional topological sector of $\mathcal N = 6$ ABJ(M) theory and study its relation with the mass-deformed partition function on $S^3$. Supersymmetric localization provides an exact representation of this partition function as a matrix integral, which interpolates between weak and strong coupling regimes. It has been proposed that correlation functions of dimension-one topological operators should be computed through suitable derivatives with respect to the masses, but a precise proof is still lacking. We present non-trivial evidence for this relation by computing the two-point function at twoloop, successfully matching the matrix model expansion at weak coupling and finite ranks. As a by-product we obtain the two-loop explicit expression for the central charge $c_T$ of ABJ(M) theory. Three- and four-point functions up to one-loop confirm the relation as well. Our result points towards the possibility to localize the one-dimensional topological sector of ABJ(M) and may also be useful in the bootstrap program for 3d SCFTs., 38 pages, 2 figures, 3 tables

Published
2021

145. X-linked lymphoproliferative disease due to SAP/SH2D1A deficiency: a multicenter study on the manifestations, management and outcome of the disease

Author

Booth, Claire, Gilmour, Kimberly C., Veys, Paul, Gennery, Andrew R., Slatter, Mary A., Chapel, Helen, Heath, Paul T., Steward, Colin G., Smith, Owen, O'Meara, Anna, Kerrigan, Hilary, Mahlaoui, Nizar, Cavazzana-Calvo, Marina, Fischer, Alain, Moshous, Despina, Blanche, Stephane, Pachlopnik Schmid, Jana, Latour, Sylvain, de Saint-Basile, Genevieve, Albert, Michael, Notheis, Gundula, Rieber, Nikolaus, Strahm, Brigitte, Ritterbusch, Henrike, Lankester, Arjan, Hartwig, Nico G., Meyts, Isabelle, Plebani, Alessandro, Soresina, Annarosa, Finocchi, Andrea, Pignata, Claudio, Cirillo, Emilia, Bonanomi, Sonia, Peters, Christina, Kalwak, Krzysztof, Pasic, Srdjan, Sedlacek, Petr, Jazbec, Janez, Kanegane, Hirokazu, Nichols, Kim E., Hanson, I. Celine, Kapoor, Neena, Haddad, Elie, Cowan, Morton, Choo, Sharon, Smart, Joanne, Arkwright, Peter D., and Gaspar, Hubert B.

Published
2011
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147. When a Nontuberculous Mycobacterial Infection Reveals an Error of Immunity

Author

Morreale, Alessia, primary, Dotta, Laura, additional, Vairo, Donatella, additional, Bazzana, Tullia, additional, Lougaris, Vassilios, additional, Soresina, Annarosa, additional, Plebani, Alessandro, additional, Giliani, Silvia Clara, additional, Porta, Fulvio, additional, Matteelli, Alberto, additional, Redaelli De Zinis, Luca Oscar, additional, and Badolato, Raffaele, additional

Published
2022
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148. Activated phosphoinositide 3‐dinase delta syndrome (APDS): An update

Author

Lougaris, Vassilios, primary, Cancrini, Caterina, additional, Rivalta, Beatrice, additional, Castagnoli, Riccardo, additional, Giardino, Giuliana, additional, Volpi, Stefano, additional, Leonardi, Lucia, additional, La Torre, Francesco, additional, Federici, Silvia, additional, Corrente, Stefania, additional, Cinicola, Bianca Laura, additional, Soresina, Annarosa, additional, Marseglia, Gian Luigi, additional, and Cardinale, Fabio, additional

Published
2022
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