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326 results on '"Azzari, Chiara"'

1. Immunological Aspects of Kabuki Syndrome: A Retrospective Multicenter Study of the Italian Primary Immunodeficiency Network (IPINet)

Author

Rossini, Linda, Ricci, Silvia, Montin, Davide, Azzari, Chiara, Gambineri, Eleonora, Tellini, Marco, Conti, Francesca, Pession, Andrea, Saettini, Francesco, Naviglio, Samuele, Valencic, Erica, Magnolato, Andrea, Baselli, Lucia, Azzolini, Sara, Consolini, Rita, Leonardi, Lucia, D’Alba, Irene, Carraro, Elisa, Romano, Roberta, Melis, Daniela, Stagi, Stefano, Cirillo, Emilia, Giardino, Giuliana, Biffi, Alessandra, Pignata, Claudio, Putti, Maria Caterina, and Marzollo, Antonio

Published
2024
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3. Effect of antimetabolite regimen on cellular and humoral immune response to SARS-COV-2 vaccination in solid organ transplant recipients

Author

Capone, Manuela, Vanni, Anna, Salvati, Lorenzo, Lamacchia, Giulia, Mazzoni, Alessio, Maggi, Laura, Cosmi, Lorenzo, Liotta, Francesco, Romagnani, Paola, Cirillo, Luigi, Buti, Elisa, Terlizzi, Vito, Azzari, Chiara, Citera, Francesco, Barbati, Federica, Rossolini, Gian Maria, Bresci, Silvia, Borchi, Beatrice, Cavallo, Annalisa, Mencarini, Jessica, Francalanci, Emanuela, Kiros, Seble Tekle, Bartoloni, Alessandro, and Annunziato, Francesco

Published
2024
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5. Similarities and differences between myocarditis following COVID-19 mRNA vaccine and multiple inflammatory syndrome with cardiac involvement in children

Author

Amodio, Donato, Pascucci, Giuseppe Rubens, Cotugno, Nicola, Rossetti, Chiara, Manno, Emma Concetta, Pighi, Chiara, Morrocchi, Elena, D'Alessandro, Annamaria, Perrone, Marco Alfonso, Valentini, Alessandra, Franceschini, Alessio, Chinali, Marcello, Deodati, Annalisa, Azzari, Chiara, Rossi, Paolo, Cianfarani, Stefano, Andreani, Marco, Porzio, Ottavia, and Palma, Paolo

Published
2023
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7. The Impact of SARS-CoV-2 Infection in Patients with Inborn Errors of Immunity: the Experience of the Italian Primary Immunodeficiencies Network (IPINet)

Author

Giardino, Giuliana, Milito, Cinzia, Lougaris, Vassilios, 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

Published
2022
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9. Progressive Depletion of B and T Lymphocytes in Patients with Ataxia Telangiectasia: Results of the Italian Primary Immunodeficiency Network

Author

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

Published
2022
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10. CD38high/HLA‐DR+CD8+ T lymphocytes display pathogen‐specific expansion regardless of hemophagocytic lymphohistiocytosis.

Author

Lodi, Lorenzo, Sarli, Walter Maria, Ricci, Silvia, Pisano, Laura, Boscia, Silvia, Mastrolia, Maria Vincenza, Malinconi, Sara, Fusco, Eleonora, Sieni, Elena, Indolfi, Giuseppe, Simonini, Gabriele, Galli, Luisa, and Azzari, Chiara

Subjects
JUVENILE idiopathic arthritis, HEMOPHAGOCYTIC lymphohistiocytosis, T cells, CHILD patients, VISCERAL leishmaniasis, MACROPHAGE activation syndrome
Abstract

The characteristic expansion of T CD38high/HLA‐DR+CD8+ lymphocytes observed in hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) proved able to distinguish HLH/MAS from sepsis and systemic juvenile idiopathic arthritis. However, the performance of this marker in differentiating HLH/MAS from other pediatric febrile conditions with similar clinical onset and yet entirely different treatments remains unexplored. CD38high/HLA‐DR+CD8+ frequencies measured in the peripheral fresh blood of pediatric patients attended for suspicion of HLH/MAS were retrospectively recorded and clinical characteristics were retrieved. CD38high/HLA‐DR+CD8+ frequencies in HLH/MAS patients (15 patients; median: 22.0%, IQR: 11.0–49.0%) were compared with those who presented febrile conditions other‐than‐HLH (28 patients; median: 13.0%, IQR: 3.9–28.7%; p = 0.24). HLH and non‐HLH patients were subsequently regrouped based on the presence of an identified infection (22 patients; median: 27.0%, IQR: 15.2‐72.1%) and compared with those without infections (21 patients; median: 7.6%, IQR: 3.7–24.3%; p = 0.0035). CD38high/HLA‐DR+CD8+ percentages were significantly higher only in the infection group compared with the noninfection one, with a patent pathogen‐specific expansion in Epstein–Barr virus primoinfection and visceral leishmaniasis regardless of the presence of HLH. CD38high/HLA‐DR+CD8+ frequencies do not appear as an HLH‐specific marker as they naturally expand in other clinical situations that are common in childhood and may mimic HLH initial presentation. [ABSTRACT FROM AUTHOR]

Published
2024
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11. Immune response to viscerotropic Leishmania: a comprehensive review.

Author

Lodi, Lorenzo, Voarino, Marta, Stocco, Silvia, Ricci, Silvia, Azzari, Chiara, Galli, Luisa, and Chiappini, Elena

Subjects
PRIMARY immunodeficiency diseases, VISCERAL leishmaniasis, SYMPTOMS, IMMUNE checkpoint proteins, DISEASE relapse
Abstract

L. donovani and L. infantum infections are associated with a broad clinical spectrum, ranging from asymptomatic cases to visceral leishmaniasis (VL) with high mortality rates. Clinical manifestations such as post-kala-azar dermal leishmaniasis (PKDL) and visceral leishmaniasis-associated hemophagocytic lymphohistiocytosis-mimic (VL-associated HLH-mimic) further contribute to the diversity of clinical manifestations. These clinical variations are intricately influenced by the complex interplay between the host's immune response and the parasite's escape mechanisms. This narrative review aims to elucidate the underlying immunological mechanisms associated with each clinical manifestation, drawing from published literature within the last 5 years. Specific attention is directed toward viscerotropic Leishmania sinfection in patients with inborn errors of immunity and acquired immunodeficiencies. In VL, parasites exploit various immune evasion mechanisms, including immune checkpoints, leading to a predominantly anti-inflammatory environment that favors parasite survival. Conversely, nearly 70% of individuals are capable of mounting an effective pro-inflammatory immune response, forming granulomas that contain the parasites. Despite this, some patients may experience reactivation of the disease upon immunosuppression, challenging current understandings of parasite eradication. Individuals living with HIV and those with inborn errors of immunity present a more severe course of infection, often with higher relapse rates. Therefore, it is crucial to exclude both primary and acquired immune deficiencies in patients presenting disease relapse and VL-associated HLH-mimic. The distinction between VL and HLH can be challenging due to clinical similarities, suggesting that the nosological entity known as VL-associated HLH may represent a severe presentation of symptomatic VL and it should be considered more accurate referring to this condition as VL-associated HLH-mimic. Consequently, excluding VL in patients presenting with HLH is essential, as appropriate antimicrobial therapy can reverse immune dysregulation. A comprehensive understanding of the immune-host interaction underlying Leishmania infection is crucial for formulating effective treatment and preventive strategies to mitigate the disease burden. [ABSTRACT FROM AUTHOR]

Published
2024
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12. Initial presenting manifestations in 16,486 patients with inborn errors of immunity include infections and noninfectious manifestations

Author

Boztug, Kaan, Brunner, Juergen, Demel, Ulrike F., Förster-Waldl, Elisabeth, Gasteiger, Lukas M., Göschl, Lisa, Kojić, Marina, Schroll, Andrea, Seidel, Markus G., Wintergerst, Uwe, Wisgrill, Lukas, Sharapova, Svetlana O., Goffard, Jean-Christophe, Kerre, Tessa, Meyts, Isabelle, Roosens, Fine, Smet, Julie, Haerynck, Filomeen, Eric, Zelimir Pavle, Milenova, Veneta, Gagro, Alenka, Richter, Darko, Chovancova, Zita, Hlavackova, Eva, Litzman, Jiri, Milota, Tomas, Sediva, Anna, Elaziz, Dalia Abd, Alkady, Radwa Salaheldin, El Sayed El Hawary, Rabab, Eldash, Alia S., Galal, Nermeen, Lotfy, Sohilla, Meshaal, Safa S., Reda, Shereen M., Sobh, Ali, Elmarsafy, Aisha, Seppänen, Mikko R.J., Brosselin, Pauline, Courteille, Virginie, De Vergnes, Nathalie, Kracker, Sven, Pergent, Martine, Randrianomenjanahary, Philippe, Ahrenstorf, Gerrit, Albert, Michael H., Ankermann, Tobias, Atschekzei, Faranaz, Baumann, Ulrich, Becker, Benjamin C., Behrends, Uta, Belohradsky, Bernd H., Biegner, Anika-Kerstin, Binder, Nadine, Bode, Sebastian F.N., Boesecke, Christoph, Boetticher, Benedikt, Borte, Michael, Borte, Stephan, Classen, Carl Friedrich, Dirks, Johannes, Dückers, Gregor, El-Helou, Sabine, Ernst, Diana, Fasshauer, Maria, Fecker, Gisela, Felgentreff, Kerstin, Foell, Dirk, Ghosh, Sujal, Girschick, Hermann J., Goldacker, Sigune, Graf, Norbert, Graf, Dagmar, Greil, Johann, Hanitsch, Leif Gunnar, Hauck, Fabian, Heeg, Maximilian, Heine, Sabine I., Henes, Joerg C., Hoenig, Manfred, Holzer, Ursula, Holzinger, Dirk, Horneff, Gerd, Hundsdoerfer, Patrick, Jablonka, Alexandra, Jakoby, Donate, Joean, Oana, Kaiser-Labusch, Petra, Klemann, Christian, Kobbe, Robin, Körholz, Julia, Kramm, Christof M., Krüger, Renate, Landwehr-Kenzel, Sybille, Lehmberg, Kai, Liese, Johannes G., Lippert, Conrad Ferdinand, Maccari, Maria Elena, Masjosthusmann, Katja, Meinhardt, Andrea, Metzler, Markus, Morbach, Henner, Müller, Ingo, Naumann-Bartsch, Nora, Neubert, Jennifer, Niehues, Tim, Peter, Hans-Hartmut, Rieber, Nikolaus, Ritterbusch, Henrike, Rockstroh, Jürgen Kurt, Roesler, Joachim, Schauer, Uwe, Scheible, Raphael, Schmalzing, Marc, Schmidt, Reinhold Ernst, Schneider, Dominik T., Schreiber, Stefan, Schuetz, Catharina, Schulz, Ansgar, Schulze-Koops, Hendrik, Schulze-Sturm, Ulf, Schuster, Volker, Schwaneck, Eva C., Schwarz, Klaus, Schwarze-Zander, Carolynne, Sirin, Mehtap, Skapenko, Alla, Sogkas, Georgios, Sparber-Sauer, Monika, Speckmann, Carsten, Steinmann, Sandra, Stiehler, Sophie, Tenbrock, Klaus, von Bernuth, Horst, Warnatz, Klaus, Wasmuth, Jan-Christian, Weiss, Michael, Witte, Torsten, Wittke, Kirsten, Wittkowski, Helmut, Zeuner, Rainald A., Farmaki, Evangelia, Hatzistilianou, Maria N., Kakkas, Ioannis, Kanariou, Maria G., Kapousouzi, Androniki, Liatsis, Emmanouil, Maggina, Paraskevi, Papadopoulou-Alataki, Efimia, Raptaki, Maria, Speletas, Matthaios, Tantou, Sofia, Goda, Vera, Kriván, Gergely, Marodi, Laszlo, Abolhassani, Hassan, Aghamohammadi, Asghar, Rezaei, Nima, Feighery, Conleth, Leahy, Timothy Ronan, Ryan, Paul, Batzir, Nurit Assia, Garty, Ben Zion, Tamary, Hannah, Aiuti, Alessandro, Amodio, Donato, Azzari, Chiara, Barzaghi, Federica, Baselli, Lucia A., Cancrini, Caterina, Carrabba, Maria, Cazzaniga, Marco, Cesaro, Simone, Chinello, Matteo, Danieli, Maria Giovanna, Dellepiane, Rosa Maria, Fabio, Giovanna, Gambineri, Eleonora, Lodi, Lorenzo, Lougaris, Vassilios, Marasco, Carolina, Martire, Baldassarre, Marzollo, Antonio, Milito, Cinzia, Moschese, Viviana, Pignata, Claudio, Plebani, Alessandro, Porta, Fulvio, Quinti, Isabella, Ricci, Silvia, Soresina, Annarosa, Tommasini, Alberto, Vacca, Angelo, Vanessa, Clementina, Blažienė, Audra, Sitkauskiene, Brigita, Gowin, Ewelina, Heropolitańska-Pliszka, Edyta, Pietrucha, Barbara, Szaflarska, Anna, Więsik-Szewczyk, Ewa, Wolska-Kuśnierz, Beata, Esteves, Isabel, Faria, Emilia, Marques, Laura Hora, Neves, João Farela, Silva, Susana L., Teixeira, Carla, Pereira da Silva, Sara, Capilna, Brindusa Ruxandra, Guseva, Marina N., Shcherbina, Anna, Bobcakova, Anna, Ciznar, Peter, Gabzdilova, Juliana, Jesenak, Milos, Kapustova, Lenka, Orosova, Jaroslava, Petrovicova, Otilia, Raffac, Stefan, Kopač, Peter, Allende, Luis M., Antolí, Arnau, Blanch, Gemma Rocamora, Carbone, Javier, Dieli-Crimi, Romina, Garcia-Prat, Marina, Gil-Herrera, Juana, Gonzalez-Granado, Luis Ignacio, Agulló, Pilar Llobet, Olbrich, Peter, Parra-Martínez, Alba, Paz-Artal, Estela, Pleguezuelo, Daniel E., Rodríguez, Nerea Salmón, Sánchez-Ramón, Silvia, Santos-Pérez, Juan Luis, Solanich, Xavier, Soler-Palacin, Pere, González-Amores, Miriam, Ekwall, Olov, Fasth, Anders, Bitzenhofer-Grüber, Michaela, Candotti, Fabio, Dimitriou, Florentia, Heininger, Ulrich, Holbro, Andreas, Jandus, Peter, Kolios, Antonios G.A., Marschall, Karin, Schmid, Jana Pachlopnik, Posfay-Barbe, Klara M., Prader, Seraina, Reichenbach, Janine, Steiner, Urs C., Trück, Johannes, Bredius, Robbert G., de Kruijf- Bazen, Suzanne, de Vries, Esther, Henriet, Stefanie S.V., Kuijpers, Taco W., Potjewijd, Judith, Rutgers, Abraham, Stol, Kim, van Aerde, Koen J., Van den Berg, J. Merlijn, van de Ven, Annick A.J.M., Montfrans, Jorisvan, Aydemir, Sezin, Baris, Safa, Dogu, Figen, Ikinciogullari, Aydan, Karakoc-Aydiner, Elif, Kilic, Sara S., Kiykim, Ayca, Kökçü Karadağ, Şefika İlknur, Kutukculer, Necil, Ocak, Suheyla, UNAL, Ekrem, Boyarchuk, Oksana, Hilfanova, Anna, Kostyuchenko, Larysa V., Alachkar, Hana, Arkwright, Peter D., Baxendale, Helen E., Bernatoniene, Jolanta, Coulter, Tanya I., Garcez, Tomaz, Goddard, Sarah, Gompels, Mark M., Grigoriadou, Sofia, Herriot, Richard, Herwadkar, Archana, Huissoon, Aarnoud, Ibberson, Lisa, Nademi, Zoreh, Noorani, Sadia, Parvin, Shahnaz, Steele, Cathal Laurence, Thomas, Moira, Waruiru, Catherine, Yong, Patrick F.K., Bourne, Helen, Thalhammer, Julian, Kindle, Gerhard, Nieters, Alexandra, Rusch, Stephan, Fischer, Alain, Grimbacher, Bodo, Edgar, David, Buckland, Matthew, Mahlaoui, Nizar, and Ehl, Stephan

Published
2021
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13. Long-term follow-up of 168 patients with X-linked agammaglobulinemia reveals increased morbidity and mortality

Author

Lougaris, Vassilios, Soresina, Annarosa, Baronio, Manuela, Montin, Davide, Martino, Silvana, Signa, Sara, Volpi, Stefano, Zecca, Marco, Marinoni, Maddalena, Baselli, Lucia Augusta, Dellepiane, Rosa Maria, Carrabba, Maria, Fabio, Giovanna, Putti, Maria Caterina, Cinetto, Francesco, Lunardi, Claudio, Gazzurelli, Luisa, Benvenuto, Alessio, Bertolini, Patrizia, Conti, Francesca, Consolini, Rita, Ricci, Silvia, Azzari, Chiara, Leonardi, Lucia, Duse, Marzia, Pulvirenti, Federica, Milito, Cinzia, Quinti, Isabella, Cancrini, Caterina, Finocchi, Andrea, Moschese, Viviana, Cirillo, Emilia, Crescenzi, Ludovica, Spadaro, Giuseppe, Marasco, Carolina, Vacca, Angelo, Cardinale, Fabio, Martire, Baldassare, Trizzino, Antonino, Licciardello, Maria, Cossu, Fausto, Di Matteo, Gigliola, Badolato, Raffaele, Ferrari, Simona, Giliani, Silvia, Pession, Andrea, Ugazio, Alberto, Pignata, Claudio, and Plebani, Alessandro

Published
2020
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14. The Italian Registry for Primary Immunodeficiencies (Italian Primary Immunodeficiency Network; IPINet): Twenty Years of Experience (1999–2019)

Author

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

Published
2020
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15. Urogenital Abnormalities in Adenosine Deaminase Deficiency

Author

Pajno, Roberta, Pacillo, Lucia, Recupero, Salvatore, Cicalese, Maria P., Ferrua, Francesca, Barzaghi, Federica, Ricci, Silvia, Marzollo, Antonio, Pecorelli, Silvia, Azzari, Chiara, Finocchi, Andrea, Cancrini, Caterina, Di Matteo, Gigliola, Russo, Gianni, Alfano, Massimo, Lesma, Arianna, Salonia, Andrea, Adams, Stuart, Booth, Claire, and Aiuti, Alessandro

Published
2020
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18. Immunophenotype Anomalies Predict the Development of Autoimmune Cytopenia in 22q11.2 Deletion Syndrome

Author

Montin, Davide, Marolda, Agostina, Licciardi, Francesco, Robasto, Francesca, Di Cesare, Silvia, Ricotti, Emanuela, Ferro, Francesca, Scaioli, Giacomo, Giancotta, Carmela, Amodio, Donato, Conti, Francesca, Giardino, Giuliana, Leonardi, Lucia, Ricci, Silvia, Volpi, Stefano, Baselli, Lucia Augusta, Azzari, Chiara, Bossi, Grazia, Consolini, Rita, Dellepiane, Rosa Maria, Duse, Marzia, Gattorno, Marco, Martire, Baldassarre, Putti, Maria Caterina, Soresina, Annarosa, Plebani, Alessandro, Ramenghi, Ugo, Martino, Silvana, Pignata, Claudio, and Cancrini, Caterina

Published
2019
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19. Epidemiology of respiratory syncytial virus in hospitalized children over a 9-year period and preventive strategy impact.

Author

Lodi, Lorenzo, Catamerò, Francesco, Voarino, Marta, Barbati, Federica, Moriondo, Maria, Nieddu, Francesco, Sarli, Walter Maria, Citera, Francesco, Astorino, Valeria, Pelosi, Caterina, Quaranta, Francesca, Stocco, Silvia, Canessa, Clementina, Lastrucci, Vieri, Ricci, Silvia, Indolfi, Giuseppe, and Azzari, Chiara

Subjects
HOSPITAL care of children, RESPIRATORY syncytial virus, RESPIRATORY syncytial virus infections, COVID-19 pandemic, EPIDEMIOLOGY, PARAINFLUENZA viruses
Abstract

Background: Respiratory Syncytial Virus (RSV) is the primary cause of respiratory infections and hospitalizations in young children globally, leading to substantial disease burden and mortality. The aim of the present study was to review and provide updates on how the SARS-CoV-2 pandemic have significantly influenced RSV epidemiology on hospitalized children due to RSV infection. A potential impact of the available preventive strategies on the same population were provided. Methods: All children aged 0-6 years hospitalized at Meyer Children's Hospital IRCCS for RSV infection from September 2014 to March 2023 were retrospectively recorded. Seasonal trends before and after SARS-CoV-2 pandemic, age distribution, ICU admission and co-infections, comorbidities and prematurity were retrieved. Predictions on the number of hospitalizations avoided by the deployment of different preventive strategies were provided. Results: A total of 1,262 children with RSV infection were included in the study. The 70% of them had less than 1 year-of-age at the moment of hospitalization and almost 50% less than 3 months. In the post-pandemic seasons, a 317% increase in the number of hospitalizations was recorded with a significant increase in older children compared to the pre-pandemic seasons. ICU support was required for 22% of children, the majority of whom were under 3 months of age. Almost 16% of hospitalized children were born preterm and only 27% of hospitalized children had prior comorbidities. The rate of comorbidities among RSV hospitalized children increased with age. Nirsevimab prophylaxis could have prevented more than 46% of hospitalizations in this cohort. A preventive strategy addressing also children aged 7 months to 6 years of age with co-existing comorbidities would increase that rate above 57%. Discussion: The identification of RSV hospitalization-related features is informing the decision-maker for the deployment of the wisest preventive approach on a population scale. [ABSTRACT FROM AUTHOR]

Published
2024
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21. HLH as an additional warning sign of inborn errors of immunity beyond familial-HLH in children: a systematic review.

Author

Ricci, Silvia, Sarli, Walter Maria, Lodi, Lorenzo, Canessa, Clementina, Lippi, Francesca, Dini, Donata, Ferrari, Marta, Pisano, Laura, Sieni, Elena, Indolfi, Giuseppe, Resti, Massimo, and Azzari, Chiara

Subjects
HEMOPHAGOCYTIC lymphohistiocytosis, IMMUNITY, CENTRAL nervous system, MACROPHAGE activation syndrome, TREATMENT effectiveness
Abstract

Background: Hemophagocytic Lymphohistiocytosis (HLH) is a rare and lifethreatening condition characterized by a severe impairment of the immune homeostasis. While Familial-HLH (FHL) is a known cause, the involvement of other Inborn Errors of Immunity (IEI) in pediatric-HLH remains understudied. Objective: This systematic review aimed to assess the clinical features, triggers, laboratory data, treatment, and outcomes of pediatric HLH patients with IEI other than FHL (IEInotFHL), emphasizing the importance of accurate identification and management. Methods: A systematic search for studies meeting inclusion criteria was conducted in PubMed, EMBASE, MEDLINE, and Cochrane Central. Quality assessment was performed through JBI criteria. Results: A comprehensive search yielded 108 records meeting inclusion criteria, involving 178 patients. We identified 46 different IEI according to IUIS 2022 Classification. Combined immunodeficiencies, immune dysregulation disorders, and phagocyte defects were the IEI most frequently associated with HLH. In 75% of cases, HLH preceded the IEI diagnosis, often with an unrecognized history of severe infections. Triggers reflected the specific infection susceptibilities within IEI groups. Liver and central nervous system involvement were less common than in FHL cases. Treatment approaches and outcomes varied, with limited long-term follow-up data, limiting the assessment of therapeutic efficacy across IEI groups. Conclusion: A comprehensive evaluation encompassing immunological, infectious, and genetic aspects is essential in pediatric-HLH. Relying solely on FHL or EBV susceptibility disorders tests is insufficient, as diverse other IEI can contribute to HLH. Early recognition of HLH as a potential warning sign can guide timely diagnostic investigations and facilitate tailored therapeutic interventions for improved outcomes. [ABSTRACT FROM AUTHOR]

Published
2024
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27. The Inborn Errors of Immunity—Virtual Consultation System Platform in Service for the Italian Primary Immunodeficiency Network: Results from the Validation Phase.

Author

Coppola, Emma, Sgrulletti, Mayla, Cortesi, Manuela, Romano, Roberta, Cirillo, Emilia, Giardino, Giuliana, Dotta, Laura, Cancrini, Caterina, Bruzzese, Dario, Badolato, Raffaele, Moschese, Viviana, Pignata, Claudio, Alessandro, Aiuti, Amodio, Donato, Azzari, Chiara, Canessa, Clementina, Caorsi, Roberta, Castagnoli, Riccardo, Cicalese, Maria Pia, and Conti, Francesca

Subjects
TELEMEDICINE, PRIMARY immunodeficiency diseases, DELAYED diagnosis, IMMUNODIAGNOSIS, IMMUNITY
Abstract

Purpose: Inborn errors of immunity (IEI) represent a heterogeneous group of rare genetically determined diseases. In some cases, patients present with complex or atypical phenotypes, not fulfilling the accepted diagnostic criteria for IEI and, thus, at high risk of misdiagnosis or diagnostic delay. This study aimed to validate a platform that, through the opinion of immunologist experts, improves the diagnostic process and the level of care of patients with atypical/complex IEI. Methods: Here, we describe the functioning of the IEI-Virtual Consultation System (VCS), an innovative platform created by the Italian Immunodeficiency Network (IPINet). Results: In the validation phase, from January 2020 to June 2021, 68 cases were entered on the IEI-VCS platform. A final diagnosis was achieved in 35/68 cases (51%, 95% CI 38.7 to 64.2). In 22 out of 35 solved cases, the diagnosis was confirmed by genetic analysis. In 3/35 cases, a diagnosis of secondary immunodeficiency was made. In the remaining 10 cases, an unequivocal clinical and immunological diagnosis was obtained, even though not substantiated by genetic analysis. Conclusion: From our preliminary study, the VCS represents an innovative and useful system to improve the diagnostic process of patients with complex unsolved IEI disorders, with benefits both in terms of reduction of time of diagnosis and access to the required therapies. These results may help the functioning of other international platforms for the management of complex cases. [ABSTRACT FROM AUTHOR]

Published
2024
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31. Carriage rates and risk factors during an outbreak of invasive meningococcal disease due to Neisseria meningitidis serogroup C ST-11 (cc11) in Tuscany, Italy: a cross-sectional study

Author

Miglietta, Alessandro, Innocenti, Francesco, Pezzotti, Patrizio, Riccobono, Eleonora, Moriondo, Maria, Pecile, Patrizia, Nieddu, Francesco, Rossolini, Gian Maria, Azzari, Chiara, Meningococcal Carriage Study Group, Balocchini, Emanuela, Rezza, Giovanni, Voller, Fabio, and Stefanelli, Paola

Published
2019
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32. Diagnosis of immunodeficiency caused by a purine nucleoside phosphorylase defect by using tandem mass spectrometry on dried blood spots

Author

la Marca, Giancarlo, Canessa, Clementina, Giocaliere, Elisa, Romano, Francesca, Malvagia, Sabrina, Funghini, Silvia, Moriondo, Maria, Valleriani, Claudia, Lippi, Francesca, Ombrone, Daniela, Della Bona, Maria Luisa, Speckmann, Carsten, Borte, Stephan, Brodszki, Nicholas, Gennery, Andrew R., Weinacht, Katja, Celmeli, Fatih, Pagel, Julia, de Martino, Maurizio, Guerrini, Renzo, Wittkowski, Helmut, Santisteban, Ines, Bali, Pawan, Ikinciogullari, Aydan, Hershfield, Michael, Notarangelo, Luigi D., Resti, Massimo, and Azzari, Chiara

Published
2014
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35. Hospitalisation trends from 2019 to 2023 illustrate the persistent resurgence of bronchiolitis in Italy.

Author

Palmas, Giordano, Resti, Massimo, Indolfi, Giuseppe, Trapani, Sandra, Azzari, Chiara, Zanobini, Alberto, Agosti, Massimo, Bresesti, Ilia, Marchisio, Paola, Milani, Gregorio Paolo, Alberti, Ilaria, Aricò, Maurizio, Bressan, Silvia, Da Dalt, Liviana, Tirelli, Francesca, Caselli, Desiree, Cazzato, Salvatore, Martelli, Gaia, Felici, Enrico, and Garazzino, Silvia

Subjects
BRONCHIOLITIS, SARS-CoV-2
Abstract

A retrospective multicentre study conducted in Italy analyzed hospitalization trends for bronchiolitis in infants between October 2019 and March 2023. The study found that bronchiolitis cases, particularly those caused by the respiratory syncytial virus (RSV), experienced a significant reduction during the 2020-2021 winter due to preventive measures implemented during the COVID-19 pandemic. However, subsequent winters saw a notable and sustained increase in bronchiolitis hospitalizations, with the 2022-2023 winter period experiencing a 47% higher rate compared to the pre-pandemic winter of 2019-2020. The changing seasonality of bronchiolitis, including altered patterns of RSV circulation, poses challenges for disease control strategies and immunoprophylaxis measures. Further research is needed to monitor the epidemiology of bronchiolitis and inform public health interventions. [Extracted from the article]

Published
2024
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37. Reshaping Our Knowledge: Advancements in Understanding the Immune Response to Human Respiratory Syncytial Virus.

Author

Attaianese, Federica, Guiducci, Sara, Trapani, Sandra, Barbati, Federica, Lodi, Lorenzo, Indolfi, Giuseppe, Azzari, Chiara, and Ricci, Silvia

Subjects
RESPIRATORY syncytial virus, IMMUNE response, T cells, RESPIRATORY infections, IMMUNOLOGIC memory, ANTIGEN presentation, T cell receptors
Abstract

Human respiratory syncytial virus (hRSV) is a significant cause of respiratory tract infections, particularly in young children and older adults. In this review, we aimed to comprehensively summarize what is known about the immune response to hRSV infection. We described the innate and adaptive immune components involved, including the recognition of RSV, the inflammatory response, the role of natural killer (NK) cells, antigen presentation, T cell response, and antibody production. Understanding the complex immune response to hRSV infection is crucial for developing effective interventions against this significant respiratory pathogen. Further investigations into the immune memory generated by hRSV infection and the development of strategies to enhance immune responses may hold promise for the prevention and management of hRSV-associated diseases. [ABSTRACT FROM AUTHOR]

Published
2023
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38. Risk of thyroid neoplasms in patients with 22q11.2 deletion and DiGeorge-like syndromes: an insight for follow-up.

Author

Sarli, Walter Maria, Ricci, Silvia, Lodi, Lorenzo, Cavone, Federica, Pacillo, Lucia, Giancotta, Carmela, Ubertini, Graziamaria, Baroncelli, Giampiero, Cancrini, Caterina, Azzari, Chiara, and Stagi, Stefano

Subjects
DIGEORGE syndrome, 22Q11 deletion syndrome, CONGENITAL heart disease, TUMORS, THYROID gland, HEAD & neck cancer
Abstract

Introduction: The chromosome 22q11.2 deletion syndrome comprises phenotypically similar diseases characterized by abnormal development of the third and fourth branchial arches, resulting in variable combinations of congenital heart defects, dysmorphisms, hypocalcemia, palatal dysfunction, developmental or neuropsychiatric disorders, and impairment of the immune system due to thymic dysfunction. Other genetic syndromes, often called DiGeorge-like, share clinical and immunological features with 22q11.2 deletion syndrome. This syndrome has been rarely associated with malignancies, mainly hematological but also hepatic, renal, and cerebral. Rarely, malignancies in the head and neck region have been described, although no aggregate of data on the development of thyroid neoplasms in patients with this clinical phenotype has been conducted so far. Materials and methods: To characterize this possible association, a multicenter survey was made. Thus, we present a case series of five pediatric patients with 22q11.2 deletion syndrome or DiGeorge-like syndrome who were occasionally found with confirmed or highly suspected neoplasms of the thyroid gland during their follow-up. In three cases, malignancies were histologically confirmed, but their outcome was good due to an early recognition of suspicious nodules and precocious surgery. Conclusions: This study underlines for clinicians the higher risk of neoplasms in the head and neck district for patients affected by these syndromes. It also emphasizes the importance of a prolonged clinical and ultrasound follow-up for patients with this clinical and immunological phenotype. [ABSTRACT FROM AUTHOR]

Published
2023
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39. Underestimation of invasive meningococcal disease in Italy

Author

Azzari, Chiara, Nieddu, Francesco, Moriondo, Maria, Indolfi, Giuseppe, Canessa, Clementina, Ricci, Silvia, Bianchi, Leila, Serranti, Daniele, Poggi, Giovanni Maria, and Resti, Massimo

Subjects
Meningitis -- Diagnosis -- Comparative analysis, Vaccination -- Comparative analysis, Health
Abstract

Neisseria meningitidis is the major etiologic agent of bacterial meningitis and one of the most important causes of invasive bacterial disease worldwide (1,2). The annual number of invasive meningococcal disease [...]

Published
2016
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44. Development and validation of a 2nd tier test for identification of purine nucleoside phosphorylase deficiency patients during expanded newborn screening by liquid chromatography-tandem mass spectrometry

Author

la Marca, Giancarlo, Giocaliere, Elisa, Malvagia, Sabrina, Villanelli, Fabio, Funghini, Silvia, Ombrone, Daniela, Bona, Maria Della, Forni, Giulia, Canessa, Clementina, Ricci, Silvia, Romano, Francesca, Guerrini, Renzo, Resti, Massimo, and Azzari, Chiara

Published
2016
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45. CD38high/HLA-DR+ CD8+ T cells as potential biomarker of hemophagocytic lymphohistiocytosis secondary to visceral Leishmania infection.

Author

Mastrolia, Maria Vincenza, Boscia, Silvia, Galli, Luisa, Lodi, Lorenzo, Pisano, Laura, Maccora, Ilaria, Ricci, Silvia, Pagnini, Ilaria, Marrani, Edoardo, Azzari, Chiara, and Simonini, Gabriele

Subjects
HEMOPHAGOCYTIC lymphohistiocytosis, T cells, LEISHMANIASIS, BIOMARKERS, SEPSIS
Abstract

Leishmaniasis is a cause of infection associated with hemophagocytic lymphohistiocytosis (HLH). The measurement of the CD8+ CD38high/HLA-DR+ T cells in children presenting with acute onset of shock and multisystem organ failure represents an important parameter to distinguish HLH from sepsis or healthy control. Conclusion: We report a case series of 4 Italian children suffering from HLH secondary to visceral Leishmaniasis in which the lymphocyte subset assay suggests a potential role of CD38high/HLA-DR+ CD8+ T cells as HLH diagnostic biomarkers. What is Known: • Visceral Leishmaniasis is a well-known cause of infection associated with hemophagocytic lymphohistiocytosis (HLH). • The measurement of the CD8+CD38high/HLA-DR+T cells in children presenting with acute onset of shock and multisystem organ failure represents an important diagnostically useful parameter to readily distinguish HLH from sepsis or healthy controls. What is New: • We report a case series of 4 Italian children suffering from HLH secondary to visceral Leishmaniasis in which the lymphocyte subset assay suggests a potential role of CD38high/HLA-DR+CD8+T cells as HLH diagnostic biomarker. • The flow cytometry assay, performed at the disease onset before starting treatment, revealed a mean percentage value of CD38 cells of 36.95% among CD8+T cells. [ABSTRACT FROM AUTHOR]

Published
2023
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46. Immunogenicity and safety of a quadrivalent meningococcal tetanus toxoid-conjugate vaccine (MenACYW-TT): A review of the evidence and expert opinion.

Author

Ricci, Silvia, Azzari, Chiara, Amodio, Emanuele, and Castiglia, Paolo

Subjects
MENINGOCOCCAL infections, TETANUS vaccines, BOOSTER vaccines, IMMUNE response, VACCINATION of children, MENINGOCOCCAL vaccines
Abstract

Serogroups A, B, C, W, X, and Y of Neisseria meningitidis are responsible for almost all cases of invasive meningococcal disease. In Italy, vaccination against serogroup B is recommended at 3–13 months, C at 13–15 months, and A, C, Y and W in adolescents (12–18 years). Four quadrivalent meningococcal conjugate vaccines are available. This review describes the available data on a quadrivalent meningococcal tetanus toxoid-conjugate vaccine (MenACYW-TT; MenQuadfi®; Sanofi). We identified articles on quadrivalent meningococcal conjugate vaccines indexed on PubMed since 2000. Of the 524 studies identified, 10 human studies investigating the immunogenicity and safety of MenACYW-TT in toddlers, children aged 2–9 years, and individuals 10–55 or ≥56 years are described in detail. In Italy, pediatric and public health groups recommend amending the current vaccination schedule to include a booster dose between 6 and 9 years and quadrivalent vaccine in young adults (≥19 years), targeting waning protection after childhood vaccination and the age cohort with the highest carrier prevalence (adolescents and young adults). MenACYW-TT is a suitable meningococcal vaccine for current and pending recommendations based on high seroprotection rates and a low incidence of adverse events in these age groups. Moreover, it does not require reconstitution. [ABSTRACT FROM AUTHOR]

Published
2023
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49. DR4/DQ2 haplotype confers susceptibility to T1DM with early clinical disease onset: A retrospective analysis in a tertiary-care hospital in Italy.

Author

Ricci, Silvia, Perugia, Francesca, Piccini, Barbara, Lodi, Lorenzo, Pegoraro, Francesco, Giovannini, Mattia, Rombolà, Giovanni, Perferi, Giancarlo, Toni, Sonia, and Azzari, Chiara

Subjects
HAPLOTYPES, TYPE 1 diabetes, DIABETES in children, CHILDREN'S hospitals, ITALIANS, INSULIN antibodies
Abstract

Introduction: T1DM is the most frequent form of diabetes in children. It has a multifactorial pathogenesis in which genetic, environmental and immunological factors are involved. Among genetic explanations a major role is attributed to second class HLA genes, with the greatest risk associated with the simultaneous presence of the haplotypes DR3DQ2 and DR4DQ8. Based on results obtained in other countries, the aim of this research is to verify a possible association between the haplotype DRB1 * 04: 05-DQA1 * 03-DQB1 * 02 and the onset of T1DM among Italian children with possible genotype-phenotype correlations. Greater knowledge of genes which increase or decrease susceptibility is important for genome analysis. Materials and methods: 165 patients with type 1 diabetes treated at the Diabetology Unit of the Meyer Children's University Hospital, were clinically analyzed. Data relating to age at diagnosis, pancreatic anti-beta cell autoimmunity, comorbidities with date of diagnosis and family history were retrospectively collected from medical data. A case-control study was conducted to investigate the HLA types of the patients compared to a control group of 819 Tuscan donors enrolled in the National Bone Marrow Donor Register. Typing was carried out using the Eurospital "DIABEGEN" kit, currently in use at the immunology laboratory of the Meyer Children's University Hospital. Results: Mean age at diagnosis was 9.3 years; most children (97%) had anti-pancreatic beta cell autoimmunity; the anti-insulin antibody (IAA) was more frequent among children with early clinical disease onset (0–5 years of age). From the case control comparison performed on HLA typing, it emerged that the greatest risk for the development of type 1 diabetes is conferred by the haplotypes DR3DQ2 and DR4DQ8, but in addition to these haplotypes, already known in other countries, we identified another haplotype, DR4DQ2 (DRB1 * 04: 05-DQA1 * 03-DQB1 * 02) which appears to predispose children to type 1 diabetes (p value 2.80E-08) and it is associated with early clinical disease onset (p-value = 0.002). Conclusions: We report a new haplotype which increases susceptibility to type 1 diabetes among Italian children and which is associated with early clinical disease onset. Given the central role attributed to genetic factors in the pathogenesis of T1DM and to the II class HLA genes, this new haplotype ought to be recognized as a risk factor and included in tests routinely carried out to identify patients with a genetic predisposition to type I diabetes in Italy. These findings could have practical implications in research and prevention programs. [ABSTRACT FROM AUTHOR]

Published
2022
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