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2. Preliminary data revealing efficacy of Streptococcus salivarius K12 (SSK12) in Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome: a multicenter study from the AIDA Network PFAPA syndrome registry

Author

La Torre, F, Sota, J, Insalaco, A, Conti, G, Del Giudice, E, Lubrano, R, Breda, L, Maggio, Mc, Civino, A, Mastrorilli, V, Loconte, R, Natale, Mf, Celani, C, Romeo, M, Patroniti, S, Gentile, C, Vitale, A, Caggiano, V, Gaggiano, C, Diomeda, F, Cattalini, M, Lopalco, G, Emmi, G, Parronchi, P, Gentileschi, S, Cardinale, F, Aragona, E, Shahram, F, Marino, A, Barone, P, Moscheo, C, Ozkiziltas, B, Carubbi, F, Alahmed, O, Iezzi, Ludovica, Ogunjimi, B, Mauro, A, Tarsia, M, Mahmoud, Aaa, Mayrink Giardini, Ham, Sfikakis, Pp, Laskari, K, Więsik-Szewczyk, E, Hernàndez-Rodriguez, J, Frediani, B, Gòmez-Caverzaschi, V, Tufan, A, Almaghlouth, Ia, Balistreri, A, Ragab, G, Fabiani, C, Cantarini, L, Rigante, Donato, La Torre F, Sota J, Insalaco A, Conti G, Del Giudice E, Lubrano R, Breda L, Maggio MC, Civino A, Mastrorilli V, Loconte R, Natale MF, Celani C, Romeo M, Patroniti S, Gentile C, Vitale A, Caggiano V, Gaggiano C, Diomeda F, Cattalini M, Lopalco G, Emmi G, Parronchi P, Gentileschi S, Cardinale F, Aragona E, Shahram F, Marino A, Barone P, Moscheo C, Ozkiziltas B, Carubbi F, Alahmed O, Iezzi L, Ogunjimi B, Mauro A, Tarsia M, Mahmoud AAA, Mayrink Giardini HAM, Sfikakis PP, Laskari K, Więsik-Szewczyk E, Hernàndez-Rodriguez J, Frediani B, Gòmez-Caverzaschi V, Tufan A, Almaghlouth IA, Balistreri A, Ragab G, Fabiani C, Cantarini L, Rigante D (ORCID:0000-0001-7032-7779), La Torre, F, Sota, J, Insalaco, A, Conti, G, Del Giudice, E, Lubrano, R, Breda, L, Maggio, Mc, Civino, A, Mastrorilli, V, Loconte, R, Natale, Mf, Celani, C, Romeo, M, Patroniti, S, Gentile, C, Vitale, A, Caggiano, V, Gaggiano, C, Diomeda, F, Cattalini, M, Lopalco, G, Emmi, G, Parronchi, P, Gentileschi, S, Cardinale, F, Aragona, E, Shahram, F, Marino, A, Barone, P, Moscheo, C, Ozkiziltas, B, Carubbi, F, Alahmed, O, Iezzi, Ludovica, Ogunjimi, B, Mauro, A, Tarsia, M, Mahmoud, Aaa, Mayrink Giardini, Ham, Sfikakis, Pp, Laskari, K, Więsik-Szewczyk, E, Hernàndez-Rodriguez, J, Frediani, B, Gòmez-Caverzaschi, V, Tufan, A, Almaghlouth, Ia, Balistreri, A, Ragab, G, Fabiani, C, Cantarini, L, Rigante, Donato, La Torre F, Sota J, Insalaco A, Conti G, Del Giudice E, Lubrano R, Breda L, Maggio MC, Civino A, Mastrorilli V, Loconte R, Natale MF, Celani C, Romeo M, Patroniti S, Gentile C, Vitale A, Caggiano V, Gaggiano C, Diomeda F, Cattalini M, Lopalco G, Emmi G, Parronchi P, Gentileschi S, Cardinale F, Aragona E, Shahram F, Marino A, Barone P, Moscheo C, Ozkiziltas B, Carubbi F, Alahmed O, Iezzi L, Ogunjimi B, Mauro A, Tarsia M, Mahmoud AAA, Mayrink Giardini HAM, Sfikakis PP, Laskari K, Więsik-Szewczyk E, Hernàndez-Rodriguez J, Frediani B, Gòmez-Caverzaschi V, Tufan A, Almaghlouth IA, Balistreri A, Ragab G, Fabiani C, Cantarini L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: To evaluate the potential role of Streptococcus salivarius K12 (SSK12) in controlling febrile flares in patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Further aims were to assess the impact of SSK12 on (i) flare duration, (ii) variation in the degree of the highest body temperature during flares, (iii) steroid-sparing effect, and (iv) change of PFAPA accompanying symptoms before and after SSK12 introduction. Patients and methods: The medical charts from 85 pediatric patients with PFAPA syndrome (49 males and 36 females) enrolled in the AIDA registry and treated with SSK12 for a median period of 6.00±7.00 months in the period between September 2017 and May 2022 were examined. Children recruited had a median time of disease duration of 19.00±28.00 months. Results: The number of febrile flares significantly decreased comparing the 12 months before [median (IQR), 13.00 (6.00)] and after SSK12 initiation [median (IQR), 5.50 (8.00), p<0.001]. The duration of fever was significantly reduced from 4.00 (2.00) days to 2.00 (2.00) days [p<0.001]. Similarly, the highest temperature in °C was found significantly lower in the last follow-up assessment [median (IQR), 39.00 (1.00)] compared to the period prior to SSK12 start [median (IQR), 40.00 (1.00), p<0.001]. Steroid load (mg/year) of betamethasone (or any equivalent steroid) significantly decreased between 12 months before treatment with SSK12 [median (IQR), 5.00 (8.00) mg/year] and the last follow-up visit [median (IQR), 2.00 (4.00) mg/year, p<0.001]. The number of patients experiencing symptoms including pharyngitis/tonsillitis (p<0.001), oral aphthae (p<0.001) and cervical lymphadenopathy (p<0.001) significantly decreased following SSK12. Conclusion: SSK12 prophylaxis given for at least 6.00±7.00 months was found to reduce febrile flares of PFAPA syndrome: in particular, it halved the total number per year of fever flares, shortened the

Published
2023

3. The administration of methotrexate in patients with Still’s disease, “real-life” findings from AIDA Network Still Disease Registry

Author

362., Ruscitti P, Sota, J, Vitale, A, Lopalco, G, Iannone, F, Morrone, M, Giardini, Ham, D'Agostin, Ma, Antonelli, Ipb, Almaghlouth, I, Asfina, Kn, Khalil, N, Sfikakis, Pp, Laskari, K, Tektonidou, M, Ciccia, F, Iacono, D, Riccio, F, Ragab, G, Hussein, Ma, Govoni, M, Ruffilli, F, Direskeneli, H, Alibaz-Oner, F, Giacomelli, R, Navarini, L, Bartoloni, E, Riccucci, I, Martín-Nares, E, Torres-Ruiz, J, Cipriani, P, Di Cola, I, Hernández-Rodríguez, J, Gómez-Caverzaschi, V, Dagna, L, Tomelleri, A, Makowska, J, Brzezinska, O, Iagnocco, A, Bellis, E, Caggiano, V, Gaggiano, C, Tarsia, M, Mormile, I, Emmi, G, Sfriso, P, Monti, S, Erten, Ş, Del Giudice, E, Lubrano, R, Conti, G, Olivieri, An, Lo Gullo, A, Tharwat, S, Karamanakos, A, Gidaro, A, Maggio, Mc, La Torre, F, Cardinale, F, Ogunjimi, B, Maier, A, Sebastiani, Gd, Opris-Belinski, D, Frassi, M, Viapiana, O, Bizzi, E, Carubbi, F, Fotis, L, Tufan, A, Kardas, Rc, Więsik-Szewczyk, E, Jahnz-Różyk, K, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, 362. Ruscitti P, Sota J, Vitale A, Lopalco G, Iannone F, Morrone M, Giardini HAM, D'Agostin MA, Antonelli IPB, Almaghlouth I, Asfina KN, Khalil N, Sfikakis PP, Laskari K, Tektonidou M, Ciccia F, Iacono D, Riccio F, Ragab G, Hussein MA, Govoni M, Ruffilli F, Direskeneli H, Alibaz-Oner F, Giacomelli R, Navarini L, Bartoloni E, Riccucci I, Martín-Nares E, Torres-Ruiz J, Cipriani P, Di Cola I, Hernández-Rodríguez J, Gómez-Caverzaschi V, Dagna L, Tomelleri A, Makowska J, Brzezinska O, Iagnocco A, Bellis E, Caggiano V, Gaggiano C, Tarsia M, Mormile I, Emmi G, Sfriso P, Monti S, Erten Ş, Del Giudice E, Lubrano R, Conti G, Olivieri AN, Lo Gullo A, Tharwat S, Karamanakos A, Gidaro A, Maggio MC, La Torre F, Cardinale F, Ogunjimi B, Maier A, Sebastiani GD, Opris-Belinski D, Frassi M, Viapiana O, Bizzi E, Carubbi F, Fotis L, Tufan A, Kardas RC, Więsik-Szewczyk E, Jahnz-Różyk K, Fabiani C, Frediani B, Balistreri A, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, 362., Ruscitti P, Sota, J, Vitale, A, Lopalco, G, Iannone, F, Morrone, M, Giardini, Ham, D'Agostin, Ma, Antonelli, Ipb, Almaghlouth, I, Asfina, Kn, Khalil, N, Sfikakis, Pp, Laskari, K, Tektonidou, M, Ciccia, F, Iacono, D, Riccio, F, Ragab, G, Hussein, Ma, Govoni, M, Ruffilli, F, Direskeneli, H, Alibaz-Oner, F, Giacomelli, R, Navarini, L, Bartoloni, E, Riccucci, I, Martín-Nares, E, Torres-Ruiz, J, Cipriani, P, Di Cola, I, Hernández-Rodríguez, J, Gómez-Caverzaschi, V, Dagna, L, Tomelleri, A, Makowska, J, Brzezinska, O, Iagnocco, A, Bellis, E, Caggiano, V, Gaggiano, C, Tarsia, M, Mormile, I, Emmi, G, Sfriso, P, Monti, S, Erten, Ş, Del Giudice, E, Lubrano, R, Conti, G, Olivieri, An, Lo Gullo, A, Tharwat, S, Karamanakos, A, Gidaro, A, Maggio, Mc, La Torre, F, Cardinale, F, Ogunjimi, B, Maier, A, Sebastiani, Gd, Opris-Belinski, D, Frassi, M, Viapiana, O, Bizzi, E, Carubbi, F, Fotis, L, Tufan, A, Kardas, Rc, Więsik-Szewczyk, E, Jahnz-Różyk, K, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, 362. Ruscitti P, Sota J, Vitale A, Lopalco G, Iannone F, Morrone M, Giardini HAM, D'Agostin MA, Antonelli IPB, Almaghlouth I, Asfina KN, Khalil N, Sfikakis PP, Laskari K, Tektonidou M, Ciccia F, Iacono D, Riccio F, Ragab G, Hussein MA, Govoni M, Ruffilli F, Direskeneli H, Alibaz-Oner F, Giacomelli R, Navarini L, Bartoloni E, Riccucci I, Martín-Nares E, Torres-Ruiz J, Cipriani P, Di Cola I, Hernández-Rodríguez J, Gómez-Caverzaschi V, Dagna L, Tomelleri A, Makowska J, Brzezinska O, Iagnocco A, Bellis E, Caggiano V, Gaggiano C, Tarsia M, Mormile I, Emmi G, Sfriso P, Monti S, Erten Ş, Del Giudice E, Lubrano R, Conti G, Olivieri AN, Lo Gullo A, Tharwat S, Karamanakos A, Gidaro A, Maggio MC, La Torre F, Cardinale F, Ogunjimi B, Maier A, Sebastiani GD, Opris-Belinski D, Frassi M, Viapiana O, Bizzi E, Carubbi F, Fotis L, Tufan A, Kardas RC, Więsik-Szewczyk E, Jahnz-Różyk K, Fabiani C, Frediani B, Balistreri A, Rigante D (ORCID:0000-0001-7032-7779), and Cantarini L

Abstract

Objectives: To describe clinical characteristics of patients with Still's disease treated with methotrexate (MTX) and to assess drug effectiveness evaluating change in disease activity, reduction of inflammatory markers, and glucocorticoid (GC)-sparing effect. Methods: Patients with Still's disease treated with MTX were assessed among those included in AIDA Network Still Disease Registry. Results: In this registry, 171 patients with Still's disease were treated with MTX (males 43.3%, age 37.1 ± 16.0 years). They were mainly characterised by joint features and fever without a prominent multiorgan involvement. MTX was administered with GCs in 68.4% of patients, with other conventional synthetic DMARDs in 6.4%, and with biologic DMARDs in 25.1%. A significant reduction of the modified systemic score was observed, and 38.6% patients were codified as being in clinical remission at the end of follow-up. The concomitant administration of a biologic DMARD resulted a predictor of the clinical remission. Furthermore, a reduction of inflammatory markers and ferritin levels was observed following the administration of MTX. Additionally, a marked reduction of the dosage of concomitant GCs was identified, while 36.7% discontinued such drugs. Male gender appeared as a predictor of GC discontinuation. MTX was discontinued in 12.3% of patients because of adverse effects, and in 12.3% for lack of efficacy. Conclusions: Clinical characteristics of patients with Still's disease treated with MTX were described, mainly joint features and fever without a prominent multiorgan involvement. The clinical usefulness of MTX was reported in reducing the disease activity, decreasing the inflammatory markers, and as GC-sparing agent.

Published
2023

6. The AutoInflammatory Diseases Alliance Registry of monogenic autoinflammatory diseases

Author

Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, Conti G, Gaggiano, C, Vitale, A, Tufan, A, Ragab, G, Aragona, E, Wiesik-Szewczyk, E, Ait-Idir, D, Conti, G, Iezzi, L, Maggio, Mc, Cattalini, M, La Torre, F, Lopalco, G, Verrecchia, E, de Paulis, A, Sahin, A, Insalaco, A, Sfikakis, Pp, Marino, A, Frassi, M, Ogunjimi, B, Opris-Belinski, D, Parronchi, P, Emmi, G, Shahram, F, Ciccia, F, Piga, M, Hernández-Rodríguez, J, Pereira, Rmr, Alessio, M, Naddei, R, Olivieri, An, Giudice, Ed, Sfriso, P, Ruscitti, P, Gobbi, Fl, Kucuk, H, Sota, J, Hussein, Ma, Malizia, G, Jahnz-Różyk, K, Sari-Hamidou, R, Romeo, M, Ricci, F, Cardinale, F, Iannone, F, Casa, Fd, Natale, Mf, Laskari, K, Giani, T, Franceschini, F, Sabato, V, Yildirim, D, Caggiano, V, Hegazy, Mt, Marzo, Rd, Kucharczyk, A, Khellaf, G, Tarsia, M, Almaghlouth, Ia, Laymouna, Ah, Mastrorilli, V, Dotta, L, Benacquista, L, Grosso, S, Crisafulli, F, Parretti, V, Giordano, Hf, Mahmoud, Aaa, Nuzzolese, R, Musso, M, Chighizola, Cb, Gentileschi, S, Morrone, M, Cola, Id, Spedicato, V, Giardini, Ham, Vasi, I, Renieri, A, Fabbiani, A, Mencarelli, Ma, Frediani, B, Balistreri, A, Tosi, Gm, Fabiani, C, Lidar, M, Rigante, D, Cantarini, L, Gaggiano C, Vitale A, Tufan A, Ragab G, Aragona E, Wiesik-Szewczyk E, Ait-Idir D, Iezzi L, Maggio MC, Cattalini M, La Torre F, Lopalco G, Verrecchia E, de Paulis A, Sahin A, Insalaco A, Sfikakis PP, Marino A, Frassi M, Ogunjimi B, Opris-Belinski D, Parronchi P, Emmi G, Shahram F, Ciccia F, Piga M, Hernández-Rodríguez J, Pereira RMR, Alessio M, Naddei R, Olivieri AN, Giudice ED, Sfriso P, Ruscitti P, Gobbi FL, Kucuk H, Sota J, Hussein MA, Malizia G, Jahnz-Różyk K, Sari-Hamidou R, Romeo M, Ricci F, Cardinale F, Iannone F, Casa FD, Natale MF, Laskari K, Giani T, Franceschini F, Sabato V, Yildirim D, Caggiano V, Hegazy MT, Marzo RD, Kucharczyk A, Khellaf G, Tarsia M, Almaghlouth IA, Laymouna AH, Mastrorilli V, Dotta L, Benacquista L, Grosso S, Crisafulli F, Parretti V, Giordano HF, Mahmoud AAA, Nuzzolese R, Musso M, Chighizola CB, Gentileschi S, Morrone M, Cola ID, Spedicato V, Giardini HAM, Vasi I, Renieri A, Fabbiani A, Mencarelli MA, Frediani B, Balistreri A, Tosi GM, Fabiani C, Lidar M, Rigante D (ORCID:0000-0001-7032-7779), Cantarini L, and Conti G

Abstract

Objective: The present manuscript aims to describe an international, electronic-based, user-friendly and interoperable patient registry for monogenic autoinflammatory diseases (mAIDs), developed in the contest of the Autoinflammatory Diseases Alliance (AIDA) Network. Methods: This is an electronic platform, based on the Research Electronic Data Capture (REDCap) tool, used for real-world data collection of demographics, clinical, laboratory, instrumental and socioeconomic data of mAIDs patients. The instrument has flexibility, may change over time based on new scientific acquisitions, and communicate potentially with other similar registries; security, data quality and data governance are corner stones of the platform. Results: AIDA project will share knowledge and expertise on mAIDs. Since its start, 118 centers from 24 countries and 4 continents have joined the AIDA project. Fifty-nine centers have already obtained the approval from their local Ethics Committees. Currently, the platform counts 337 users (122 Principal Investigators, 210 Site Investigators, 2 Lead Investigators, and 3 data managers). The Registry collects baseline and follow-up data using 3,748 fields organized into 21 instruments, which include demographics, patient history, symptoms, trigger/risk factors, therapies, and healthcare information for mAIDs patients. Conclusions: The AIDA mAIDs Registry, acts both as a research tool for future collaborative real-life studies on mAIDs and as a service to connect all the figures called to participate. On this basis, the registry is expected to play a pivotal role in generating new scientific evidence on this group of rare diseases, substantially improving the management of patients, and optimizing the impact on the healthcare system. NCT 05200715 available at https://clinicaltrials.gov

Published
2022

7. Diagnosis and management of urinary tract infections in children aged 2 months to 3 years in the Italian emergency units: the ItaUTI study

Author

Cenzato, F, Milani, G, Amigoni, A, Sperotto, F, Bianchetti, M, Agostoni, C, Montini, G, Farello, G, Chiarelli, F, Greco, R, Di Lollo, F, Rocco Forte, F, Manieri, S, Carpino, L, Caloiero, M, Cirisano, A, Bragho, S, Della Casa, R, Nunziata, F, Pecoraro, C, Pacifico, R, Lanari, M, Ghizzi, C, Serra, L, Stella, M, Maggiore, G, Fiorini, R, Dodi, I, Morelli, A, Lughetti, L, Cella, A, Vergine, G, De Fanti, A, Dragovic, D, Santori, D, Cozzi, G, Cogo, P, Raponi, M, Lubrano, R, de Martinis, M, Gatto, A, Barbieri, M, Reale, A, Bracaglia, G, Piccotti, E, Borea, R, Gaiero, A, Martelli, L, Arrighini, A, Cianci, P, Cavalli, C, De Santis, L, Pietra, B, Biondi, A, Sala, M, Pogliani, L, Cherubini, S, Bellini, M, Bruni, P, Traina, G, Tommasi, P, Del Barba, P, Arrigoni, S, Salvini, F, Bernardo, L, Bertolozzi, G, Fasoli, S, Marseglia, G, Palumbo, E, Bosco, A, Mirri, G, Fabiani, E, Ruffini, E, Pieragostini, L, Fornaro, M, Ripanti, G, Pannoni, D, Enrico, F, Perona, A, Tappi, E, Nis Haitink, O, Rabbone, I, Capalbo, P, Urbino, A, Guala, A, Cosi, G, Barracchia, M, Martire, B, Cardinale, F, Moramarco, F, Perrone, C, Campanozzi, A, Cecinati, V, Canetto, A, Clemente, C, Cualbu, A, Narducci, F, Mula, G, Bulciolu, P, Antonucci, R, Gramaglia, G, Cavaleri, G, Salpietro, C, Corsello, G, Salvo, R, Palmeri, M, Vitale, M, Morgano, A, Falorni, S, Peroni, D, Masi, S, Bertini, A, Vaccaro, A, Vasarri, P, Reinstadler, P, Soffiati, M, Stefanelli, M, Verrotti di Pianella, A, Bertone, C, Marzini, S, Da Dalt, L, Rugolotto, S, Scozzola, F, Ecclesio Livio, L, Cinquetti, M, Silvagni, D, Bellettato, M, Cenzato F., Milani G. P., Amigoni A., Sperotto F., Bianchetti M. G., Agostoni C., Montini G., Farello G., Chiarelli F., Greco R., Di Lollo F., Rocco Forte F., Manieri S., Carpino L., Caloiero M., Cirisano A., Bragho S., Della Casa R., Nunziata F., Pecoraro C., Pacifico R., Lanari M., Ghizzi C., Serra L., Stella M., Maggiore G., Fiorini R., Dodi I., Morelli A., Lughetti L., Cella A., Vergine G., De Fanti A., Dragovic D., Santori D., Cozzi G., Cogo P., Raponi M., Lubrano R., de Martinis M., Gatto A., Barbieri M. A., Reale A., Bracaglia G., Piccotti E., Borea R., Gaiero A., Martelli L., Arrighini A., Cianci P., Cavalli C., De Santis L., Pietra B. C., Biondi A., Sala M., Pogliani L. M., Cherubini S., Bellini M., Bruni P., Traina G., Tommasi P., Del Barba P., Arrigoni S., Salvini F. M., Bernardo L., Bertolozzi G., Fasoli S., Marseglia G. L., Palumbo E., Bosco A., Mirri G., Fabiani E., Ruffini E., Pieragostini L., Fornaro M., Ripanti G., Pannoni D., Enrico F., Perona A., Tappi E., Nis Haitink O., Rabbone I., Capalbo P. T., Urbino A., Guala A., Cosi G., Barracchia M. G., Martire B., Cardinale F., Moramarco F., Perrone C., Campanozzi A., Cecinati V., Canetto A., Clemente C., Cualbu A., Narducci F., Mula G., Bulciolu P., Antonucci R., Gramaglia G., Cavaleri G., Salpietro C., Corsello G., Salvo R., Palmeri M., Vitale M. A., Morgano A., Falorni S., Peroni D., Masi S., Bertini A., Vaccaro A., Vasarri P., Reinstadler P., Soffiati M., Stefanelli M., Verrotti di Pianella A., Bertone C., Marzini S., Da Dalt L., Rugolotto S., Scozzola F., Ecclesio Livio L., Cinquetti M., Silvagni D., Bellettato M., Cenzato, F, Milani, G, Amigoni, A, Sperotto, F, Bianchetti, M, Agostoni, C, Montini, G, Farello, G, Chiarelli, F, Greco, R, Di Lollo, F, Rocco Forte, F, Manieri, S, Carpino, L, Caloiero, M, Cirisano, A, Bragho, S, Della Casa, R, Nunziata, F, Pecoraro, C, Pacifico, R, Lanari, M, Ghizzi, C, Serra, L, Stella, M, Maggiore, G, Fiorini, R, Dodi, I, Morelli, A, Lughetti, L, Cella, A, Vergine, G, De Fanti, A, Dragovic, D, Santori, D, Cozzi, G, Cogo, P, Raponi, M, Lubrano, R, de Martinis, M, Gatto, A, Barbieri, M, Reale, A, Bracaglia, G, Piccotti, E, Borea, R, Gaiero, A, Martelli, L, Arrighini, A, Cianci, P, Cavalli, C, De Santis, L, Pietra, B, Biondi, A, Sala, M, Pogliani, L, Cherubini, S, Bellini, M, Bruni, P, Traina, G, Tommasi, P, Del Barba, P, Arrigoni, S, Salvini, F, Bernardo, L, Bertolozzi, G, Fasoli, S, Marseglia, G, Palumbo, E, Bosco, A, Mirri, G, Fabiani, E, Ruffini, E, Pieragostini, L, Fornaro, M, Ripanti, G, Pannoni, D, Enrico, F, Perona, A, Tappi, E, Nis Haitink, O, Rabbone, I, Capalbo, P, Urbino, A, Guala, A, Cosi, G, Barracchia, M, Martire, B, Cardinale, F, Moramarco, F, Perrone, C, Campanozzi, A, Cecinati, V, Canetto, A, Clemente, C, Cualbu, A, Narducci, F, Mula, G, Bulciolu, P, Antonucci, R, Gramaglia, G, Cavaleri, G, Salpietro, C, Corsello, G, Salvo, R, Palmeri, M, Vitale, M, Morgano, A, Falorni, S, Peroni, D, Masi, S, Bertini, A, Vaccaro, A, Vasarri, P, Reinstadler, P, Soffiati, M, Stefanelli, M, Verrotti di Pianella, A, Bertone, C, Marzini, S, Da Dalt, L, Rugolotto, S, Scozzola, F, Ecclesio Livio, L, Cinquetti, M, Silvagni, D, Bellettato, M, Cenzato F., Milani G. P., Amigoni A., Sperotto F., Bianchetti M. G., Agostoni C., Montini G., Farello G., Chiarelli F., Greco R., Di Lollo F., Rocco Forte F., Manieri S., Carpino L., Caloiero M., Cirisano A., Bragho S., Della Casa R., Nunziata F., Pecoraro C., Pacifico R., Lanari M., Ghizzi C., Serra L., Stella M., Maggiore G., Fiorini R., Dodi I., Morelli A., Lughetti L., Cella A., Vergine G., De Fanti A., Dragovic D., Santori D., Cozzi G., Cogo P., Raponi M., Lubrano R., de Martinis M., Gatto A., Barbieri M. A., Reale A., Bracaglia G., Piccotti E., Borea R., Gaiero A., Martelli L., Arrighini A., Cianci P., Cavalli C., De Santis L., Pietra B. C., Biondi A., Sala M., Pogliani L. M., Cherubini S., Bellini M., Bruni P., Traina G., Tommasi P., Del Barba P., Arrigoni S., Salvini F. M., Bernardo L., Bertolozzi G., Fasoli S., Marseglia G. L., Palumbo E., Bosco A., Mirri G., Fabiani E., Ruffini E., Pieragostini L., Fornaro M., Ripanti G., Pannoni D., Enrico F., Perona A., Tappi E., Nis Haitink O., Rabbone I., Capalbo P. T., Urbino A., Guala A., Cosi G., Barracchia M. G., Martire B., Cardinale F., Moramarco F., Perrone C., Campanozzi A., Cecinati V., Canetto A., Clemente C., Cualbu A., Narducci F., Mula G., Bulciolu P., Antonucci R., Gramaglia G., Cavaleri G., Salpietro C., Corsello G., Salvo R., Palmeri M., Vitale M. A., Morgano A., Falorni S., Peroni D., Masi S., Bertini A., Vaccaro A., Vasarri P., Reinstadler P., Soffiati M., Stefanelli M., Verrotti di Pianella A., Bertone C., Marzini S., Da Dalt L., Rugolotto S., Scozzola F., Ecclesio Livio L., Cinquetti M., Silvagni D., and Bellettato M.

Abstract

Urinary tract infections (UTIs) are among the most frequent bacterial diseases in infants and children. Physician adherence to recommendations is notoriously often poor, but no data are available on UTIs management in the emergency setting. In this multicenter national study, we investigated the policies regarding UTIs management in children aged 2 months to 3 years in Italian emergency units. Between April and June 2021, directors of the emergency units were invited to answer an online survey on the following items: diagnostic approach to children with fever without an apparent source, therapeutic approach to UTIs, the use of kidney and urinary tract ultrasound, and the criteria for hospitalization. A total of 121 (89%) out of 139 of invited units participated in the study. Overall, units manage children with a suspected or confirmed UTI according to available recommendations for most of the items. However, in almost 80% (n = 94) of units, a sterile perineal bag is used to collect urine for culture. When urine is collected by cathether, heterogeneity exists on the threshold of bacterial load considered for UTI diagnosis. Conclusions: Available recommendations on UTIs in children are followed by Italian emergency units for most of the items. However, the methods to collect urine specimens for culture, one of the crucial steps of the diagnostic work-up, often do not align with current recommendations and CFU thresholds considered for diagnosis largely vary among centers. Efforts should be addressed to validate and implement new child and family friendly urine collection techniques.What is Known:• Several guidelines are published on the management of children with suspected or confirmed urinary tract infection.• No data are available on the management of pediatric urinary tract infections in the emergency setting.What is New:• Almost 80% of the Italian emergency units employ a sterile perineal bag to collect urine for culture.• Diagnostic

Published
2022

8. AB0431 CANAKINUMAB AS FIRST-LINE BIOLOGICAL THERAPY IN STILL’S DISEASE AND DIFFERENCES BETWEEN THE SYSTEMIC AND THE CHRONIC-ARTICULAR COURSES: REAL-LIFE EXPERIENCE FROM THE INTERNATIONAL AIDA REGISTRY

Author

Vitale, A., primary, Caggiano, V., additional, Maggio, M. C., additional, Lopalco, G., additional, Emmi, G., additional, Sota, J., additional, La Torre, F., additional, Ruscitti, P., additional, Bartoloni, E., additional, Conti, G., additional, Fabiani, C., additional, Mattioli, I., additional, Gaggiano, C., additional, Cardinale, F., additional, Dagna, L., additional, Campochiaro, C., additional, Giacomelli, R., additional, Balistreri, A., additional, Laskari, K., additional, Tufan, A., additional, Ragab, G., additional, Almaghlouth, I., additional, Więsik-Szewczyk, E., additional, Pereira, R. M., additional, Frediani, B., additional, Iannone, F., additional, Sfikakis, P., additional, and Cantarini, L., additional

Published
2023
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9. Musculoskeletal manifestations in children with Behçet's syndrome: data from the AIDA Network Behçet's Syndrome Registry

Author

Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Gaggiano, C, Maselli, A, Sfikakis, Pp, Laskari, K, Ragab, G, Hegazy, Mt, Laymouna, Ah, Lopalco, G, Almaghlouth, Ia, Asfina, Kn, Alahmed, O, Giardini Mayrink, Ha, Parente de Brito Antonelli, I, Cattalini, M, Piga, M, Sota, J, Gentileschi, S, Maggio, Mc, Opris-Belinski, D, Hatemi, G, Insalaco, A, Olivieri, An, Tufan, A, Karadeniz, H, Kardaş, Rc, La Torre, F, Cardinale, F, Marino, A, Guerriero, S, Ruscitti, P, Tarsia, M, Vitale, A, Caggiano, V, Telesca, S, Iannone, F, Parretti, V, Frassi, M, Aragona, E, Ciccia, F, Wiesik-Szewczyk, E, Ionescu, R, Şahin, A, Akkoç, N, Hinojosa-Azaola, A, Tharwat, S, Hernández-Rodríguez, J, Espinosa, G, Conti, G, Del Giudice, E, Govoni, M, Emmi, G, Fabiani, C, Balistreri, A, Frediani, B, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

This study aims to describe musculoskeletal manifestations (MSM) in children with Behçet’s syndrome (BS), their association with other disease manifestations, response to therapy, and long-term prognosis. Data were retrieved from the AIDA Network Behçet’s Syndrome Registry. Out of a total of 141 patients with juvenile BS, 37 had MSM at disease onset (26.2%). The median age at onset was 10.0 years (IQR 7.7). The median follow-up duration was 21.8 years (IQR 23.3). Recurrent oral (100%) and genital ulcers (67.6%) and pseudofolliculitis (56.8%) were the most common symptoms associated with MSM. At disease onset, 31 subjects had arthritis (83.8%), 33 arthralgia (89.2%), and 14 myalgia (37.8%). Arthritis was monoarticular in 9/31 cases (29%), oligoarticular in 10 (32.3%), polyarticular in 5 (16.1%), axial in 7 (22.6%). Over time, arthritis became chronic-recurrent in 67.7% of cases and 7/31 patients had joint erosions (22.6%). The median Behçet's Syndrome Overall Damage Index was 0 (range 0–4). Colchicine was inefcacious for MSM in 4/14 cases (28.6%), independently from the type of MSM (p=0.46) or the concomitant therapy (p=0.30 for cDMARDs, p=1.00 for glucocorticoids); cDMARDs and bDMARDs were inefcacious for MSM in 6/19 (31.4%) and 5/12 (41.7%) cases. The presence of myalgia was associated with bDMARDs inefcacy (p=0.014). To conclude, MSM in children with BS are frequently associated with recurrent ulcers and pseudofolliculitis. Arthritis is mostly mono- or oligoarticular, but sacroiliitis is not unusual. Prognosis of this subset of BS is overall favorable, though the presence of myalgia negatively afects response to biologic therapies.

Published
2023

10. A method for the assessment of time-varying brain shift during navigated epilepsy surgery

Author

De Momi, E., Ferrigno, G., Bosoni, G., Bassanini, P., Blasi, P., Casaceli, G., Fuschillo, D., Castana, L., Cossu, M., Lo Russo, G., and Cardinale, F.

Abstract

Image guidance is widely used in neurosurgery. Tracking systems (neuronavigators) allow registering the preoperative image space to the surgical space. The localization accuracy is influenced by technical and clinical factors, such as brain shift. This paper aims at providing quantitative measure of the time-varying brain shift during open epilepsy surgery, and at measuring the pattern of brain deformation with respect to three potentially meaningful parameters: craniotomy area, craniotomy orientation and gravity vector direction in the images reference frame. We integrated an image-guided surgery system with 3D Slicer, an open-source package freely available in the Internet. We identified the preoperative position of several cortical features in the image space of 12 patients, inspecting both the multiplanar and the 3D reconstructions. We subsequently repeatedly tracked their position in the surgical space. Therefore, we measured the cortical shift, following its time-related changes and estimating its correlation with gravity and craniotomy normal directions. The mean of the median brain shift amount is 9.64 mm ($$\hbox {SD}=4.34$$ SD=4.34 mm). The brain shift amount resulted not correlated with respect to the gravity direction, the craniotomy normal, the angle between the gravity and the craniotomy normal and the craniotomy area. Our method, which relies on cortex surface 3D measurements, gave results, which are consistent with literature. Our measurements are useful for the neurosurgeon, since they provide a continuous monitoring of the intra-operative sinking or bulking of the brain, giving an estimate of the preoperative images validity versus time.

Published
2024
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13. Inventory of collections multiplied, made available to partners - Deliverable 2.2

Author

Salazar-Licea, LC, Mendiondo, G, Santos, CS, Nunes da Silva, M, Lemos, T, Schubert, A, Cardinale, F, George, T, Ntatsi, G, Krenz, L, Pleissner, D, Vasconcelos, A, Fonseca, A, Migliorini, P, Bassignana, C, Agost-Andreu, P, Navarro-Miró, D, Boile, M, Boyanova, S, Bortolazzo, E, Rubiales, D, Iannetta, P, Mayes, S, and Vasconcelos, MW

Abstract

D2.2 - Inventory of collections multiplied, made available to partners

Published
2022
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15. Biologic drugs in chronic spontaneous urticaria

Author

Licari A., Manti S., Leonardi S., Minasi D., Caffarelli C., Cardinale F., Miraglia Del Giudice M., Calvani M., Ciprandi G., Marseglia G. L., Licari, A., Manti, S., Leonardi, S., Minasi, D., Caffarelli, C., Cardinale, F., Miraglia Del Giudice, M., Calvani, M., Ciprandi, G., and Marseglia, G. L.

Subjects
Adult, Male, Biological Products, Urticaria, Chronic Disease, Quality of Life, Humans, Chronic Urticaria, Female, Child
Abstract

Chronic spontaneous urticaria (CSU) is a condition defined by the presence of recurrent urticaria, angioedema, or both, which persist for more than six weeks in duration and occurs in the absence of an identifiable trigger. Both children and adults can develop CSU, although it is more common in adults and in women than in men, with a peak occurrence in the third to fifth decades of life. It imposes a significant burden on patients, families and healthcare systems. The goal of therapy in patients with CSU is to achieve a level of symptom control and improvement in quality of life that is acceptable to the patient, while minimizing therapy-related side effects. The recent introduction of biologic drugs has changed the management of the disease. This work aims to provide a narrative review of the current state of biological therapy and the promising drugs under development for CSU.

Published
2021

16. Digital health in the management of allergic diseases

Author

Pattini S., Pingitore G., Cardinale F., Licari A., Miraglia Del Giudice M., Calvani M., Chiappini E., Cravidi C., Marseglia G. L., Ricci G., Duse M., Tripodi S., Pattini, S., Pingitore, G., Cardinale, F., Licari, A., Miraglia Del Giudice, M., Calvani, M., Chiappini, E., Cravidi, C., Marseglia, G. L., Ricci, G., Duse, M., and Tripodi, S.

Subjects
Immune-allergic patient, Pandemic, SARS-CoV-2, Hypersensitivity, COVID-19, Humans, App, Pandemics, Telemedicine, Human
Abstract

In recent years there has been an important implementation in the medical field of both Mobile Health, such as the use of mobile communication devices, and of other telemedicine tools in general, with the aim of supporting the supervision of diseases from the moment of the first diagnosis to the therapeutic follow-up. In fact, Digital Health can also have a very positive impact on the management of allergic patients, who are known to have the greatest need for regular monitoring, simplifying contact between doctor and patient, but there is still a need to improve implementation regulations, define certification programs and adequate reimbursement systems, as well as to guarantee a high level of attention to the protection of sensitive data. The hope is that one positive outcome of the Covid-19 pandemic will be an acceleration, by all stake-holders involved, of the process of the modernization of health care. (www.actabiomedica.it).

Published
2021

17. Hypersensitivity to polyethylene glycol in adults and children: An emerging challenge

Author

Bianchi A., Bottau P., Calamelli E., Caimmi S., Crisafulli G., Franceschini F., Liotti L., Mori F., Paglialunga C., Saretta F., Tosca M., Cardinale F., Licari A., Miraglia Del Giudice M., Caffarelli C., Bianchi, A., Bottau, P., Calamelli, E., Caimmi, S., Crisafulli, G., Franceschini, F., Liotti, L., Mori, F., Paglialunga, C., Saretta, F., Tosca, M., Cardinale, F., Licari, A., Miraglia Del Giudice, M., and Caffarelli, C.

Subjects
Adult, COVID-19 Vaccines, SARS-CoV-2, COVID-19 Vaccine, technology, industry, and agriculture, COVID-19, macromolecular substances, PEG allergy, Drug allergy, Polyethylene Glycol, Polyethylene Glycols, Polyethylene glycol hypersensitivity, Vaccine allergy, Anaphylaxi, Humans, Child, Anaphylaxis, Human
Abstract

Hypersensitivity reactions to polyethylene glycol (PEG) is an emerging challenge and the interest about this disease is growing since PEG is considered one of the possible causes of coronavirus disease 2019 (COVID 19) vaccine-associated anaphylaxis. PEG is used in a wide variety of pharmaceutical, medical, in-dustrial, cosmetic, and food products and can be an active ingredient or used as an excipient. PEG is present in several medications, and it may or may not be present in different formulations and dosages of the same drug. Lack of standardization nomenclature, inadequate labelling of products and lack of knowledge about PEG involvement in hypersensitivity reactions expose patients at risk of presenting multiple reactions before a diagnosis could be made. In this review we describe the main cases published in literature and propose an allergy work-up and management. (www.actabiomedica.it).

Published
2021

18. Update on Food protein-induced enterocolitis syndrome (FPIES)

Author

Calvani M., Anania C., Bianchi A., D'Auria E., Cardinale F., Votto M., Martelli A., Tosca M., Chiappini E., Brambilla I., Miraglia Del Giudice M., Caffarelli C., Calvani, M., Anania, C., Bianchi, A., D'Auria, E., Cardinale, F., Votto, M., Martelli, A., Tosca, M., Chiappini, E., Brambilla, I., Miraglia Del Giudice, M., and Caffarelli, C.

Subjects
Enterocolitis, Humans, Infant, Syndrome, Allergens, Biomarkers, Food Hypersensitivity
Abstract

Food protein-induced enterocolitis syndrome (FPIES) is a non-IgE mediated food allergy (FA) characterized by delayed and severe gastrointestinal symptoms that typically occurs within the first year of life. Many aspects of this pathology are currently unclear. FPIES is classified as a non-IgE immune-mediated FA in which the immune response is thought to act mainly through cell-mediated mechanisms. In patients with FPIES, the symptom pattern is determined by the frequency and dose of food allergen in the diet. Diagnosis of FPIES may be difficult, mainly due to the lack of specific biomarkers to confirm or exclude the diagnosis. FPIES is a clinical diagnosis, mainly based on clinical features which, although not specific, are reproducible every time the patient takes the food. Different diagnostic criteria of FPIES were published over time in the literature. The present narrative review aims to analyze the current clinical evidence in epidemiology, pathophysiology, diagnosis, and management of this condition.

Published
2021

19. 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

20. Prevalence of Immunological Defects in a Cohort of 97 Rubinstein–Taybi Syndrome Patients

Author

Saettini, F, Herriot, R, Prada, E, Nizon, M, Zama, D, Marzollo, A, Romaniouk, I, Lougaris, V, Cortesi, M, Morreale, A, Kosaki, R, Cardinale, F, Ricci, S, Dominguez-Garrido, E, Montin, D, Vincent, M, Milani, D, Biondi, A, Gervasini, C, Badolato, R, Saettini F., Herriot R., Prada E., Nizon M., Zama D., Marzollo A., Romaniouk I., Lougaris V., Cortesi M., Morreale A., Kosaki R., Cardinale F., Ricci S., Dominguez-Garrido E., Montin D., Vincent M., Milani D., Biondi A., Gervasini C., Badolato R., Saettini, F, Herriot, R, Prada, E, Nizon, M, Zama, D, Marzollo, A, Romaniouk, I, Lougaris, V, Cortesi, M, Morreale, A, Kosaki, R, Cardinale, F, Ricci, S, Dominguez-Garrido, E, Montin, D, Vincent, M, Milani, D, Biondi, A, Gervasini, C, Badolato, R, Saettini F., Herriot R., Prada E., Nizon M., Zama D., Marzollo A., Romaniouk I., Lougaris V., Cortesi M., Morreale A., Kosaki R., Cardinale F., Ricci S., Dominguez-Garrido E., Montin D., Vincent M., Milani D., Biondi A., Gervasini C., and Badolato R.

Abstract

Although recurrent infections in Rubinstein–Taybi syndrome (RSTS) are common, and probably multifactorial, immunological abnormalities have not been extensively described with only isolated cases or small case series of immune deficiency and dysregulation having been reported. The objective of this study was to investigate primary immunodeficiency (PID) and immune dysregulation in an international cohort of patients with RSTS. All published cases of RSTS were identified. The corresponding authors and researchers involved in the diagnosis of inborn errors of immunity or genetic syndromes were contacted to obtain up-to-date clinical and immunological information. Ninety-seven RSTS patients were identified. For 45 patients, we retrieved data from the published reports while for 52 patients, a clinical update was provided. Recurrent or severe infections, autoimmune/autoinflammatory complications, and lymphoproliferation were observed in 72.1%, 12.3%, and 8.2% of patients. Syndromic immunodeficiency was diagnosed in 46.4% of individuals. Despite the broad heterogeneity of immunodeficiency disorders, antibody defects were observed in 11.3% of subjects. In particular, these patients presented hypogammaglobulinemia associated with low B cell counts and reduction of switched memory B cell numbers. Immunoglobulin replacement therapy, antibiotic prophylaxis, and immunosuppressive treatment were employed in 16.4%, 8.2%, and 9.8% of patients, respectively. Manifestations of immune dysfunctions, affecting mostly B cells, are more common than previously recognized in patients with RSTS. Full immunological assessment is warranted in these patients, who may require detailed investigation and specific supportive treatment. [Figure not available: see fulltext.]

Published
2020

21. Valorising Stress-Resilient Tomato Landraces

Author

Cardinale, F, Visentin, I, and Schubert, A

Subjects
stress-resilience, drought stress, dynamic value chain, biodiversity
Abstract

Practice Abstract -The potential of tomato landraces to increase farming sustainability.

Published
2022
Full Text
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22. Development and implementation of the AIDA International Registry for patients with undifferentiated systemic autoinflammatory diseases

Author

Della Casa, F, Vitale, A, Lopalco, G, Ruscitti, P, Ciccia, F, Emmi, G, Cattalini, M, Wiesik-Szewczyk, E, Maggio, Mc, Ogunjimi, B, Sfikakis, Pp, Tufan, A, Al-Mayouf, Sm, Del Giudice, E, Aragona, E, La Torre, F, Sota, J, Colella, S, Di Cola, I, Iacono, D, Mattioli, I, Jahnz-Rózyk, K, Joos, R, Laskari, K, Gaggiano, C, Abbruzzese, A, Cipriani, P, Rozza, G, Alsaleem, A, Yildirim, D, Tarsia, M, Ragab, G, Ricci, F, Cardinale, F, Korzeniowska, M, Frassi, M, Caggiano, V, Saad, Ma, Pereira, Rm, Berlengiero, V, Gentileschi, S, Guerriero, S, Giani, T, Gelardi, V, Iannone, F, Giardini, Ham, Almaghlouth, Ia, Kardas, Rc, Ait-Idir, D, Frediani, B, Balistreri, A, Fabiani, C, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Della Casa, F, Vitale, A, Lopalco, G, Ruscitti, P, Ciccia, F, Emmi, G, Cattalini, M, Wiesik-Szewczyk, E, Maggio, Mc, Ogunjimi, B, Sfikakis, Pp, Tufan, A, Al-Mayouf, Sm, Del Giudice, E, Aragona, E, La Torre, F, Sota, J, Colella, S, Di Cola, I, Iacono, D, Mattioli, I, Jahnz-Rózyk, K, Joos, R, Laskari, K, Gaggiano, C, Abbruzzese, A, Cipriani, P, Rozza, G, Alsaleem, A, Yildirim, D, Tarsia, M, Ragab, G, Ricci, F, Cardinale, F, Korzeniowska, M, Frassi, M, Caggiano, V, Saad, Ma, Pereira, Rm, Berlengiero, V, Gentileschi, S, Guerriero, S, Giani, T, Gelardi, V, Iannone, F, Giardini, Ham, Almaghlouth, Ia, Kardas, Rc, Ait-Idir, D, Frediani, B, Balistreri, A, Fabiani, C, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: This paper points out the design, development and deployment of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to pediatric and adult patients affected with Undifferentiated Systemic AutoInflammatory Diseases (USAIDs). Methods: This is a physician-driven, population- and electronic-based registry employed for real-world data collection about demographics, clinical, laboratory, instrumental and socioeconomic data of USAIDs patients. Data recruitment, based on the Research Electronic Data Capture (REDCap) tool, is thought to gain standardised information for real-life research and has been developed to change over time according to the scientific acquisitions and potentially communicate with other similar instruments; this platform ensures security, data quality and data governance. Results: The focus of the AIDA project is connecting physicians and researchers from all over the world to shed a new light on heterogeneous rare diseases. Since its birth, 99 centres from 20 countries and 4 continents have joined the AIDA project. Forty-eight centres have already obtained the approval from their local Ethics Committees. Currently, the platform counts 265 users (99 Principal Investigators, 162 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry is collecting baseline and follow-up data using 3357 fields organised into 23 instruments, which include demographics, history, symptoms, trigger/risk factors, therapies, and healthcare access for USAIDs patients. Conclusions: The development of the AIDA International Registry for USAIDs patients will facilitate the on-line collection of standardised data, connecting a worldwide group of researchers: the Registry constitutes an international multicentre observational groundwork aimed at increasing the study cohort of patients with USAIDs in order to improve our knowledge of this peculiar cluster of autoinflammatory diseases.

Published
2022

23. Development and implementation of the AIDA International Registry for patients with VEXAS syndrome

Author

Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Caggiano, V, Della Casa, F, Hernández-Rodríguez, J, Frassi, M, Monti, S, Tufan, A, Telesca, S, Conticini, E, Ragab, G, Lopalco, G, Almaghlouth, I, Pereira, Rmr, Yildirim, D, Cattalini, M, Marino, A, Giani, T, La Torre, F, Ruscitti, P, Aragona, E, Wiesik-Szewczyk, E, Del Giudice, E, Sfikakis, Pp, Govoni, M, Emmi, G, Maggio, Mc, Giacomelli, R, Ciccia, F, Conti, Giorgio, Ait-Idir, D, Lomater, C, Sabato, V, Piga, M, Sahin, A, Opris-Belinski, D, Ionescu, R, Bartoloni, E, Franceschini, F, Parronchi, P, de Paulis, A, Espinosa, G, Maier, A, Sebastiani, Gd, Insalaco, A, Shahram, F, Sfriso, P, Minoia, F, Alessio, M, Makowska, J, Hatemi, G, Akkoç, N, Li Gobbi, F, Gidaro, A, Olivieri, An, Al-Mayouf, Sm, Erten, S, Gentileschi, S, Vasi, I, Tarsia, M, Mahmoud, Aaa, Frediani, B, Fares Alzahrani, M, Laymouna, Ah, Ricci, F, Cardinale, F, Jahnz-Rózyk, K, Tosi, Gm, Crisafulli, F, Balistreri, A, Dagostin, Ma, Ghanema, M, Gaggiano, C, Sota, J, Di Cola, I, Fabiani, C, Giardini, Ham, Renieri, A, Fabbiani, A, Carrer, A, Bocchia, M, Caroni, F, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: The aim of this paper is to present the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic (VEXAS) syndrome, describing its design, construction, and modalities of dissemination. Methods: The present registry is a clinical, physician-driven, population- and electronic-based instrument designed for the retrospective and prospective collection of real-life data. Data gathering is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain real-world evidence for daily patients’ management; the registry may potentially communicate with other on-line tools dedicated to VEXAS syndrome, enhancing international collaboration and data sharing for research purposes. The registry is handy enough to be easily modified to meet future needs regarding VEXAS syndrome. Results: To date (April 2022), 105 Centers from 23 Countries in 4 continents have been involved; 287 users (106 Principal Investigators, 177 Site Investigators, 2 Lead Investigators, and 2 data managers) may already enter the registry for data collection and sharing. The registry includes 4950 fields organised into 18 instruments designed to fully describe patient’s details about demographics, clinical manifestations, symptoms, histologic details about skin and bone marrow biopsies and aspirate, laboratory features, complications, comorbidities, therapies, and healthcare access. Conclusions: This international Registry for patients with VEXAS syndrome will allow the achievement of a comprehensive knowledge about this new disease in a relatively short time with the final goal to obtain real-world evidence data for daily clinical practice. This project can be found on https://clinicaltrials.gov NCT 05200715

Published
2022

24. Efficacy and safety of tocilizumab in adult-onset Still's disease: Real-life experience from the international AIDA registry

Author

Sota, J, Vitale, A, Lopalco, G, Pereira, Rmr, Giordano, Hf, Antonelli, Ipb, Makowska, J, Brzezińska, O, Lewandowska-Polak, A, Ruscitti, P, Cipriani, P, Cola, Id, Govoni, M, Ruffili, F, Sfikakis, Pp, Laskari, K, Ragab, G, Hussein, Ma, Gentileschi, S, Gaggiano, C, La Torre, F, Maier, A, Emmi, G, Marino, A, Ciccia, F, Sfriso, P, Maggio, Mc, Bartoloni, E, Lomater, C, Hegazy, Mt, Tektonidou, M, Dagostin, Ma, Opinc, A, Sebastiani, Gd, Giacomelli, R, Giudice, Ed, Olivieri, An, Tufan, A, Kardas, Rk, Nuzzolese, R, Cardinale, F, Więsik-Szewczyk, E, Veronica, P, Tarsia, M, Iannone, F, Della Casa, F, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, Rigante D (ORCID:0000-0001-7032-7779), Sota, J, Vitale, A, Lopalco, G, Pereira, Rmr, Giordano, Hf, Antonelli, Ipb, Makowska, J, Brzezińska, O, Lewandowska-Polak, A, Ruscitti, P, Cipriani, P, Cola, Id, Govoni, M, Ruffili, F, Sfikakis, Pp, Laskari, K, Ragab, G, Hussein, Ma, Gentileschi, S, Gaggiano, C, La Torre, F, Maier, A, Emmi, G, Marino, A, Ciccia, F, Sfriso, P, Maggio, Mc, Bartoloni, E, Lomater, C, Hegazy, Mt, Tektonidou, M, Dagostin, Ma, Opinc, A, Sebastiani, Gd, Giacomelli, R, Giudice, Ed, Olivieri, An, Tufan, A, Kardas, Rk, Nuzzolese, R, Cardinale, F, Więsik-Szewczyk, E, Veronica, P, Tarsia, M, Iannone, F, Della Casa, F, Fabiani, C, Frediani, B, Balistreri, A, Rigante, Donato, Cantarini, L, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Background/objectives: Long-term efficacy and safety of tocilizumab (TCZ) in adult-onset Still’s disease (AOSD) mostly derive from small case series. Herein we report a registry-based study investigating TCZ efficacy and safety in a cohort of patients with AOSD evaluated by clinical and serum inflammatory markers as well as drug retention rate analysis. Methods: This is an international multicentre study analyzing data from patients with AOSD regularly enrolled in the AIDA registry. TCZ efficacy was evaluated between baseline and last follow-up assessment in terms of changes in the Pouchot score and laboratory findings. Drug-retention rate was estimated by the Kaplan-Meier method, while Cox-regression analysis was employed to detect potential predictive factors of treatment withdrawal. Results: Data from 31 patients (15 men, 16 women) refractory to the conventional therapies and treated with TCZ were extracted from the AIDA registry. Mean ± SD time of treatment duration with TCZ was 24.32 ± 20.57 months. Pouchot score significantly decreased from baseline 2.00 (4.00) to the last follow-up assessment 00.00 (00.00), p=0.003). Similarly, laboratory parameters significantly decreased from baseline to the last follow-up visit. With regard to drug survival, cumulative TCZ retention rate at 12-, 24-, and 36-month follow-up visit were 83.1%, 71.7% and 63.7%, respectively, without significant differences between biologic naïve patients and those previously treated with other biologics (p=0.329). Likewise, no significant differences were observed between chronic articular course of AOSD and other types of disease course (p=0.938) or between patients co-administered with conventional immunosuppressants and patients receiving TCZ as monotherapy (p=0.778). Cox-regression analysis identified no variable associated with a higher hazard of treatment withdrawal. Treatment was discontinued in 9 patients due to long-term remission (n=4), adverse events (n=2), loss of efficacy (n=1)

Published
2022

25. Development and implementation of the AIDA International Registry for patients with Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis syndrome

Author

Della Casa, F, Vitale, A, Cattalini, M, La Torre, F, Capozio, G, Del Giudice, E, Maggio, Mc, Conti, Giorgio, Alessio, M, Ogunjimi, B, Ragab, G, Emmi, G, Aragona, E, Giani, T, Lopalco, G, Parronchi, P, Shahram, F, Verrecchia, Elena, Ricci, F, Cardinale, F, Di Noi, Silvia, Nuzzolese, R, Lubrano, R, Patroniti, S, Naddei, R, Sabato, V, Hussein, Ma, Dotta, L, Mastrorilli, V, Gentileschi, S, Tufan, A, Caggiano, V, Hegazy, Mt, Sota, J, Almaghlouth, Ia, Ibrahim, A, Wiȩsik-Szewczyk, E, Ozkiziltas, B, Grosso, S, Frassi, M, Tarsia, M, Pereira, Rmr, Taymour, M, Gaggiano, C, Colella, S, Fabiani, C, Morrone, M, Ruscitti, P, Frediani, B, Spedicato, V, Giardini, Ham, Balistreri, A, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Verrecchia E, Di Noi S, Rigante D (ORCID:0000-0001-7032-7779), Della Casa, F, Vitale, A, Cattalini, M, La Torre, F, Capozio, G, Del Giudice, E, Maggio, Mc, Conti, Giorgio, Alessio, M, Ogunjimi, B, Ragab, G, Emmi, G, Aragona, E, Giani, T, Lopalco, G, Parronchi, P, Shahram, F, Verrecchia, Elena, Ricci, F, Cardinale, F, Di Noi, Silvia, Nuzzolese, R, Lubrano, R, Patroniti, S, Naddei, R, Sabato, V, Hussein, Ma, Dotta, L, Mastrorilli, V, Gentileschi, S, Tufan, A, Caggiano, V, Hegazy, Mt, Sota, J, Almaghlouth, Ia, Ibrahim, A, Wiȩsik-Szewczyk, E, Ozkiziltas, B, Grosso, S, Frassi, M, Tarsia, M, Pereira, Rmr, Taymour, M, Gaggiano, C, Colella, S, Fabiani, C, Morrone, M, Ruscitti, P, Frediani, B, Spedicato, V, Giardini, Ham, Balistreri, A, Rigante, Donato, Cantarini, L, Conti G (ORCID:0000-0002-8566-9365), Verrecchia E, Di Noi S, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: Aim of this paper is to illustrate the methodology, design, and development of the AutoInflammatory Disease Alliance (AIDA) International Registry dedicated to patients with the Periodic Fever, Aphthous stomatitis, Pharyngitis, and cervical Adenitis (PFAPA) syndrome. Methods: This is a physician-driven, population- and electronic-based registry proposed to gather real-world demographics, clinical, laboratory, instrumental and socioeconomic data from patients suffering from PFAPA syndrome. Data recruitment is realized through the on-line Research Electronic Data Capture (REDCap) tool. This registry is thought to collect standardized information for clinical research leading to solid real-life evidence. The international scope and the flexibility of the registry will facilitate the realization of cutting-edge study projects through the constant updating of variables - as required by future scientific acquisitions - and the possible merging and transfer of data between current and future registries devoted to this disease. Results: One hundred and twelve centers have already been involved from 23 countries and 4 continents starting from August 24th, 2021, to March 21st, 2022. Fifty-five out of 112 have already obtained the formal approval from their local Ethics Committees. At current, the platform counts 287 users (108 principal investigators, 179 site investigators, 2 lead investigators, and 2 data managers). The registry collects retrospective and prospective data using 3845 fields organized into 24 instruments, including PFAPA patient’s demographics, medical histories, symptoms, triggers/risk factors, therapies, and impact on the healthcare systems. Conclusions: The development of the AIDA International Registry for patients with PFAPA syndrome will enable the on-line collection of standardized data prompting real-life studies through the connection of worldwide groups of physicians and researchers. This project can be found on https://clinicaltrials.gov

Published
2022

26. Development and implementation of the AIDA International Registry for patients with Still’s disease

Author

Vitale, A, Della Casa, F, Lopalco, G, Pereira, Rm, Ruscitti, P, Giacomelli, R, Ragab, G, La Torre, F, Bartoloni, E, Del Giudice, E, Lomater, C, Emmi, G, Govoni, M, Maggio, Mc, Maier, A, Makowska, J, Ogunjimi, B, Sfikakis, Pp, Sfriso, P, Gaggiano, C, Iannone, F, Dagostin, Ma, Di Cola, I, Navarini, L, Ahmed Mahmoud, Aa, Cardinale, F, Riccucci, I, Paroli, Mp, Marucco, Em, Mattioli, I, Sota, J, Abbruzzese, A, Antonelli, Ipb, Cipriani, P, Tufan, A, Fabiani, C, Ramadan, Mm, Cattalini, M, Kardas, Rc, Sebastiani, Gd, Giardini, Ham, Hernández-Rodríguez, J, Mastrorilli, V, Więsik-Szewczyk, E, Frassi, M, Caggiano, V, Telesca, S, Giordano, Hf, Guadalupi, E, Balistreri, A, Rigante, Donato, Cantarini, L., Rigante D (ORCID:0000-0001-7032-7779), Vitale, A, Della Casa, F, Lopalco, G, Pereira, Rm, Ruscitti, P, Giacomelli, R, Ragab, G, La Torre, F, Bartoloni, E, Del Giudice, E, Lomater, C, Emmi, G, Govoni, M, Maggio, Mc, Maier, A, Makowska, J, Ogunjimi, B, Sfikakis, Pp, Sfriso, P, Gaggiano, C, Iannone, F, Dagostin, Ma, Di Cola, I, Navarini, L, Ahmed Mahmoud, Aa, Cardinale, F, Riccucci, I, Paroli, Mp, Marucco, Em, Mattioli, I, Sota, J, Abbruzzese, A, Antonelli, Ipb, Cipriani, P, Tufan, A, Fabiani, C, Ramadan, Mm, Cattalini, M, Kardas, Rc, Sebastiani, Gd, Giardini, Ham, Hernández-Rodríguez, J, Mastrorilli, V, Więsik-Szewczyk, E, Frassi, M, Caggiano, V, Telesca, S, Giordano, Hf, Guadalupi, E, Balistreri, A, Rigante, Donato, Cantarini, L., and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Objective: Aim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still’s disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder. Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients’ management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still’s disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions. Results: Starting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient’s demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: This international Registry for patients with Still’s disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represents the ultimate goal of t

Published
2022

29. Inter-society consensus for the use of inhaled corticosteroids in infants, children and adolescents with airway diseases (vol 47, pg 1, 2021)

Author

Duse, M, Santamaria, F, Verga, M, Bergamini, M, Simeone, G, Leonardi, L, Tezza, G, Bianchi, A, Capuano, A, Cardinale, F, Cerimoniale, G, Landi, M, Malventano, M, Tosca, M, Varricchio, A, Zicari, A, Alfaro, C, Barberi, S, Becherucci, P, Bernardini, R, Biasci, P, Caffarelli, C, Caldarelli, V, Capristo, C, Castronuovo, S, Chiappini, E, Cutrera, R, De Castro, G, De Franciscis, L, Decimo, F, Iacono, I, Diaferio, L, Di Cicco, M, Di Mauro, C, Di Mauro, D, Di Mauro, F, Di Mauro, G, Doria, M, Falsaperla, R, Ferraro, V, Fanos, V, Galli, E, Ghiglioni, D, Indinnimeo, L, Kantar, A, Lamborghini, A, Licari, A, Lubrano, R, Luciani, S, Macri, F, Marseglia, G, Martelli, A, Masini, L, Midulla, F, Minasi, D, Miniello, V, Del Giudice, M, Morandini, S, Nardini, G, Nocerino, A, Novembre, E, Pajno, G, Paravati, F, Piacentini, G, Piersantelli, C, Pozzobon, G, Ricci, G, Spanevello, V, Turra, R, Zanconato, S, Borrelli, M, Villani, A, Corsello, G, and Peroni, D

Subjects
Settore MED/38
Published
2022

30. 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

31. Development and Implementation of the AIDA International Registry for Patients With Still's Disease

Author

Vitale, A. Della Casa, F. Lopalco, G. Pereira, R.M. Ruscitti, P. Giacomelli, R. Ragab, G. La Torre, F. Bartoloni, E. Del Giudice, E. Lomater, C. Emmi, G. Govoni, M. Maggio, M.C. Maier, A. Makowska, J. Ogunjimi, B. Sfikakis, P.P. Sfriso, P. Gaggiano, C. Iannone, F. Dagostin, M.A. Di Cola, I. Navarini, L. Ahmed Mahmoud, A.A. Cardinale, F. Riccucci, I. Paroli, M.P. Marucco, E.M. Mattioli, I. Sota, J. Abbruzzese, A. Antonelli, I.P.B. Cipriani, P. Tufan, A. Fabiani, C. Ramadan, M.M. Cattalini, M. Kardas, R.C. Sebastiani, G.D. Giardini, H.A.M. Hernández-Rodríguez, J. Mastrorilli, V. Więsik-Szewczyk, E. Frassi, M. Caggiano, V. Telesca, S. Giordano, H.F. Guadalupi, E. Giani, T. Renieri, A. Colella, S. Cataldi, G. Gentile, M. Fabbiani, A. Al-Maghlouth, I.A. Frediani, B. Balistreri, A. Rigante, D. Cantarini, L. The Autoinflammatory Diseases Alliance (AIDA) Network

Abstract

Objective: Aim of this paper is to present the design, construction, and modalities of dissemination of the AutoInflammatory Disease Alliance (AIDA) International Registry for patients with systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD), which are the pediatric and adult forms of the same autoinflammatory disorder. Methods: This Registry is a clinical, physician-driven, population- and electronic-based instrument implemented for the retrospective and prospective collection of real-world data. The collection of data is based on the Research Electronic Data Capture (REDCap) tool and is intended to obtain evidence drawn from routine patients' management. The collection of standardized data is thought to bring knowledge about real-life clinical research and potentially communicate with other existing and future Registries dedicated to Still's disease. Moreover, it has been conceived to be flexible enough to easily change according to future scientific acquisitions. Results: Starting from June 30th to February 7th, 2022, 110 Centers from 23 Countries in 4 continents have been involved. Fifty-four of these have already obtained the approval from their local Ethics Committees. Currently, the platform counts 290 users (111 Principal Investigators, 175 Site Investigators, 2 Lead Investigators, and 2 data managers). The Registry collects baseline and follow-up data using 4449 fields organized into 14 instruments, including patient's demographics, history, clinical manifestations and symptoms, trigger/risk factors, therapies and healthcare access. Conclusions: This international Registry for patients with Still's disease will allow a robust clinical research through collection of standardized data, international consultation, dissemination of knowledge, and implementation of observational studies based on wide cohorts of patients followed-up for very long periods. Solid evidence drawn from “real-life” data represents the ultimate goal of this Registry, which has been implemented to significantly improve the overall management of patients with Still's disease. NCT 05200715 available at https://clinicaltrials.gov/. Copyright © 2022 Vitale, Della Casa, Lopalco, Pereira, Ruscitti, Giacomelli, Ragab, La Torre, Bartoloni, Del Giudice, Lomater, Emmi, Govoni, Maggio, Maier, Makowska, Ogunjimi, Sfikakis, Sfriso, Gaggiano, Iannone, Dagostin, Di Cola, Navarini, Ahmed Mahmoud, Cardinale, Riccucci, Paroli, Marucco, Mattioli, Sota, Abbruzzese, Antonelli, Cipriani, Tufan, Fabiani, Ramadan, Cattalini, Kardas, Sebastiani, Giardini, Hernández-Rodríguez, Mastrorilli, Więsik-Szewczyk, Frassi, Caggiano, Telesca, Giordano, Guadalupi, Giani, Renieri, Colella, Cataldi, Gentile, Fabbiani, Al-Maghlouth, Frediani, Balistreri, Rigante and Cantarini.

Published
2022

35. Urticaria in childhood

Author

Caffarelli C., Duse M., Martelli A., Calvani M., Cardinale F., Chiappini E., Marseglia G. L., Miraglia Del Giudice M., Tosca M. A., Castagnoli R., Brambilla I., Santoro A., Procaccianti M., Giannetti A., Ricci G., Minasi D., Caffarelli, C., Duse, M., Martelli, A., Calvani, M., Cardinale, F., Chiappini, E., Marseglia, G. L., Miraglia Del Giudice, M., Tosca, M. A., Castagnoli, R., Brambilla, I., Santoro, A., Procaccianti, M., Giannetti, A., Ricci, G., and Minasi, D.

Subjects
IgE antibodie, food allergy, allergic rhinitis, Urticaria, angioedema, Review, asthma, vaccination, children, Anaphylaxi, immune system diseases, parasitic diseases, Allergic rhiniti, anaphylaxis, Humans, Chronic Urticaria, skin and connective tissue diseases, Child, IgE antibodies
Abstract

Histaminergic urticaria-angiodema is a common complaint in children. According to clinical criteria, it is classified as acute and chronic urticaria. A further clinical classification relies on triggering factors. We focus on diagnosis and therapeutic strategies. We report the main progresses in the field and issues that remain to be understood. (www.actabiomedica.it)

Published
2020

36. Immunological basis of virus-host interaction in COVID-19

Author

La Torre, F, Leonardi, L, Giardino, G, Volpi, S, Federici, S, Soresina, A, Cancrini, C, Lougaris, V, Castagnoli, R, Corrente, S, Cardinale, F, Immunology Commission of the Italian Society of Pediatric Allergy, I, La Torre, F., Leonardi, L., Giardino, G., Volpi, S., Federici, S., Soresina, A., Cancrini, C., Lougaris, V., Castagnoli, R., Corrente, S., and Cardinale, F.

Subjects
Coronavirus disease 2019 (COVID-19), Immunology, Inflammation, Virus-host interaction, Covid‐19 in Children and Adolescents, 03 medical and health sciences, 2020 Update from The Italian Society of Pediatric Allergy and Immunology, 0302 clinical medicine, Immune system, Host Microbial Interaction, children, COVID‐19, Medicine, Immunology and Allergy, Humans, 030212 general & internal medicine, Pediatrics, Perinatology, and Child Health, Host Microbial Interactions, Kawasaki disease, business.industry, SARS-CoV-2, COVID-19, cytokine storm, immunopathogenesis, Cytokine Release Syndrome, Pneumonia, medicine.disease, Cytokine release syndrome, Settore MED/02, 030228 respiratory system, immunopathogenesi, Pediatrics, Perinatology and Child Health, Etiology, Supplement Article, Viral disease, medicine.symptom, business, Cytokine storm, Human
Abstract

COVID‐19 is a complex new viral disease, in which a strict balance between anti‐viral immune response and the ensuing organ inflammation has a critical role in determining the clinical course. In adults, compelling evidence exists indicating that an uncontrolled inflammatory response ("cytokine storm") is pivotal in determining disease progression and mortality. Children may rarely present with severe disease. Modulating factors related to the host's genetic factors, age‐related susceptibility, and the capability to mount appropriate immune responses might play a role in control virus load at an early stage and regulating the inflammatory reaction. Elucidating these mechanisms seems crucial in developing target therapies according to patient's age, immunologic status, and disease evolution in COVID‐19.

Published
2020

37. Relationship between quality of life and behavioural disorders in children with persistent asthma: a Multiple Indicators Multiple Causes (MIMIC) model

Author

Montalbano, L., Ferrante, G., Montella, S., Cilluffo, G., Di Marco, A., Bozzetto, S., Di Palmo, E., Licari, A., Leonardi, L., Caldarelli, V., Ghezzi, M., La Grutta, S., Rusconi, F., Amarri, S., Barni, S., Capizzi, A., Cardinale, F., Carraro, S., Cazzato, S., Cutrera, R., Di Pillo, S., Duse, M., Fenu, G., Kantar, A., Leonardi, S., Lombardi, E., Marseglia, G. L., Nosetti, L., Novembre, E., Patria, M. F., Piacentini, G., Pisi, G., Ricci, G., Sacco, O., Santamaria, F., Tenero, L., Tosca, M. A., Tripodi, M. C., Volpini, A., Montalbano, L., Ferrante, G., Montella, S., Cilluffo, G., Di Marco, A., Bozzetto, S., Di Palmo, E., Licari, A., Leonardi, L., Caldarelli, V., Ghezzi, M., La Grutta, S., Rusconi, F., Amarri, S., Barni, S., Capizzi, A., Cardinale, F., Carraro, S., Cazzato, S., Cutrera, R., Di Pillo, S., Duse, M., Fenu, G., Kantar, A., Leonardi, S., Lombardi, E., Marseglia, G. L., Nosetti, L., Novembre, E., Patria, M. F., Piacentini, G., Pisi, G., Ricci, G., Sacco, O., Santamaria, F., Tenero, L., Tosca, M. A., Tripodi, M. C., Volpini, A., and Montalbano L, Ferrante G, Montella S, Cilluffo G, Di Marco A, Bozzetto S, Di Palmo E, Licari A, Leonardi L, Caldarelli V, Ghezzi M, La Grutta S, Rusconi F

Subjects
Male, Moderate asthma, Asthma severity, lcsh:Medicine, 010501 environmental sciences, Behavioural disorders, Logistic regression, Paediatric research, 01 natural sciences, Article, 03 medical and health sciences, 0302 clinical medicine, Medical research, Quality of life, children, Human behaviour, medicine, Humans, Psychology, Child, lcsh:Science, 0105 earth and related environmental sciences, Asthma, Problem Behavior, Multidisciplinary, business.industry, children asthma, lcsh:R, Case-control study, asthma, medicine.disease, humanities, behavioural disorder, Logistic Models, 030228 respiratory system, behavioural disorders, quality of life, Case-Control Studies, Female, lcsh:Q, Persistent asthma, business, Clinical psychology
Abstract

Knowledge on multiple interdependences between quality of life (QoL) and behavioural problems in relation to asthma severity and control is undetermined. The aims of the study were: (i) to assess the relationship of QoL and behavioural problems with asthma severity and control (ii) to predict children’s “abnormal/borderline” status with variation in QoL. For these purposes a multicenter case-control study on 47 Severe Asthma (SA) and 94 Moderate Asthma (MA) children was performed. The MIMIC approach was applied to investigate the effect of SA and non-controlled asthma (NC) on QoL and behavioural disorders. Logistic regression was used to estimate probabilities of having an “abnormal/borderline” status with variation in QoL. The MIMIC model showed that the magnitude of the effect of SA and NC was larger on QoL (β = −0.37 and β = −0.30, respectively) than on behavioural problems (β = 0.27). With regards to the probability of having a borderline status, in MA a QoL of 1 returned a probability of 0.81, whereas in SA a QoL of 1 returned a probability of 0.89. In conclusion, SA children are highly affected by impaired QoL and behavioural problems. The MIMIC model allowed us to obtain a comprehensive assessment of QoL and behavioural problems with asthma severity and control.

Published
2020

38. Multicentre Italian study of SARS-CoV-2 infection in children and adolescents, preliminary data as at 10 April 2020

Author

Garazzino, S., Montagnani, C., Dona, D., Meini, A., Felici, E., Vergine, G., Bernardi, S., Giacchero, R., Vecchio, A. L., Marchisio, P., Nicolini, G., Pierantoni, L., Rabbone, I., Banderali, G., Denina, M., Venturini, E., Krzysztofiak, A., Badolato, R., Bianchini, S., Galli, L., Villani, A., Castelli-Gattinara, G., Salvini, F., Abbagnato, L., Castagnola, E., Dodi, I., Ghitti, C., Lippi, P., Agostiniani, R., Cherubini, S., Valentini, P., Gianino, P., Vaccaro, A., Manzoni, P., Verna, P., Comberiati, P., Di Filippo, P., Gallia, P., Battezzati, G., Fiore, L., Dalmazzo, C., Tappi, E., Lazzerini, M., Tovo, P. -A., Scolfaro, C., Pruccoli, G., Ramenghi, U., Giaquinto, C., da Dalt, L., Tornese, G., Berlese, P., Plebani, A., Manno, E. C., Santilli, V., Lancella, L., Cursi, L., Campana, A., Bozzola, E., Bosis, S., Lanari, M., Pecoraro, C., Del Barba, P., Nicastro, E., Esposito, S., Zuccotti, G. V., Corsello, G., Cardinale, F., Tocco, A. M., Ballardini, G., Agostoni, C., Chiappini, E., Indolfi, G., Anna, B., Cazzato, S., Zavarise, G., Pignata, C., Marchetti, F., Garazzino S., Montagnani C., Dona D., Meini A., Felici E., Vergine G., Bernardi S., Giacchero R., Vecchio A.L., Marchisio P., Nicolini G., Pierantoni L., Rabbone I., Banderali G., Denina M., Venturini E., Krzysztofiak A., Badolato R., Bianchini S., Galli L., Villani A., Castelli-Gattinara G., Salvini F., Abbagnato L., Castagnola E., Dodi I., Ghitti C., Lippi P., Agostiniani R., Cherubini S., Valentini P., Gianino P., Vaccaro A., Manzoni P., Verna P., Comberiati P., Di Filippo P., Gallia P., Battezzati G., Fiore L., Dalmazzo C., Tappi E., Lazzerini M., Tovo P.-A., Scolfaro C., Pruccoli G., Ramenghi U., Giaquinto C., da Dalt L., Tornese G., Berlese P., Plebani A., Manno E.C., Santilli V., Lancella L., Cursi L., Campana A., Bozzola E., Bosis S., Lanari M., Pecoraro C., Del Barba P., Nicastro E., Esposito S., Zuccotti G.V., Corsello G., Cardinale F., Tocco A.M., Ballardini G., Agostoni C., Chiappini E., Indolfi G., Anna B., Cazzato S., Zavarise G., Pignata C., Marchetti F., Garazzino, S., Montagnani, C., Dona, D., Meini, A., Felici, E., Vergine, G., Bernardi, S., Giacchero, R., Vecchio, A. L., Marchisio, P., Nicolini, G., Pierantoni, L., Rabbone, I., Banderali, G., Denina, M., Venturini, E., Krzysztofiak, A., Badolato, R., Bianchini, S., Galli, L., Villani, A., Castelli-Gattinara, G, Tornese, G, Filippo Salvini, Laura Abbagnato, Elio Castagnola, Icilio Dodi, Cesare Ghitti, Paola Lippi, Rino Agostiniani, Simonetta Cherubini, Piero Valentini, Paola Gianino, Angelina Vaccaro, Paolo Manzoni, Paola Verna, Pasquale Comberiati, Paola Di Filippo, Paola Gallia, Gianna Battezzati, Ludovica Fiore, Cristina Dalmazzo, Eleonora Tappi, Marta Lazzerini, PierAngelo Tovo, Carlo Scolfaro, Giulia Pruccoli, Ugo Ramenghi, Carlo Giaquinto, Liviana Da Dalt, Gianluca Tornese, Paola Berlese, Alessandro Plebani, Emma Concetta Manno, Veronica Santilli, Laura Lancella, Laura Cursi, Andrea Campana, Elena Bozzola, Samantha Bosis, Marcello Lanari, Carmine Pecoraro, Paolo Del Barba, Emanuele Nicastro, Silvia Garazzino, Carlotta Montagnani, Daniele Donà, Antonella Meini, Enrico Felici, Gianluca Vergine, Stefania Bernardi, Roberta Giacchero, Andrea Lo Vecchio, Paola Marchisio, Giangiacomo Nicolini, Luca Pierantoni, Ivana Rabbone, Giuseppe Banderali, Marco Denina, Elisabetta Venturini, Andrzej Krzysztofiak , Raffaele Badolato, Sonia Bianchini, Luisa Galli, Alberto Villani , Guido Castelli-Gattinara, Susanna Esposito, Gian Vincenzo Zuccotti, Giovanni Corsello, Fabio Cardinale, Anna Maria Tocco, Giuseppina Ballardini, Carlo Agostoni, Elena Chiappini, Giuseppe Indolfi, Bussolini Anna, Salvatore Cazzato, Giorgio Zavarise, Claudio Pignata, Federico Marchetti, Lo Vecchio, A., and Castelli-Gattinara, G.

Subjects
Male, Pediatrics, Epidemiology, Protease Inhibitor, Comorbidity, medicine.disease_cause, Clinical Laboratory Technique, Severe Acute Respiratory Syndrome, Disease Outbreaks, Feces, 0302 clinical medicine, Settore MED/38 - Pediatria Generale E Specialistica, COVID-19 Testing, Retrospective Studie, Pandemic, 030212 general & internal medicine, Viral, Child, Coronavirus, Pediatric, Disease Outbreak, Coinfection, Hospitals, Pediatric, Settore MED/38, Hospitals, Diarrhea, Treatment Outcome, SARS-CoV-2 infection, children, covid-19, hydroxychloroquine, pneumonia, Adolescent, Antiviral Agents, Betacoronavirus, COVID-19, Child, Preschool, Chronic Disease, Clinical Laboratory Techniques, Coronavirus Infections, Female, Fever, Humans, Immunocompromised Host, Infant, Infant, Newborn, Italy, Noninvasive Ventilation, Pandemics, Pneumonia, Viral, Protease Inhibitors, Retrospective Studies, SARS-CoV-2, medicine.symptom, Rapid Communication, Human, medicine.medical_specialty, Coronaviru, 03 medical and health sciences, 030225 pediatrics, Virology, Intensive care, medicine, Preschool, Antiviral Agent, Betacoronaviru, business.industry, Coronavirus Infection, Public Health, Environmental and Occupational Health, Retrospective cohort study, medicine.disease, Newborn, Pneumonia, Fece, business
Abstract

Data on features of severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) in children and adolescents are scarce. We report preliminary results of an Italian multicentre study comprising 168 laboratory-confirmed paediatric cases (median: 2.3 years, range: 1 day–17.7 years, 55.9% males), of which 67.9% were hospitalised and 19.6% had comorbidities. Fever was the most common symptom, gastrointestinal manifestations were frequent; two children required intensive care, five had seizures, 49 received experimental treatments and all recovered.

Published
2020

39. Primary atopic disorders and chronic skin disease

Author

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

Subjects
IgE, allergy, inborn errors of immunity, primary atopic disorders, skin, Hypersensitivity, Immediate, Immediate, Urticaria, Immunology, Eczema, Skin Diseases, Settore MED/02, Humans, Job Syndrome, Pediatrics, Perinatology and Child Health, Hypersensitivity, Immunology and Allergy
Abstract

Primary atopic disorders (PADs) are monogenic diseases characterized by allergy or atopy-related symptoms as fundamental features. In patients with PADs, primary immune deficiency and immune dysregulation symptoms are usually coexist. Chronic skin disease, manifesting with erythroderma, severe atopic dermatitis or eczema, and urticaria, is one of the main features observed in PADs, such as hyper-IgE syndromes, Omenn syndrome, Wiskott-Aldrich syndrome, IPEX-linked syndrome, skin barrier disorders, as well as some autoinflammatory diseases. The recognition of PADs in the context of an allergic phenotype is crucial to ensure prompt diagnosis and appropriate treatment. This article provides an overview of the main PADs with skin involvement.

Published
2021

43. Characteristics and risk factors for SARS-CoV-2 in children tested in the early phase of the pandemic: a cross-sectional study, Italy, 23 February to 24 May 2020

Author

Lazzerini, M., Sforzi, I., Trapani, S., Biban, P., Silvagni, D., Villa, G., Tibaldi, J., Bertacca, L., Felici, E., Perricone, G., Parrino, R., Gioe, C., Lega, S., Conte, Fabrizio Paolo Massimo, Marchetti, Fabio, Magista, A., Berlese, P., Martelossi, S., Vaienti, F., Valletta, E., Mauro, Michele, Dall'Amico, R., Fasoli, S., Gatto, Antonio, Chiaretti, Antonio, Dragovic, D., Pascolo, P., Pilotto, C., Liguoro, I., Miorin, E., Saretta, F., Trobia, G. L., Di Stefano, A., Orlandi, Armando, Cardinale, F., Lubrano, R., Testa, A., Binotti, M., Moressa, V., Barbi, E., Armocida, B., Mariani, I., Conte M., Marchetti F., Mauro M., Gatto A., Chiaretti A. (ORCID:0000-0002-9971-1640), Orlandi A. (ORCID:0000-0001-5253-4678), Lazzerini, M., Sforzi, I., Trapani, S., Biban, P., Silvagni, D., Villa, G., Tibaldi, J., Bertacca, L., Felici, E., Perricone, G., Parrino, R., Gioe, C., Lega, S., Conte, Fabrizio Paolo Massimo, Marchetti, Fabio, Magista, A., Berlese, P., Martelossi, S., Vaienti, F., Valletta, E., Mauro, Michele, Dall'Amico, R., Fasoli, S., Gatto, Antonio, Chiaretti, Antonio, Dragovic, D., Pascolo, P., Pilotto, C., Liguoro, I., Miorin, E., Saretta, F., Trobia, G. L., Di Stefano, A., Orlandi, Armando, Cardinale, F., Lubrano, R., Testa, A., Binotti, M., Moressa, V., Barbi, E., Armocida, B., Mariani, I., Conte M., Marchetti F., Mauro M., Gatto A., Chiaretti A. (ORCID:0000-0002-9971-1640), and Orlandi A. (ORCID:0000-0001-5253-4678)

Abstract

BackgroundVery few studies describe factors associated with COVID-19 diagnosis in children.AimWe here describe characteristics and risk factors for COVID-19 diagnosis in children tested in 20 paediatric centres across Italy.MethodsWe included cases aged 0-18 years tested between 23 February and 24 May 2020. Our primary analysis focused on children tested because of symptoms/signs suggestive of COVID-19.ResultsAmong 2,494 children tested, 2,148 (86.1%) had symptoms suggestive of COVID-19. Clinical presentation of confirmed COVID-19 cases included besides fever (82.4%) and respiratory signs or symptoms (60.4%) also gastrointestinal (18.2%), neurological (18.9%), cutaneous (3.8%) and other unspecific influenza-like presentations (17.8%). In multivariate analysis, factors significantly associated with SARS-CoV-2 positivity were: exposure history (adjusted odds ratio (AOR): 39.83; 95% confidence interval (CI): 17.52-90.55; p < 0.0001), cardiac disease (AOR: 3.10; 95% CI: 1.19-5.02; p < 0.0001), fever (AOR: 3.05%; 95% CI: 1.67-5.58; p = 0.0003) and anosmia/ageusia (AOR: 4.08; 95% CI: 1.69-9.84; p = 0.002). Among 190 (7.6%) children positive for SARS-CoV-2, only four (2.1%) required respiratory support and two (1.1%) were admitted to intensive care; all recovered.ConclusionRecommendations for SARS-CoV-2 testing in children should consider the evidence of broader clinical features. Exposure history, fever and anosmia/ageusia are strong risk factors in children for positive SARS-CoV-2 testing, while other symptoms did not help discriminate positive from negative individuals. This study confirms that COVID-19 was a mild disease in the general paediatric population in Italy. Further studies are needed to understand risk, clinical spectrum and outcomes of COVID-19 in children with pre-existing conditions.

Published
2021

44. Revised recommendations of the Italian Society of Pediatrics about the general management of Kawasaki disease.

Author

Marchesi, A, Rigante, Donato, Cimaz, R, Ravelli, A, Tarissi de Jacobis, I, Rimini, A, Cardinale, F, Cattalini, M, De Zorzi, A, Dellepiane, Rm, Salice, P, Secinaro, A, Taddio, A, Palma, P, El Hachem, M, Cortis, E, Maggio, Mc, Corsello, G, Villani, A, Rigante D (ORCID:0000-0001-7032-7779), Marchesi, A, Rigante, Donato, Cimaz, R, Ravelli, A, Tarissi de Jacobis, I, Rimini, A, Cardinale, F, Cattalini, M, De Zorzi, A, Dellepiane, Rm, Salice, P, Secinaro, A, Taddio, A, Palma, P, El Hachem, M, Cortis, E, Maggio, Mc, Corsello, G, Villani, A, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

Aim of these revised recommendations for the general management of Kawasaki disease is to encourage its prompter recognition and warrant the most appropriate therapy, based on ascertained scientific data, raising awareness of the complications related to misdiagnosis or delayed treatment. A set of 20 synthetic operative statements is herein provided, including the definition of Kawasaki disease, its protean presentations, clinical course and seminal treatment modalities of all disease phases. The application of these recommendations should improve prognosis of Kawasaki disease and prevent the progression to permanent vascular abnormalities, thereby diminishing morbidity and mortality.

Published
2021

49. Foreword

Author

Caffarelli, C., Calvani, M., Cardinale, F., Chiappini, E., Ciprandi, G., Cravidi, C., Duse, M., Galli, E., Licari, A., Manti, S., Martelli, A., Minasi, D., Del Giudice, M. M., Pajno, G. B., Ricci, G., Tosca, M. A., and Marseglia, G. L.

Subjects
Italy, Hypersensitivity, Humans, Foreword, Child, Delivery of Health Care
Abstract

The management of chronic diseases are paramount in health care in these days. Among them, there has been an expansion of allergic and immunologic diseases, especially in children. Thanks to the action of the Italian Society of Pediatric Allergy and Immunology (SIAIP), the quality level of care has progressively grown. SIAIP developed a task force with the purpose of proposing updated models for assessing, and prescribing treatment in the allergy and immunology field that have been issued in this supplement. In a very difficult time for everyone, current developments covering a broad range of topics in the field are presented. All Authors have to be thanked, since they have participated with passion and have taken valuable time away from their professional and private interests. We hope that the readership will enjoy these papers.

Published
2020

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