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148 results on '"Mannarino, S"'

2. Ventricular pre-excitation in young patients: long term follow-up and natural history in a contemporary cohort

Author

Seganti, A, primary, Sanzo, A, additional, Codazzi, A C, additional, Raso, I, additional, Bongiorno, A, additional, Fino, R, additional, Munafo', A R, additional, Apicella, A, additional, Loiacono, F, additional, Frassica, R, additional, Santacesaria, S, additional, Petracci, B, additional, Mannarino, S, additional, and Rordorf, R, additional

Published
2023
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5. Multisystem Inflammatory Syndrome in Children Unmasking Brugada Type 1 Pattern

Author

Corti, C.G., Seganti, A., Sanzo, A., Nespoli, L.F., Goletto, S., Izzo, F., Zuccotti, G.V., and Mannarino, S.

Subjects
Settore MED/38 - Pediatria Generale e Specialistica, autonomic system dysregulation, Brugada phenocopy, cytokine storm, Brugada syndrome, multisystem inflammatory syndrome in children
Published
2022

6. Congenital heart defects in monochorionic twin pregnancy complicated by selective fetal growth restriction.

Author

Faiola, S., Casati, D., Nelva Stellio, L., Laoreti, A., Corti, C., Mannarino, S., Lanna, M., and Cetin, I.

Subjects
MULTIPLE pregnancy, FETAL growth retardation, CONGENITAL heart disease, TWINS, PREGNANCY complications, FETAL monitoring
Abstract

Objectives: To evaluate the prevalence, subtypes and postnatal outcomes of congenital heart defects (CHD) in a cohort of monochorionic diamniotic (MCDA) twin pregnancies complicated by selective fetal growth restriction (sFGR), and to compare this population with a cohort of uncomplicated MCDA pregnancies evaluated during the same period. Methods: This was a retrospective analysis of all consecutive MCDA pregnancies referred between 2009 and 2018, including those complicated by sFGR (Group A) and those without complications (Group B). All neonates delivered in our center were screened for CHD before discharge. Discharge letters for all those delivered elsewhere were retrieved. Pregnancies with complications other than sFGR and those without perinatal follow‐up were excluded. Pregnancies in Group A were divided into three types according to the Gratacós system of sFGR classification. Results: A total of 870 MCDA twin pregnancies were included: 296 in Group A and 574 in Group B. In Group A, the prevalence of CHD was 3.7% (22/592 twins), with no significant difference in CHD frequency between the three types of sFGR (Type I, 3.7%; Type II, 3.2%; Type III, 4.2%; P = 0.55). Of four Type‐III sFGR pregnancies with CHD, one had pulmonary stenosis (PS) in the larger twin and isolated coarctation of the aorta in the smaller cotwin, and three had PS in the larger twin only. No Type‐III sFGR pregnancies in which only the smaller twin was affected by CHD were observed. Of 11 CHD cases in the larger twin, 10 (91%) were right ventricular outflow tract abnormalities (RVOTA), and one (9%) was a ventricular septal defect. In the smaller twins, 11 cases of CHD were observed, covering a broad spectrum of cardiac abnormalities. In Group B, the CHD prevalence was 1.1% (13/1148 twins), which was similar to that in the general population, according to the EUROCAT registry for the same period and geographical area of the study (0.96%; P = 0.579). The CHD prevalence was significantly higher in Group A compared with Group B (3.7% vs 1.1%; P = 0.0002; odds ratio, 3.57 (95% CI, 1.78–7.22)). In all pregnancies with CHD in the study population, the anomaly was discordant. Conclusions: In MCDA twin pregnancy, sFGR was associated with a three‐fold higher prevalence of CHD. Women with such pregnancies should be referred to a tertiary care hospital for pre‐ and postnatal cardiac evaluation, treatment and long‐term follow‐up. In larger twins, the only major CHD observed was RVOTA, while a broad spectrum of CHD was noted in smaller twins. The higher risk of CHD in MCDA pregnancies appears to be due to the typical complications of the monochorionic pregnancy, rather than to the monochorionic nature of the pregnancy itself. © 2022 International Society of Ultrasound in Obstetrics and Gynecology. [ABSTRACT FROM AUTHOR]

Published
2023
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8. Lombardy regional urgent reorganization for congenital cardiac patients following the Covid-19 pandemic

Author

Chessa M, Varrica A, Andronache A, Carminati M, Colli AM, D'Aiello AF, Ferrero P, Mannarino S, Marcora S, Marianeschi SM, Micheletti A, Piazza L, Saracino A, Uricchio N, Vignati G, Giamberti A., Chessa, M, Varrica, A, Andronache, A, Carminati, M, Colli, Am, D'Aiello, Af, Ferrero, P, Mannarino, S, Marcora, S, Marianeschi, Sm, Micheletti, A, Piazza, L, Saracino, A, Uricchio, N, Vignati, G, and Giamberti, A.

Published
2020

10. Non-thyroidal illness syndrome and SARS-CoV-2-associated multisystem inflammatory syndrome in children

Author

Calcaterra, V., primary, Biganzoli, G., additional, Dilillo, D., additional, Mannarino, S., additional, Fiori, L., additional, Pelizzo, G., additional, Zoia, E., additional, Fabiano, V., additional, Carlucci, P., additional, Camporesi, A., additional, Corti, C., additional, Mercurio, G., additional, Izzo, F., additional, Biganzoli, E., additional, and Zuccotti, G., additional

Published
2021
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11. Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey

Author

Cattalini, M, Della Paolera, S, Zunica, F, Bracaglia, C, Giangreco, M, Verdoni, L, Meini, A, Sottile, R, Caorsi, R, Zuccotti, G, Fabi, M, Montin, D, Meneghel, A, Consolaro, A, Dellepiane, Rm, Maggio, Mc, La Torre, F, Marchesi, A, Simonini, G, Villani, A, Cimaz, R, Ravelli, A, Taddio, A, Adamoli, P, Alberelli, Mc, Alizzi, C, Barone, P, Bennato, V, Biscaro, F, Bossi, G, Campana, A, Carone, M, Civino, A, Conti, Giorgio, Dei Rossi, E, Del Giudice, E, Dell'Anna, A, De Luca, M, Felici, E, Filocamo, G, Floretta, I, Foschini, Ml, Lanari, M, Lattanzi, B, Lazzerotti, A, Licciardi, F, Manerba, A, Mannarino, S, Marino, A, Marolda, A, Martelli, L, Martini, G, Mauro, A, Mastrolia, Mv, Mazza, A, Miniaci, A, Minoia, F, Olivieri, A, Pennoni, G, Pignataro, R, Ricci, F, Rigante, Donato, Rossi, M, Santagati, C, Soliani, M, Sonego, S, Sperlì, D, Stucchi, S, Teruzzi, B, Tierno, E, Utytatnikova, T, Valentini, Piero, Vergine, G, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), Valentini P (ORCID:0000-0001-6095-9510), Cattalini, M, Della Paolera, S, Zunica, F, Bracaglia, C, Giangreco, M, Verdoni, L, Meini, A, Sottile, R, Caorsi, R, Zuccotti, G, Fabi, M, Montin, D, Meneghel, A, Consolaro, A, Dellepiane, Rm, Maggio, Mc, La Torre, F, Marchesi, A, Simonini, G, Villani, A, Cimaz, R, Ravelli, A, Taddio, A, Adamoli, P, Alberelli, Mc, Alizzi, C, Barone, P, Bennato, V, Biscaro, F, Bossi, G, Campana, A, Carone, M, Civino, A, Conti, Giorgio, Dei Rossi, E, Del Giudice, E, Dell'Anna, A, De Luca, M, Felici, E, Filocamo, G, Floretta, I, Foschini, Ml, Lanari, M, Lattanzi, B, Lazzerotti, A, Licciardi, F, Manerba, A, Mannarino, S, Marino, A, Marolda, A, Martelli, L, Martini, G, Mauro, A, Mastrolia, Mv, Mazza, A, Miniaci, A, Minoia, F, Olivieri, A, Pennoni, G, Pignataro, R, Ricci, F, Rigante, Donato, Rossi, M, Santagati, C, Soliani, M, Sonego, S, Sperlì, D, Stucchi, S, Teruzzi, B, Tierno, E, Utytatnikova, T, Valentini, Piero, Vergine, G, Conti G (ORCID:0000-0002-8566-9365), Rigante D (ORCID:0000-0001-7032-7779), and Valentini P (ORCID:0000-0001-6095-9510)

Abstract

Background: There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities. Methods: The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group – KDG) or KD-like (Kawacovid Group - KCG) disease between February 1st 2020, and May 31st 2020. Demographic, clinical, laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAP®). Relationship between clinical presentation and SARSCoV-2 infection was also taken into account. Moreover, clinical characteristics of KDG during SARS-CoV-2 epidemic (KDG-CoV2) were compared to Kawasaki Disease patients (KDG-Historical) seen in three different Italian tertiary pediatric hospitals (Institute for Maternal and Child Health, IRCCS “Burlo Garofolo”, Trieste; AOU Meyer, Florence;IRCCS Istituto Giannina Gaslini, Genoa) from January 1st 2000 to December 31st 2019. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groups. Results: One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis. 37,8% of patients among KCG presented hypotension/ non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated ferritin and troponin-T characterized KCG. KDG received more freque

Published
2021

12. Diagnostic accuracy of a clinical diagnosis of idiopathic pulmonary fibrosis: an international case-cohort study

Author

Walsh S. L. F., Maher T. M., Kolb M., Poletti V., Nusser R., Richeldi L., Vancheri C., Wilsher M. L., Antoniou K. M., Behr J., Bendstrup E., Brown K., Calandriello L., Corte T. J., Cottin V., Crestani B., Flaherty K., Glaspole I., Grutters J., Inoue Y., Kokosi M., Kondoh Y., Kouranos V., Kreuter M., Johannson K., Judge E., Ley B., Margaritopoulos G., Martinez F. J., Molina-Molina M., Morais A., Nunes H., Raghu G., Ryerson C. J., Selman M., Spagnolo P., Taniguchi H., Tomassetti S., Valeyre D., Wijsenbeek M., Wuyts W., Hansell D., Wells A., Zhu P. S., Yuan Y., Yoshito Fukuda C., Yoshimatsu Y., Xaubet A., Wong A. M., White P., Westney G., West A., Wessendorf T., Waseda Y., Wang C., Vienna J. M., Videnovic Ivanov J., Vicens Zygmunt V., Venero Caceres M. C., Velasquez Pinto G., Veitch E., Vasakova M., Varone F., Varela B. E., Van Hal P., Van De Ven M., Van Der Lee I., Van Den Toorn L., Urrutia Gajate A., Urban J., Ugarte Fornell L. G., Tzouvelekis A., Twohig K., Turner A., Trujillo S., Triani A., Traila D., Torres V., Tomioka H., Tomii K., Tomic R., Toma C., Tokgoz Akyil F., Tobino K., Tobar R., Tiwari A., Tibana R., Tian X., Thillai M., Tham W., Teo F., Tekavec Trkanjec J., Teixeira P., Tarpey D., Tapias L., Tanizawa K., Tanino Y., Takada T., Tabaj G., Szolnoki E., Swarnakar R., Strambu I., Sterclova M., Spinks K., Soo C. I., Soltani A., Solanki S., Sobh E., Soares M. R., Smith J., Smith B., Slocum P., Slabbynck H., Sivokozov I., Shifren A., Shen S. M., Sharp C., Shanmuganathan A., Sebastiani A., Scarlata S., Savas R., Sasaki S., Santeliz J., Santana ANC., Sanchez R., Salinas M., Saito S., Ryan F., Royo Prats J. A., Rosi E., Rokadia H., Robles Perez A., Rivera Ortega P., Rio Ramirez M., Righetti S., Reichner C., Ravaglia C., Ratanawatkul P., Ramalingam V., Rajasekaran A., Radzikowska E., Ra S. W., Quadrelli S., Precerutti J., Prasad J., Popa D., Pizzalato S., Piotrowski W., Pineiro A., Piloni D., Peros Golubicic T., Perez R., Pereira C., Pereira B., Perch M., Patel N., Patel D., Papanikolaou I., Papakosta D., Panselinas E., Pang Y. K., Pandya P., Padrao E., Ozdemir Kumbasar O., Overbeek M. J., Otto Minasian A., O'Riordan D., Ora J., Oldham J., Okutan O., Ohshimo S., Oguzulgen I. K., Ogura T., O'Donnell T., O'Dochartaigh C., O'Beirne S., Novikova L., Novelli L., Noth I., Nogueira Mendes Neto N., Niroumand M., Nieto A., Neves A., Nambiar A., Nair S., Nadama R., Murtagh E., Mura M., Muller Quernheim J., Mukhopadhyay A., Mukherjee S., Morisset J., Moran O., Mooney J., Moller J., Mogulkoc N., Miyamoto A., Milenkovic B., Mette S., Mejia M., Mei F., Mazzei M., Matsuda T., Mason C., Martinez Frances M., Mannarino S., Mancuzo E., Malli F., Malhotra P., Maillo M., Maia J., Mahdavian M., Madsen F., Luckhardt T., Lucht W., Low S. Y., Lopez Miguel C. P., Lipchik R., Levy S., Levin K., Lee K. L., Lederer D., Lammi M. R., Kwan H. Y., Kukreja S., Kruavit A., Kotecki M., Kolilekas L., Knoop H., Kiyan E., Kishaba T., King Biggs M., Khor Y. H., Khan A., Khalil N., Kedia R., Kebba N., Kawano Dourado L., Kapitan K., Kan C. D., Kalyoncu A. F., Kalluri M., Kabasakal Y., Jyothula S., Juretschke M. A., Jovanovic D., Jonkers R., Jo H., Izumi S., Ishii H., Ikeda S., Ibrahim A., Hyldgaard C., Hunninghake G., Huie T., Hufton A., Hu X., Hseih W. C., Hoyos R., Hoyles R., Holguin Rodriguez O., Hogan M. P., Hodgson U., Hilkin Sogoloff H., Herrera E., Henry B. M., Hellemons M., Hecimovic A., Hayashi R., Hart S., Harari S., Haney S., Hambly N., Hakkim R., Gutierrez M., Gripaldo R., Gomez A., Goh N., Godoy R., Gilbert C., Giannarakis I., Gasparini S., Garcha P., Furtado S., Fois A., Flood Page P., Fletcher S., Fiss E., Figueroa Casas J., Figueroa Casas M., Fiddler C. A., Ferrara G., Fernandez Casares M., Felton C., Faverio P., Fabro A. T., Estrada A., Errhalt P., Enomoto N., Enghelmayer J. I., El Kersh K., Eiger G., Dubaniewicz A., Drakopanagiotakis F., Disayabutr S., Dijkstra A., Diaz Patino J. C., Diaz Castanon J. J., Dhooria S., Dhasmana D. J., De Rosa M., De Luca S., Delobbe A., Delgado D., Delgado C., De La Fuente I., De Kruif M., De Gier M., De Andrade J., Davidsen J. R., Daoud B., Dalhoff K., Cotera Solano J. V., Costa A. N., Coronel S., Confalonieri M., Conemans L., Comellas A., Colella S., Clemente S., Clark J., Ciuffreda M., Chung C. L., Chong S. G., Chirita D., Chen P. L., Chaudhuri N., Chambers D., Chalmers G., Chairman D., Chai G. T., Chacon Chaves R., Cetinsu V., Ceruti M., Ceballos Zuniga C. O., Castillo D., Carbone R. G., Caminati A., Callejas Gonzalez F. J., Butler M., Bustos C., Bukowczan M., Buendia I., Brunetti G., Brockway B., Bresser P., Breseghello J., Bouros D., Botero Zaccour J. A., Borzone G., Borie R., Blum H. C., Blank J., Biswas A., Bennett D., Benjamin M., Belaconi I. N., Beirne P., Beckert L., Bastiampillai S., Bascom R., Bartholmai B., Barros M., Ban AYL., Balestro E., Baldi B., Baddini Martinez J., Baburao A., Babu S., Averyanov A., Avdeev S., Athanazio R., Atahan E., Asuquo B., Assayag D., Antuni J., Antillon S., Anderson K. C., Anderson A., Alwani F., Altinisik G., Alsouofi N., Allam J. S., Al Jahdali H., Al Farttoosi A., Alfaro T., Al Busaidi N., Alavi Foumani A., Agreda Vedia M. G., Agarwal A., Afridi F., Adeyeye O. O., Adegunsoye A., Adamali H., Abedini A., Walsh, S. L. F., Maher, T. M., Kolb, M., Poletti, V., Nusser, R., Richeldi, L., Vancheri, C., Wilsher, M. L., Antoniou, K. M., Behr, J., Bendstrup, E., Brown, K., Calandriello, L., Corte, T. J., Cottin, V., Crestani, B., Flaherty, K., Glaspole, I., Grutters, J., Inoue, Y., Kokosi, M., Kondoh, Y., Kouranos, V., Kreuter, M., Johannson, K., Judge, E., Ley, B., Margaritopoulos, G., Martinez, F. J., Molina-Molina, M., Morais, A., Nunes, H., Raghu, G., Ryerson, C. J., Selman, M., Spagnolo, P., Taniguchi, H., Tomassetti, S., Valeyre, D., Wijsenbeek, M., Wuyts, W., Hansell, D., Wells, A., Zhu, P. S., Yuan, Y., Yoshito Fukuda, C., Yoshimatsu, Y., Xaubet, A., Wong, A. M., White, P., Westney, G., West, A., Wessendorf, T., Waseda, Y., Wang, C., Vienna, J. M., Videnovic Ivanov, J., Vicens Zygmunt, V., Venero Caceres, M. C., Velasquez Pinto, G., Veitch, E., Vasakova, M., Varone, F., Varela, B. E., Van Hal, P., Van De Ven, M., Van Der Lee, I., Van Den Toorn, L., Urrutia Gajate, A., Urban, J., Ugarte Fornell, L. G., Tzouvelekis, A., Twohig, K., Turner, A., Trujillo, S., Triani, A., Traila, D., Torres, V., Tomioka, H., Tomii, K., Tomic, R., Toma, C., Tokgoz Akyil, F., Tobino, K., Tobar, R., Tiwari, A., Tibana, R., Tian, X., Thillai, M., Tham, W., Teo, F., Tekavec Trkanjec, J., Teixeira, P., Tarpey, D., Tapias, L., Tanizawa, K., Tanino, Y., Takada, T., Tabaj, G., Szolnoki, E., Swarnakar, R., Strambu, I., Sterclova, M., Spinks, K., Soo, C. I., Soltani, A., Solanki, S., Sobh, E., Soares, M. R., Smith, J., Smith, B., Slocum, P., Slabbynck, H., Sivokozov, I., Shifren, A., Shen, S. M., Sharp, C., Shanmuganathan, A., Sebastiani, A., Scarlata, S., Savas, R., Sasaki, S., Santeliz, J., Santana, Anc., Sanchez, R., Salinas, M., Saito, S., Ryan, F., Royo Prats, J. A., Rosi, E., Rokadia, H., Robles Perez, A., Rivera Ortega, P., Rio Ramirez, M., Righetti, S., Reichner, C., Ravaglia, C., Ratanawatkul, P., Ramalingam, V., Rajasekaran, A., Radzikowska, E., Ra, S. W., Quadrelli, S., Precerutti, J., Prasad, J., Popa, D., Pizzalato, S., Piotrowski, W., Pineiro, A., Piloni, D., Peros Golubicic, T., Perez, R., Pereira, C., Pereira, B., Perch, M., Patel, N., Patel, D., Papanikolaou, I., Papakosta, D., Panselinas, E., Pang, Y. K., Pandya, P., Padrao, E., Ozdemir Kumbasar, O., Overbeek, M. J., Otto Minasian, A., O'Riordan, D., Ora, J., Oldham, J., Okutan, O., Ohshimo, S., Oguzulgen, I. K., Ogura, T., O'Donnell, T., O'Dochartaigh, C., O'Beirne, S., Novikova, L., Novelli, L., Noth, I., Nogueira Mendes Neto, N., Niroumand, M., Nieto, A., Neves, A., Nambiar, A., Nair, S., Nadama, R., Murtagh, E., Mura, M., Muller Quernheim, J., Mukhopadhyay, A., Mukherjee, S., Morisset, J., Moran, O., Mooney, J., Moller, J., Mogulkoc, N., Miyamoto, A., Milenkovic, B., Mette, S., Mejia, M., Mei, F., Mazzei, M., Matsuda, T., Mason, C., Martinez Frances, M., Mannarino, S., Mancuzo, E., Malli, F., Malhotra, P., Maillo, M., Maia, J., Mahdavian, M., Madsen, F., Luckhardt, T., Lucht, W., Low, S. Y., Lopez Miguel, C. P., Lipchik, R., Levy, S., Levin, K., Lee, K. L., Lederer, D., Lammi, M. R., Kwan, H. Y., Kukreja, S., Kruavit, A., Kotecki, M., Kolilekas, L., Knoop, H., Kiyan, E., Kishaba, T., King Biggs, M., Khor, Y. H., Khan, A., Khalil, N., Kedia, R., Kebba, N., Kawano Dourado, L., Kapitan, K., Kan, C. D., Kalyoncu, A. F., Kalluri, M., Kabasakal, Y., Jyothula, S., Juretschke, M. A., Jovanovic, D., Jonkers, R., Jo, H., Izumi, S., Ishii, H., Ikeda, S., Ibrahim, A., Hyldgaard, C., Hunninghake, G., Huie, T., Hufton, A., Hu, X., Hseih, W. C., Hoyos, R., Hoyles, R., Holguin Rodriguez, O., Hogan, M. P., Hodgson, U., Hilkin Sogoloff, H., Herrera, E., Henry, B. M., Hellemons, M., Hecimovic, A., Hayashi, R., Hart, S., Harari, S., Haney, S., Hambly, N., Hakkim, R., Gutierrez, M., Gripaldo, R., Gomez, A., Goh, N., Godoy, R., Gilbert, C., Giannarakis, I., Gasparini, S., Garcha, P., Furtado, S., Fois, A., Flood Page, P., Fletcher, S., Fiss, E., Figueroa Casas, J., Figueroa Casas, M., Fiddler, C. A., Ferrara, G., Fernandez Casares, M., Felton, C., Faverio, P., Fabro, A. T., Estrada, A., Errhalt, P., Enomoto, N., Enghelmayer, J. I., El Kersh, K., Eiger, G., Dubaniewicz, A., Drakopanagiotakis, F., Disayabutr, S., Dijkstra, A., Diaz Patino, J. C., Diaz Castanon, J. J., Dhooria, S., Dhasmana, D. J., De Rosa, M., De Luca, S., Delobbe, A., Delgado, D., Delgado, C., De La Fuente, I., De Kruif, M., De Gier, M., De Andrade, J., Davidsen, J. R., Daoud, B., Dalhoff, K., Cotera Solano, J. V., Costa, A. N., Coronel, S., Confalonieri, M., Conemans, L., Comellas, A., Colella, S., Clemente, S., Clark, J., Ciuffreda, M., Chung, C. L., Chong, S. G., Chirita, D., Chen, P. L., Chaudhuri, N., Chambers, D., Chalmers, G., Chairman, D., Chai, G. T., Chacon Chaves, R., Cetinsu, V., Ceruti, M., Ceballos Zuniga, C. O., Castillo, D., Carbone, R. G., Caminati, A., Callejas Gonzalez, F. J., Butler, M., Bustos, C., Bukowczan, M., Buendia, I., Brunetti, G., Brockway, B., Bresser, P., Breseghello, J., Bouros, D., Botero Zaccour, J. A., Borzone, G., Borie, R., Blum, H. C., Blank, J., Biswas, A., Bennett, D., Benjamin, M., Belaconi, I. N., Beirne, P., Beckert, L., Bastiampillai, S., Bascom, R., Bartholmai, B., Barros, M., Ban, Ayl., Balestro, E., Baldi, B., Baddini Martinez, J., Baburao, A., Babu, S., Averyanov, A., Avdeev, S., Athanazio, R., Atahan, E., Asuquo, B., Assayag, D., Antuni, J., Antillon, S., Anderson, K. C., Anderson, A., Alwani, F., Altinisik, G., Alsouofi, N., Allam, J. S., Al Jahdali, H., Al Farttoosi, A., Alfaro, T., Al Busaidi, N., Alavi Foumani, A., Agreda Vedia, M. G., Agarwal, A., Afridi, F., Adeyeye, O. O., Adegunsoye, A., Adamali, H., Abedini, A., National Institute for Health Research, British Lung Foundation, Walsh, S, Maher, T, Kolb, M, Poletti, V, Nusser, R, Richeldi, L, Vancheri, C, Wilsher, M, Antoniou, K, Behr, J, Bendstrup, E, Brown, K, Calandriello, L, Corte, T, Cottin, V, Crestani, B, Flaherty, K, Glaspole, I, Grutters, J, Inoue, Y, Kokosi, M, Kondoh, Y, Kouranos, V, Kreuter, M, Johannson, K, Judge, E, Ley, B, Margaritopoulos, G, Martinez, F, Molina-Molina, M, Morais, A, Nunes, H, Raghu, G, Ryerson, C, Selman, M, Spagnolo, P, Taniguchi, H, Tomassetti, S, Valeyre, D, Wijsenbeek, M, Wuyts, W, Hansell, D, Wells, A, Zhu, P, Yuan, Y, Yoshito Fukuda, C, Yoshimatsu, Y, Xaubet, A, Wong, A, White, P, Westney, G, West, A, Wessendorf, T, Waseda, Y, Wang, C, Vienna, J, Videnovic Ivanov, J, Vicens Zygmunt, V, Venero Caceres, M, Velasquez Pinto, G, Veitch, E, Vasakova, M, Varone, F, Varela, B, Van Hal, P, Van De Ven, M, Van Der Lee, I, Van Den Toorn, L, Urrutia Gajate, A, Urban, J, Ugarte Fornell, L, Tzouvelekis, A, Twohig, K, Turner, A, Trujillo, S, Triani, A, Traila, D, Torres, V, Tomioka, H, Tomii, K, Tomic, R, Toma, C, Tokgoz Akyil, F, Tobino, K, Tobar, R, Tiwari, A, Tibana, R, Tian, X, Thillai, M, Tham, W, Teo, F, Tekavec Trkanjec, J, Teixeira, P, Tarpey, D, Tapias, L, Tanizawa, K, Tanino, Y, Takada, T, Tabaj, G, Szolnoki, E, Swarnakar, R, Strambu, I, Sterclova, M, Spinks, K, Soo, C, Soltani, A, Solanki, S, Sobh, E, Soares, M, Smith, J, Smith, B, Slocum, P, Slabbynck, H, Sivokozov, I, Shifren, A, Shen, S, Sharp, C, Shanmuganathan, A, Sebastiani, A, Scarlata, S, Savas, R, Sasaki, S, Santeliz, J, Santana, A, Sanchez, R, Salinas, M, Saito, S, Ryan, F, Royo Prats, J, Rosi, E, Rokadia, H, Robles Perez, A, Rivera Ortega, P, Rio Ramirez, M, Righetti, S, Reichner, C, Ravaglia, C, Ratanawatkul, P, Ramalingam, V, Rajasekaran, A, Radzikowska, E, Ra, S, Quadrelli, S, Precerutti, J, Prasad, J, Popa, D, Pizzalato, S, Piotrowski, W, Pineiro, A, Piloni, D, Peros Golubicic, T, Perez, R, Pereira, C, Pereira, B, Perch, M, Patel, N, Patel, D, Papanikolaou, I, Papakosta, D, Panselinas, E, Pang, Y, Pandya, P, Padrao, E, Ozdemir Kumbasar, O, Overbeek, M, Otto Minasian, A, O'Riordan, D, Ora, J, Oldham, J, Okutan, O, Ohshimo, S, Oguzulgen, I, Ogura, T, O'Donnell, T, O'Dochartaigh, C, O'Beirne, S, Novikova, L, Novelli, L, Noth, I, Nogueira Mendes Neto, N, Niroumand, M, Nieto, A, Neves, A, Nambiar, A, Nair, S, Nadama, R, Murtagh, E, Mura, M, Muller Quernheim, J, Mukhopadhyay, A, Mukherjee, S, Morisset, J, Moran, O, Mooney, J, Moller, J, Mogulkoc, N, Miyamoto, A, Milenkovic, B, Mette, S, Mejia, M, Mei, F, Mazzei, M, Matsuda, T, Mason, C, Martinez Frances, M, Mannarino, S, Mancuzo, E, Malli, F, Malhotra, P, Maillo, M, Maia, J, Mahdavian, M, Madsen, F, Luckhardt, T, Lucht, W, Low, S, Lopez Miguel, C, Lipchik, R, Levy, S, Levin, K, Lee, K, Lederer, D, Lammi, M, Kwan, H, Kukreja, S, Kruavit, A, Kotecki, M, Kolilekas, L, Knoop, H, Kiyan, E, Kishaba, T, King Biggs, M, Khor, Y, Khan, A, Khalil, N, Kedia, R, Kebba, N, Kawano Dourado, L, Kapitan, K, Kan, C, Kalyoncu, A, Kalluri, M, Kabasakal, Y, Jyothula, S, Juretschke, M, Jovanovic, D, Jonkers, R, Jo, H, Izumi, S, Ishii, H, Ikeda, S, Ibrahim, A, Hyldgaard, C, Hunninghake, G, Huie, T, Hufton, A, Hu, X, Hseih, W, Hoyos, R, Hoyles, R, Holguin Rodriguez, O, Hogan, M, Hodgson, U, Hilkin Sogoloff, H, Herrera, E, Henry, B, Hellemons, M, Hecimovic, A, Hayashi, R, Hart, S, Harari, S, Haney, S, Hambly, N, Hakkim, R, Gutierrez, M, Gripaldo, R, Gomez, A, Goh, N, Godoy, R, Gilbert, C, Giannarakis, I, Gasparini, S, Garcha, P, Furtado, S, Fois, A, Flood Page, P, Fletcher, S, Fiss, E, Figueroa Casas, J, Figueroa Casas, M, Fiddler, C, Ferrara, G, Fernandez Casares, M, Felton, C, Faverio, P, Fabro, A, Estrada, A, Errhalt, P, Enomoto, N, Enghelmayer, J, El Kersh, K, Eiger, G, Dubaniewicz, A, Drakopanagiotakis, F, Disayabutr, S, Dijkstra, A, Diaz Patino, J, Diaz Castanon, J, Dhooria, S, Dhasmana, D, De Rosa, M, De Luca, S, Delobbe, A, Delgado, D, Delgado, C, De La Fuente, I, De Kruif, M, De Gier, M, De Andrade, J, Davidsen, J, Daoud, B, Dalhoff, K, Cotera Solano, J, Costa, A, Coronel, S, Confalonieri, M, Conemans, L, Comellas, A, Colella, S, Clemente, S, Clark, J, Ciuffreda, M, Chung, C, Chong, S, Chirita, D, Chen, P, Chaudhuri, N, Chambers, D, Chalmers, G, Chairman, D, Chai, G, Chacon Chaves, R, Cetinsu, V, Ceruti, M, Ceballos Zuniga, C, Castillo, D, Carbone, R, Caminati, A, Callejas Gonzalez, F, Butler, M, Bustos, C, Bukowczan, M, Buendia, I, Brunetti, G, Brockway, B, Bresser, P, Breseghello, J, Bouros, D, Botero Zaccour, J, Borzone, G, Borie, R, Blum, H, Blank, J, Biswas, A, Bennett, D, Benjamin, M, Belaconi, I, Beirne, P, Beckert, L, Bastiampillai, S, Bascom, R, Bartholmai, B, Barros, M, Ban, A, Balestro, E, Baldi, B, Baddini Martinez, J, Baburao, A, Babu, S, Averyanov, A, Avdeev, S, Athanazio, R, Atahan, E, Asuquo, B, Assayag, D, Antuni, J, Antillon, S, Anderson, K, Anderson, A, Alwani, F, Altinisik, G, Alsouofi, N, Allam, J, Al Jahdali, H, Al Farttoosi, A, Alfaro, T, Al Busaidi, N, Alavi Foumani, A, Agreda Vedia, M, Agarwal, A, Afridi, F, Adeyeye, O, Adegunsoye, A, Adamali, H, Abedini, A, and Pulmonary Medicine

Subjects
Male, Pediatrics, International Cooperation, Respiratory System, Hospitals, University, Idiopathic pulmonary fibrosis, 0302 clinical medicine, Cohen's kappa, Diagnosis, UK, 030212 general & internal medicine, Medical diagnosis, Referral and Consultation, Pulmonologists, Idiopathic Pulmonary Fibrosi, Interstitial lung disease, 11 Medical And Health Sciences, Middle Aged, respiratory system, Prognosis, Hospitals, humanities, Dimensional Measurement Accuracy, Clinical Competence, Diagnosis, Differential, Diagnostic Techniques, Respiratory System, Female, Humans, Idiopathic Pulmonary Fibrosis, Quality of Health Care, Reproducibility of Results, Human, Cohort study, Pulmonary and Respiratory Medicine, medicine.medical_specialty, Prognosi, education, MEDLINE, Reproducibility of Result, INTERSTITIAL PNEUMONIA, Interstitial Lung Diseases, 03 medical and health sciences, Internal medicine, PARENCHYMAL LUNG-DISEASE, MANAGEMENT, medicine, Idiopathic pulmonary fibrosis, diagnosis, Pulmonologist, University, business.industry, MORTALITY, Original Articles, medicine.disease, respiratory tract diseases, Diagnostic Techniques, IPF Project Consortium, 030228 respiratory system, Differential, INTEROBSERVER AGREEMENT, UPDATE, COOPERAÇÃO INTERNACIONAL, Differential diagnosis, business
Abstract

We conducted an international study of idiopathic pulmonary fibrosis (IPF) diagnosis among a large group of physicians and compared their diagnostic performance to a panel of IPF experts. A total of 1141 respiratory physicians and 34 IPF experts participated. Participants evaluated 60 cases of interstitial lung disease (ILD) without interdisciplinary consultation. Diagnostic agreement was measured using the weighted kappa coefficient (κw). Prognostic discrimination between IPF and other ILDs was used to validate diagnostic accuracy for first-choice diagnoses of IPF and were compared using the C-index. A total of 404 physicians completed the study. Agreement for IPF diagnosis was higher among expert physicians (κw=0.65, IQR 0.53–0.72, p20 years of experience (C-index=0.72, IQR 0.0–0.73, p=0.229) and non-university hospital physicians with more than 20 years of experience, attending weekly MDT meetings (C-index=0.72, IQR 0.70–0.72, p=0.052), did not differ significantly (p=0.229 and p=0.052 respectively) from the expert panel (C-index=0.74 IQR 0.72–0.75). Experienced respiratory physicians at university-based institutions diagnose IPF with similar prognostic accuracy to IPF experts. Regular MDT meeting attendance improves the prognostic accuracy of experienced non-university practitioners to levels achieved by IPF experts., Academic status, access to MDT meetings and clinician experience predict accuracy of a clinical diagnosis of IPF http://ow.ly/k43W30cTMg1

Published
2017

15. Kawasaki disease: guidelines of the Italian Society of Pediatrics, part I - definition, epidemiology, etiopathogenesis, clinical expression and management of the acute phase

Author

Marchesi, A, Tarissi de Jacobis, I, Rigante, Donato, Rimini, A, Malorni, W, Corsello, G, Bossi, G, Buonuomo, S, Cardinale, F, Cortis, E, De Benedetti, F, De Zorzi, A, Duse, M, Del Principe, D, Dellepiane, Rm, D’Isanto, L, El Hachem, M, Esposito, S, Falcini, F, Giordano, U, Maggio, Mc, Mannarino, S, Marseglia, G, Martino, S, Marucci, G, Massaro, R, Pescosolido, C, Pietraforte, D, Pietrogrande, Mc, Salice, P, Secinaro, A, Straface, E, Villani, A, Rigante D (ORCID:0000-0001-7032-7779), Marchesi, A, Tarissi de Jacobis, I, Rigante, Donato, Rimini, A, Malorni, W, Corsello, G, Bossi, G, Buonuomo, S, Cardinale, F, Cortis, E, De Benedetti, F, De Zorzi, A, Duse, M, Del Principe, D, Dellepiane, Rm, D’Isanto, L, El Hachem, M, Esposito, S, Falcini, F, Giordano, U, Maggio, Mc, Mannarino, S, Marseglia, G, Martino, S, Marucci, G, Massaro, R, Pescosolido, C, Pietraforte, D, Pietrogrande, Mc, Salice, P, Secinaro, A, Straface, E, Villani, A, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

The primary purpose of these practical guidelines related to Kawasaki disease (KD) is to contribute to prompt diagnosis and appropriate treatment on the basis of different specialists’ contributions in the field. A set of 40 recommendations is provided, divided in two parts: the first describes the definition of KD, its epidemiology, etiopathogenetic hints, presentation, clinical course and general management, including treatment of the acute phase, through specific 23 recommendations. Their application is aimed at improving the rate of treatment with intravenous immunoglobulin and the overall potential development of coronary artery abnormalities in KD. Guidelines, however, should not be considered a norm that limits treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient’s condition, and disease severity or complications.

Published
2018

16. Kawasaki disease: guidelines of the Italian Society of Pediatrics, part II - treatment of resistant forms and cardiovascular complications, follow-up, lifestyle and prevention of cardiovascular risks

Author

Marchesi, A, Tarissi de Jacobis, I, Rigante, Donato, Rimini, A, Malorni, W, Corsello, G, Bossi, G, Buonuomo, S, Cardinale, F, Cortis, E, De Benedetti, F, De Zorzi, A, Duse, M, Del Principe, D, Dellepiane, Rm, D’Isanto, L, El Hachem, M, Esposito, S, Falcini, F, Giordano, U, Maggio, Mc, Mannarino, S, Marseglia, G, Martino, S, Marucci, G, Massaro, R, Pescosolido, C, Pietraforte, D, Pietrogrande, Mc, Salice, P, Secinaro, A, Straface, E, Villani, A, Rigante D (ORCID:0000-0001-7032-7779), Marchesi, A, Tarissi de Jacobis, I, Rigante, Donato, Rimini, A, Malorni, W, Corsello, G, Bossi, G, Buonuomo, S, Cardinale, F, Cortis, E, De Benedetti, F, De Zorzi, A, Duse, M, Del Principe, D, Dellepiane, Rm, D’Isanto, L, El Hachem, M, Esposito, S, Falcini, F, Giordano, U, Maggio, Mc, Mannarino, S, Marseglia, G, Martino, S, Marucci, G, Massaro, R, Pescosolido, C, Pietraforte, D, Pietrogrande, Mc, Salice, P, Secinaro, A, Straface, E, Villani, A, and Rigante D (ORCID:0000-0001-7032-7779)

Abstract

This second part of practical Guidelines related to Kawasaki disease (KD) has the goal of contributing to prompt diagnosis and most appropriate treatment of KD resistant forms and cardiovascular complications, including non-pharmacologic treatments, follow-up, lifestyle and prevention of cardiovascular risks in the long-term through a set of 17 recommendations. Guidelines, however, should not be considered a norm that limits the treatment options of pediatricians and practitioners, as treatment modalities other than those recommended may be required as a result of peculiar medical circumstances, patient’s condition, and disease severity or individual complications.

Published
2018

17. Prevalence of the congenital long-qt syndrome

Author

Schwartz Peter, J, Stramba-Badiale, M, Crotti, L, Pedrazzini, M, Besana, A, Bosi, G, Gabbarini, F, Goulene, K, Insolia, R, Mannarino, S, Mosca, F, Nespoli, L, Rimini, A, Rosati, E, Salice, P, Spazzolini, C, Schwartz Peter John, Stramba-Badiale M, Crotti Lia, Pedrazzini M, Besana A, Bosi G, Gabbarini F, Goulene K, Insolia Roberto, Mannarino S, Mosca F, Nespoli L, Rimini A, Rosati E, Salice P, Spazzolini C, Schwartz Peter, J, Stramba-Badiale, M, Crotti, L, Pedrazzini, M, Besana, A, Bosi, G, Gabbarini, F, Goulene, K, Insolia, R, Mannarino, S, Mosca, F, Nespoli, L, Rimini, A, Rosati, E, Salice, P, Spazzolini, C, Schwartz Peter John, Stramba-Badiale M, Crotti Lia, Pedrazzini M, Besana A, Bosi G, Gabbarini F, Goulene K, Insolia Roberto, Mannarino S, Mosca F, Nespoli L, Rimini A, Rosati E, Salice P, and Spazzolini C

Abstract

BACKGROUND-: The prevalence of genetic arrhythmogenic diseases is unknown. For the long-QT syndrome (LQTS), figures ranging from 1:20 000 to 1:5000 were published, but none was based on actual data. Our objective was to define the prevalence of LQTS. METHODS AND RESULTS-: In 18 maternity hospitals, an ECG was performed in 44 596 infants 15 to 25 days old (43 080 whites). In infants with a corrected QT interval (QTc) >450 ms, the ECG was repeated within 1 to 2 weeks. Genetic analysis, by screening 7 LQTS genes, was performed in 28 of 31 (90%) and in 14 of 28 infants (50%) with, respectively, a QTc >470 ms or between 461 and 470 ms. A QTc of 451 to 460, 461 to 470, and >470 ms was observed in 177 (0.41%), 28 (0.06%), and 31 infants (0.07%). Among genotyped infants, disease-causing mutations were found in 12 of 28 (43%) with a QTc >470 ms and in 4 of 14 (29%) with a QTc of 461 to 470 ms. One genotype-negative infant (QTc 482 ms) was diagnosed as affected by LQTS on clinical grounds. Among family members of genotype-positive infants, 51% were found to carry disease-causing mutations. In total, 17 of 43 080 white infants were affected by LQTS, demonstrating a prevalence of at least 1:2534 apparently healthy live births (95% confidence interval, 1:1583 to 1:4350). CONCLUSIONS-: This study provides the first data-based estimate of the prevalence of LQTS among whites. On the basis of the nongenotyped infants with QTc between 451 and 470 ms, we advance the hypothesis that this prevalence might be close to 1:2000. ECG-guided molecular screening can identify most infants affected by LQTS and unmask affected relatives, thus allowing effective preventive measures

Published
2009

19. [In Process Citation]

Author

RUSSO, Maria Giovanna, FESSLOVA V, CERINI E, SADOU Y, PISACANE C, CALABRO MP, MANNARINO S., Russo, Maria Giovanna, Fesslova, V, Cerini, E, Sadou, Y, Pisacane, C, Calabro, Mp, and Mannarino, S.

Published
2003

24. EP18.06: Congenital heart defects in monochorionic twins complicated by selective fetal growth restriction.

Author

Faiola, S., Casati, D., Laoreti, A., Zambon, M., Corti, C., Mannarino, S., Lanna, M., and Cetin, I.

Abstract

To evaluate prevalence, types and postnatal follow-up of congenital heart disease (CHD) in a cohort of monochorionic-diamniotic (MC/DA) twin pregnancies complicated by selective fetal growth restriction (sFRG) without Twin-twin transfusion syndrome (TTTS). Conclusions In MC/DA twin pregnancies without TTTS, sFGR increases 3 times the risk of CHD. The higher risk of CHD in MC/DA pregnancies appears not to be due to MC itself but to the typical complications of MC pregnancies as sFGR and TTTS. [Extracted from the article]

Published
2022
Full Text
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27. Prevalence of the congenital long-QT syndrome.

Author

Schwartz PJ, Stramba-Badiale M, Crotti L, Pedrazzini M, Besana A, Bosi G, Gabbarini F, Goulene K, Insolia R, Mannarino S, Mosca F, Nespoli L, Rimini A, Rosati E, Salice P, Spazzolini C, Schwartz, Peter J, Stramba-Badiale, Marco, Crotti, Lia, and Pedrazzini, Matteo

Published
2009
Full Text
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28. Neonatal lupus: fetal myocarditis progressing to atrioventricular block in triplets.

Author

Fessolova, V., Mannarino, S., Salice, P., Boschetto, C., Trespidi, L., Acaia, B., Mosca, F., Cimaz, R., and Meroni, P. L.

Subjects
*FETAL diseases, *LUPUS erythematosus, *MYOCARDITIS, *TRIPLETS, *FERTILIZATION in vitro
Abstract

We report a case of neonatal lupus syndrome (NLS) in an in vitro fertilization induced triplet pregnancy. Echocardiographic signs of myocarditis were evident at the 21st week of gestation (w.g.) in twin I, with a subsequent development of a complete atrioventricular (AV) block at the 25th w.g.; twin III also displayed echocardiographic signs of myocarditis at the same time. Treatment with dexamethasone (4mg/day) was started at the 25th w.g. A complete echocardiographic regression of the myocarditis signs was achieved, while AV block was unaffected. Caesarian section was performed at the 31.5 w.g. after a premature rupture of the membranes. Complete AV block was confirmed in twin I with a heart rate of 51 beats/min that required a pacemaker implant 40 days after. Twin III developed a first-degree AV block that switched to a periodic second-degree block later, while twin II displayed only liver enzyme abnormalities. [ABSTRACT FROM AUTHOR]

Published
2003

31. VARIATION OF B-TYPE NATRIURETIC PEPTIDE CONCENTRATIONS AND INTRAUTERINE GROWTH RESTRICTION: MOTHER, FETUS AND NEWBORN

Author

Garofoli, F., Mannarino, S., Montanari, L., Cerbo, R., Tzialla, C., Mazzucchelli, I., Angelini, M., Codazzi, A. C., Mongini, M. E., Manzoni, P., Tinelli, C., Arsenio Spinillo, and Stronati, M.

Subjects
Fetal Growth Retardation, Echocardiography, Pregnancy, Natriuretic Peptide, Brain, Infant, Newborn, Humans, Female, Fetal Blood, Ultrasonography, Prenatal
Abstract

To evaluate maternal, fetal, neonatal B-type natriuretic peptide (BNP) concentrations related to Intra Uterine Growth Restriction (IUGR). BNP concentrations in 43 IUGR and 35 healthy, Appropriate for Gestational Age (AGA) infants/paired mothers have been compared, from delivery/birth to first month of life. Maternal and IUGR cord BNP concentrations were coupled to fetal ultrasonography. Neonatal echocardiography was performed too. On delivery BNP was higher in all IUGR mothers, suffering or not from gestational hypertension, than in AGA (median 37.14 vs 11.1 pg/ml p=0.002). Maternal BNP was not associated to cord/neonatal BNP or fetal ultrasonographic parameters. Cord BNP was higher in IUGR than AGA newborns (median 23.9 vs 11.4 pg/ml p=0.0007) independently of gestational age, while varied with amniotic fluid (p=0.0044) and umbilical artery flowmetry (p=0.0121). Earlier drop of BNP on day 3 was reported in IUGR neonates (p=0.0001).Ventricular mass change/body weight varied positively in AGA newborns (p0.001), while declined in IUGR ones (p=0.003). Carrying IUGR fetus is a stress factor resulting in high maternal BNP concentration. Altered fetal ultrasonographic parameters in IUGR newborns lead to higher BNP cord levels. A rapid BNP drop and probable ventricular mass adjustment of IUGR newborns may indicate earlier post-natal cardiovascular adaptation than AGA infants.

40. Defining Kawasaki disease and pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection during SARS-CoV-2 epidemic in Italy: results from a national, multicenter survey

Author

Cattalini M, Della Paolera S, Zunica F, Bracaglia C, Giangreco M, Verdoni L, Meini A, Sottile R, Caorsi R, Zuccotti G, Fabi M, Montin D, Meneghel A, Consolaro A, Dellepiane RM, Maggio MC, La Torre F, Marchesi A, Simonini G, Villani A, Cimaz R, Ravelli A, Taddio A Maria Concetta Alberelli: UOC Pediatria, Marche-Nord, Clotilde Alizzi: Department of Health Promotion Sciences Maternal and Infantile Care, Internal Medicine and Medical Specialities 'G. D’Alessandro', University of Palermo, Palermo Italy, Patrizia Barone: Unità Operativa Complessa di Broncopneumologia Pediatrica AOU 'Policlinico - Vittorio Emanuele Via Santa Sofia 78 Catania, Lucia Augusta Baselli: Pediatric Intermediate Care Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore Policlinico, Milan Italy, Veronica Bennato: U. O. Pediatria, Ospedale A, Manzoni Lecco, Francesca Biscaro: UOC Pediatria, Ospedale Ca’ Foncello, Treviso, Grazia Bossi: UOC Pediatria, Fondazione IRCCS Policlinico San Matteo, Pavia Italy, Andrea Campana: Bambino Gesù Children’s Hospital, Rome Italy, Maurizio Carone: UO Malattie Infettive, Ospedale Pediatrico ‘Giovanni XXIII’, Bari Italy, Adele Civino: U. O. C. Pediatria P. O. Vito Fazzi, Lecce, Giovanni Conti: Nefrologia e Reumatologia Pediatrica con Dialisi, Azienda Ospedaliero-Universitario 'G. Martino', Eleonora Dei Rossi: University of Trieste, Trieste Italy, Emanuela Del Giudice: Department of Maternal Infantile and Urological Sciences, Sapeinza University of Rome, Polo Pontini, Alice Dell’Anna: U. O. C. Pediatria P. O. Vito Fazzi Lecce, Maia De Luca: Bambino Gesù Children’s Hospital, Piazza S. Onofrio n. 4, 00165 Rome, Italy, Enrico Felici: Pediatric and Pediatric Emergency Unit, The Children Hospital, AO SS Antonio e Biago e C. Arrigo, Alessandria Italy, Giovanni Filocamo: Fondazione IRCCS Cà Granda, Ospedale Maggiore Policlinico, Milano, Ilenia Floretta: Pediatria, Ospedale Santa Chiara, Trento Italy, Maria Loreta Foschini: SC Pediatria, PO SAN MICHELE AOBrotzu, Cagliari Italy, Marcello Lanari: Department of Pediatrics, University of Bologna, IRCCS S. Orsola-Malpighi Hospital, Bologna Italy, Bianca Lattanzi: SOD Pediatria, Ospedali Riuniti, Ancona Italy, Alessandra Lazzerotti: Clinica Pediatrica, Università Milano Bicocca, Fondazione MBBM - onlus c/o Ospedale San Gerardo, Monza Italy, Francesco Licciardi: Department of Pediatrics and Public Health, University of Turin, Turin Italy, Alessandra Manerba: Child Cardiology, ASST Spedali Civili di Brescia and University of Brescia, Brescia Italy, Savina Mannarino: Division of Cardiology, Children’s Hospital V Buzzi, ASST FBF Sacco, Achille Marino: Department of Pediatrics, Desio Hospital, ASST Monza, Desio Italy, Agostina Marolda: Pediatrics and Neonatology Dipartment, ASST Ovest Milanese, 'G. Fornaroli' Hospital, Magenta Milan, Laura Martelli: Paediatric Department, Hospital Papa Giovanni XXIII, Bergamo Italy, Giorgia Martini, Department of Woman’s and Child’s Health, University of Padova, Padua Italy, Angela Mauro: Department of Paediatrics, Emergency Department, Santobono-Pausilipon Children’s Hospital, Naples, Italy. Maria Vincenza Mastrolia: Pediatric Rheumatology Unit, AOU Meyer, University of Florence, Florence, Italy. Angelo Mazza: Paediatric Department, Angela Miniaci: Clinica Pediatrica, Reumatologia, Azienda Ospedaliero-Universitaria di Bologna, Francesca Minoia: Fondazione IRCCS Cà Granda, Alma Olivieri: Dipartimento della donna, del bambino e di chirurgia generale e specialistica, Università della Campania, 'L Vanvitelli, Napoli, Guido Pennoni: Dipartimento Materno-Infantile, Gubbio-Gualdo Tadino, Italy, Rossana Pignataro: UOC Pediatria e Neonatologia, ASST Lodi, Lodi, Francesca Ricci, Clinica Pediatrica, ASST Spedali Civili di Brescia e Università degli Studi di Brescia, Donato Rigante: Department of Pediatrics, Univarsità Cattolica Sacro Cuore, Matilde Rossi: UOC di Pediatrai e Neonatologia, Ospedale di Macerata, Macerata, Claudia Santagati: Dipartimento di Pediatria, Ospedale di Rovigo, Rovigo, Martina Soliani: Pediatria ASST Cremona, Italy, Silvia Sonego: University of Trieste, Domenico Sperlì: UOC di Pediatria, S. O. 'Annunziata' - A. O. di Cosenza, Sara Stucchi: Maternal and Child Health, Division of Paediatrics, ASST Grande Ospedale Metropolitano Niguarda, Milano Italy, Barbara Teruzzi: Maternal and Child Health, Elpidio Tierno: UOC di Pediatria, Dipartimento della Salute della Donna e del Bambin, AORN 'Sant’Anna e San Sebastiano'- Caserta, Tatiana Utytatnikova: Dipartimento Materno-Infantile, Pediatria, ASST Bergamo-EST, Seriate Bergamo, Piero Valentini, Department of Pediatrics, Gianluca Vergine, UOC Pediatria Rimini, Ospedale Infermi, ASL Romagna, Rimini Italy., Cattalini, Marco, Della Paolera, Sara, Zunica, Fiammetta, Bracaglia, Claudia, Giangreco, Manuela, Verdoni, Lucio, Meini, Antonella, Sottile, Rita, Caorsi, Roberta, Zuccotti, Gianvincenzo, Fabi, Marianna, Montin, Davide, Meneghel, Alessandra, Consolaro, Alessandro, Dellepiane, Rosa Maria, Maggio, Maria Cristina, La Torre, Francesco, Marchesi, Alessandra, Simonini, Gabriele, Villani, Alberto, Cimaz, Rolando, Ravelli, Angelo, Taddio, Andrea, Cattalini, M, Della Paolera, S, Zunica, F, Bracaglia, C, Giangreco, M, Verdoni, L, Meini, A, Sottile, R, Caorsi, R, Zuccotti, G, Fabi, M, Montin, D, Meneghel, A, Consolaro, A, Dellepiane, Rm, Maggio, Mc, La Torre, F, Marchesi, A, Simonini, G, Villani, A, Cimaz, R, Ravelli, A, Taddio, A Maria Concetta Alberelli: UOC Pediatria, Marche-Nord, Clotilde Alizzi: Department of Health Promotion Sciences Maternal and Infantile, Care, Internal Medicine and Medical Specialities 'G., D’Alessandro', University of, Palermo, Palermo, Italy, Patrizia Barone: Unità Operativa Complessa di Broncopneumologia Pediatrica AOU 'Policlinico, - Vittorio Emanuele Via Santa Sofia 78 Catania, Lucia Augusta Baselli: Pediatric Intermediate Care, Unit, Fondazione IRCCS Ca’ Granda Ospedale Maggiore, Policlinico, Milan, Italy, Veronica Bennato: U. O., Pediatria, Ospedale, A, Manzoni, Lecco, Francesca Biscaro: UOC, Pediatria, Ospedale Ca’, Foncello, Treviso, Grazia Bossi: UOC, Pediatria, Fondazione IRCCS Policlinico San, Matteo, Pavia, Italy, Andrea Campana: Bambino Gesù Children’s, Hospital, Rome, Italy, Maurizio Carone: UO Malattie, Infettive, Ospedale Pediatrico ‘Giovanni, Xxiii’, Bari, Italy, Adele Civino: U. O. C. Pediatria P. O. Vito Fazzi, Lecce, Giovanni Conti: Nefrologia e Reumatologia Pediatrica con Dialisi, Azienda Ospedaliero-Universitario 'G. Martino', Eleonora Dei Rossi: University of, Trieste, Trieste, Italy, Emanuela Del Giudice: Department of Maternal Infantile and Urological, Science, Sapeinza University of, Rome, Polo, Pontini, Alice Dell’Anna: U. O. C. Pediatria P. O., Vito Fazzi Lecce, Maia De Luca: Bambino Gesù Children’s, Hospital, Piazza, S. Onofrio n. 4., 00165, Rome, Italy, Enrico Felici: Pediatric and Pediatric Emergency, Unit, The Children, Hospital, AO SS Antonio e Biago e C., Arrigo, Alessandria, Italy, Giovanni Filocamo: Fondazione IRCCS Cà, Granda, Ospedale Maggiore, Policlinico, Milano, Ilenia Floretta:, Pediatria, Ospedale Santa, Chiara, Trento, Italy, Maria Loreta Foschini: SC, Pediatria, PO SAN MICHELE, Aobrotzu, Cagliari, Italy, Marcello Lanari: Department of, Pediatric, University of, Bologna, IRCCS S., Orsola-Malpighi Hospital, Bologna, Italy, Bianca Lattanzi: SOD, Pediatria, Ospedali, Riuniti, Ancona, Italy, Alessandra Lazzerotti: Clinica, Pediatrica, Università Milano, Bicocca, Fondazione MBBM, - onlus c/o Ospedale San Gerardo, Monza, Italy, Francesco Licciardi: Department of Pediatrics and Public, Health, University of, Turin, Turin, Italy, Alessandra Manerba: Child, Cardiology, ASST Spedali Civili di Brescia and University of, Brescia, Brescia, Italy, Savina Mannarino: Division of, Cardiology, Children’s Hospital, V Buzzi, ASST FBF, Sacco, Achille Marino: Department of, Pediatric, Desio, Hospital, Asst, Monza, Desio, Italy, Agostina Marolda: Pediatrics and Neonatology, Dipartment, ASST Ovest, Milanese, 'G., Fornaroli' Hospital, Magenta, Milan, Laura Martelli: Paediatric, Department, Hospital Papa Giovanni, Xxiii, Bergamo, Italy, Giorgia, Martini, Department of Woman’s and Child’s, Health, University of, Padova, Padua, Italy, Angela Mauro: Department of, Paediatric, Emergency, Department, Santobono-Pausilipon Children’s, Hospital, Naples, Italy., Maria Vincenza Mastrolia: Pediatric Rheumatology Unit, Aou, Meyer, University of, Florence, Florence, Italy., Angelo Mazza: Paediatric Department, Angela Miniaci: Clinica, Pediatrica, Reumatologia, Azienda Ospedaliero-Universitaria di, Bologna, Francesca Minoia: Fondazione IRCCS Cà, Granda, Alma Olivieri: Dipartimento della, Donna, del bambino e di chirurgia generale, e specialistica, Università della, Campania, 'L, Vanvitelli, Napoli, Claudio, Guido Pennoni: Dipartimento, Materno-Infantile, Gubbio-Gualdo Tadino, Italy, Rossana Pignataro: UOC Pediatria, e Neonatologia, Asst, Lodi, Lodi, Francesca, Ricci, Clinica, Pediatrica, ASST Spedali Civili di Brescia, e Università degli Studi di Brescia, Donato Rigante: Department of, Pediatric, Univarsità Cattolica Sacro, Cuore, Matilde Rossi: UOC di Pediatrai, e Neonatologia, Ospedale di, Macerata, Macerata, Claudia Santagati: Dipartimento di, Pediatria, Ospedale di, Rovigo, Rovigo, Martina Soliani: Pediatria ASST Cremona, Italy, Silvia Sonego: University of, Trieste, Domenico Sperlì: UOC di Pediatria, S. O. 'Annunziata' - A. O. di Cosenza, Sara Stucchi: Maternal and Child, Health, Division of, Paediatric, ASST Grande Ospedale Metropolitano, Niguarda, Milano, Italy, Barbara Teruzzi: Maternal and Child, Health, Elpidio Tierno: UOC di, Pediatria, Dipartimento della Salute della Donna, e del Bambin, AORN 'Sant’Anna, e San Sebastiano'- Caserta, Tatiana Utytatnikova: Dipartimento, Materno-Infantile, Pediatria, Asst, Bergamo-EST, Seriate, Bergamo, Piero, Valentini, Department of, Pediatric, Gianluca, Vergine, UOC Pediatria, Rimini, Ospedale, Infermi, Asl, Romagna, Rimini, Italy., Cattalini M., Della Paolera S., Zunica F., Bracaglia C., Giangreco M., Verdoni L., Meini A., Sottile R., Caorsi R., Zuccotti G., Fabi M., Montin D., Meneghel A., Consolaro A., Dellepiane R.M., Maggio M.C., La Torre F., Marchesi A., Simonini G., Villani A., Cimaz R., Ravelli A., Taddio A., Adamoli P., Alberelli M.C., Alizzi C., Barone P., Bennato V., Biscaro F., Bossi G., Campana A., Carone M., Civino A., Conti G., Rossi E.D., Del Giudice E., Dell'Anna A., De Luca M., Felici E., Filocamo G., Floretta I., Foschini M.L., Lanari M., Lattanzi B., Lazzerotti A., Licciardi F., Manerba A., Mannarino S., Marino A., Marolda A., Martelli L., Martini G., Mauro A., Mastrolia M.V., Mazza A., Miniaci A., Minoia F., Olivieri A., Pennoni G., Pignataro R., Ricci F., Rigante D., Rossi M., Santagati C., Soliani M., Sonego S., Sperli D., Stucchi S., Teruzzi B., Tierno E., Utytatnikova T., Valentini P., and Vergine G.

Subjects
Coronary artery abnormalities, Hypotension, Kawasaki disease, Multisystem inflammatory syndrome associated with coronavirus disease, Myocarditis, Pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection, SARS-CoV-2, Age Distribution, Antirheumatic Agents, Aspirin, C-Reactive Protein, COVID-19, Child, Child, Preschool, Coronary Artery Disease, Cough, Diarrhea, Dyspnea, Female, Glucocorticoids, Heart Failure, Humans, Hyperferritinemia, Immunoglobulins, Intravenous, Immunologic Factors, Infant, Intensive Care Units, Pediatric, Interleukin 1 Receptor Antagonist Protein, Italy, Lymphopenia, Male, Mucocutaneous Lymph Node Syndrome, Platelet Aggregation Inhibitors, Shock, Systemic Inflammatory Response Syndrome, Tachypnea, Troponin T, Vomiting, lcsh:Diseases of the musculoskeletal system, coronary artery abnormalities, hypotension, kawasaki disease, multisystem inflammatory syndrome associated with coronavirus disease, myocarditis, pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection, age distribution, antirheumatic agents, aspirin, C-reactive protein, child, preschool, coronary artery disease, cough, diarrhea, yspnea, female, glucocorticoids, heart failure, humans, hyperferritinemia, immunoglobulins, intravenous, immunologic factors, infant, intensive care units, pediatric, interleukin 1 receptor antagonist protein, italy, lymphopenia, male, mucocutaneous lymph node syndrome, platelet aggregation inhibitors, shock, systemic inflammatory response syndrome, tachypnea, troponin T, vomiting, Myocarditi, 030204 cardiovascular system & hematology, SARS-CoV-2, Kawasaki disease, Pediatric inflammatory multisystem syndrome-temporally associated to SARS-CoV-2 infection, Myocarditis, Hypotension, Multisystem inflammatory syndrome associated with coronavirus disease, Coronary artery abnormalities, Coronary artery disease, Settore MED/38 - Pediatria Generale E Specialistica, 0302 clinical medicine, Glucocorticoid, Immunologic Factor, Immunology and Allergy, Coronary artery abnormalitie, Fisher's exact test, Pediatric, biology, lcsh:RJ1-570, Antirheumatic Agent, Settore MED/38, Intensive Care Units, Cohort, symbols, Platelet aggregation inhibitor, Intravenous, Human, Research Article, medicine.medical_specialty, Immunoglobulins, 03 medical and health sciences, symbols.namesake, Rheumatology, Internal medicine, medicine, Preschool, 030203 arthritis & rheumatology, business.industry, Platelet Aggregation Inhibitor, lcsh:Pediatrics, medicine.disease, Systemic inflammatory response syndrome, Immunoglobulins, Intravenou, Pediatrics, Perinatology and Child Health, biology.protein, lcsh:RC925-935, business
Abstract

Background There is mounting evidence on the existence of a Pediatric Inflammatory Multisystem Syndrome-temporally associated to SARS-CoV-2 infection (PIMS-TS), sharing similarities with Kawasaki Disease (KD). The main outcome of the study were to better characterize the clinical features and the treatment response of PIMS-TS and to explore its relationship with KD determining whether KD and PIMS are two distinct entities. Methods The Rheumatology Study Group of the Italian Pediatric Society launched a survey to enroll patients diagnosed with KD (Kawasaki Disease Group – KDG) or KD-like (Kawacovid Group - KCG) disease between February 1st 2020, and May 31st 2020. Demographic, clinical, laboratory data, treatment information, and patients’ outcome were collected in an online anonymized database (RedCAP®). Relationship between clinical presentation and SARS-CoV-2 infection was also taken into account. Moreover, clinical characteristics of KDG during SARS-CoV-2 epidemic (KDG-CoV2) were compared to Kawasaki Disease patients (KDG-Historical) seen in three different Italian tertiary pediatric hospitals (Institute for Maternal and Child Health, IRCCS “Burlo Garofolo”, Trieste; AOU Meyer, Florence; IRCCS Istituto Giannina Gaslini, Genoa) from January 1st 2000 to December 31st 2019. Chi square test or exact Fisher test and non-parametric Wilcoxon Mann-Whitney test were used to study differences between two groups. Results One-hundred-forty-nine cases were enrolled, (96 KDG and 53 KCG). KCG children were significantly older and presented more frequently from gastrointestinal and respiratory involvement. Cardiac involvement was more common in KCG, with 60,4% of patients with myocarditis. 37,8% of patients among KCG presented hypotension/non-cardiogenic shock. Coronary artery abnormalities (CAA) were more common in the KDG. The risk of ICU admission were higher in KCG. Lymphopenia, higher CRP levels, elevated ferritin and troponin-T characterized KCG. KDG received more frequently immunoglobulins (IVIG) and acetylsalicylic acid (ASA) (81,3% vs 66%; p = 0.04 and 71,9% vs 43,4%; p = 0.001 respectively) as KCG more often received glucocorticoids (56,6% vs 14,6%; p Conclusion Our study suggests that SARS-CoV-2 infection might determine two distinct inflammatory diseases in children: KD and PIMS-TS. Older age at onset and clinical peculiarities like the occurrence of myocarditis characterize this multi-inflammatory syndrome. Our patients had an optimal response to treatments and a good outcome, with few complications and no deaths.

Published
2020

41. Magnesium sulfate as a vehicle for nebulized salbutamol in acute asthma.

Author

Nannini Jr., Luis J., Pendino, Juan C., Nannini, L J Jr, Pendino, J C, Corna, R A, Mannarino, S, and Quispe, R

Subjects
*MAGNESIUM sulfate, *ASTHMA treatment, *PHYSIOLOGY
Abstract

Purpose: Magnesium sulfate is thought to be an effective bronchodilator when administered intravenously to patients with acute severe asthma, and it can be safely administered via inhalation to patients with stable asthma. Our goal was to determine if isotonic magnesium sulfate could be used as a vehicle for nebulized salbutamol for patients with acute asthma.Methods: We enrolled 35 patients with acute asthma in a randomized, double-blind, controlled trial. After measurement of peak expiratory flow, patients received 2.5 mg salbutamol plus either 3 mL normal saline solution (n = 16) or isotonic magnesium sulfate (n = 19) through a jet nebulizer. Peak flow was reassessed 10 and 20 minutes after treatment.Results: Peak flow at baseline was similar in the two groups. Ten minutes after baseline, the mean (+/- SD) percentage increase in peak flow was greater in the magnesium sulfate-salbutamol group (61% +/- 45%) than in the normal saline-salbutamol group (31% +/- 28%; difference = 30%; 95% confidence interval [CI] for the difference: 3% to 56%; P = 0.03). At 20 minutes, the percentage increase in peak flow was 57% greater in the magnesium sulfate group (95% CI: 4% to 110%, P = 0.04). There was a significant inverse correlation between baseline peak flow (percent of predicted) and the percentage increase in peak flow at 20 minutes in the magnesium sulfate group (r = -0.82, P <0.0001), but not in the saline group (r = -0.12, P = 0.67).Conclusion: In patients with acute asthma, isotonic magnesium sulfate, as a vehicle for nebulized salbutamol, increased the peak flow response to treatment in comparison with salbutamol plus normal saline. [ABSTRACT FROM AUTHOR]

Published
2000
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42. Prevalence of the Congenital Long-QT Syndrome

Author

Patrizia Salice, Luigi Nespoli, Karine Goulene, Peter J. Schwartz, Savina Mannarino, Roberto Insolia, Marco Stramba-Badiale, Alessandra Besana, Alessandro Rimini, Carla Spazzolini, Giuliano Bosi, Lia Crotti, Matteo Pedrazzini, Fulvio Gabbarini, Enrico Rosati, Fabio Mosca, Schwartz Peter, J, Stramba-Badiale, M, Crotti, L, Pedrazzini, M, Besana, A, Bosi, G, Gabbarini, F, Goulene, K, Insolia, R, Mannarino, S, Mosca, F, Nespoli, L, Rimini, A, Rosati, E, Salice, P, and Spazzolini, C

Subjects
congenital, hereditary, and neonatal diseases and abnormalities, Pediatrics, medicine.medical_specialty, Genotype, Heart disease, Heart block, Long QT syndrome, DNA Mutational Analysis, Prevalence, QT interval, Article, Electrocardiography, long qt syndrome, prevalence, genetics, ALTE, Physiology (medical), SIDS, Epidemiology, Humans, Mass Screening, Medicine, Prospective Studies, cardiovascular diseases, Prospective cohort study, Mass screening, Family Health, business.industry, Infant, Newborn, MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, long-QT syndrome, newborn ECG, medicine.disease, Long QT Syndrome, Mutation, Cardiology and Cardiovascular Medicine, business
Abstract

Background— The prevalence of genetic arrhythmogenic diseases is unknown. For the long-QT syndrome (LQTS), figures ranging from 1:20 000 to 1:5000 were published, but none was based on actual data. Our objective was to define the prevalence of LQTS. Methods and Results— In 18 maternity hospitals, an ECG was performed in 44 596 infants 15 to 25 days old (43 080 whites). In infants with a corrected QT interval (QTc) >450 ms, the ECG was repeated within 1 to 2 weeks. Genetic analysis, by screening 7 LQTS genes, was performed in 28 of 31 (90%) and in 14 of 28 infants (50%) with, respectively, a QTc >470 ms or between 461 and 470 ms. A QTc of 451 to 460, 461 to 470, and >470 ms was observed in 177 (0.41%), 28 (0.06%), and 31 infants (0.07%). Among genotyped infants, disease-causing mutations were found in 12 of 28 (43%) with a QTc >470 ms and in 4 of 14 (29%) with a QTc of 461 to 470 ms. One genotype-negative infant (QTc 482 ms) was diagnosed as affected by LQTS on clinical grounds. Among family members of genotype-positive infants, 51% were found to carry disease-causing mutations. In total, 17 of 43 080 white infants were affected by LQTS, demonstrating a prevalence of at least 1:2534 apparently healthy live births (95% confidence interval, 1:1583 to 1:4350). Conclusions— This study provides the first data-based estimate of the prevalence of LQTS among whites. On the basis of the nongenotyped infants with QTc between 451 and 470 ms, we advance the hypothesis that this prevalence might be close to 1:2000. ECG-guided molecular screening can identify most infants affected by LQTS and unmask affected relatives, thus allowing effective preventive measures.

Published
2009

43. Long-Term Outcome and Risk Stratification in Dilated Cardiolaminopathies

Author

Alessandra Serio, Andrea Mortara, Mario Viganò, Carlo Campana, Giovanni Piccolo, Nicola Marziliano, Fabiana Isabella Gambarin, Maurizia Grasso, Andrea Pilotto, Michele Pasotti, Eloisa Arbustini, Catherine Klersy, Manuela Agozzino, Maurizio Landolina, Savina Mannarino, Claudio Rapezzi, Oreste Febo, Massimiliano Marini, Antonello Gavazzi, Valentina Favalli, Luigi Tavazzi, Pasotti M, Klersy C, Pilotto A, Marziliano N, Rapezzi C, Serio A, Mannarino S, Gambarin F, Favalli V, Grasso M, Agozzino M, Campana C, Gavazzi A, Febo O, Marini M, Landolina M, Mortara A, Piccolo G, Viganò M, Tavazzi L, and Arbustini E

Subjects
Adult, Cardiomyopathy, Dilated, Male, medicine.medical_specialty, LMNA gene mutation, Gene mutation, Ventricular tachycardia, Article, Sudden cardiac death, LMNA, Risk Factors, Internal medicine, Idiopathic dilated cardiomyopathy, atrioventricular block, medicine, Humans, cardiovascular diseases, Aged, Fibrillation, business.industry, Middle Aged, medicine.disease, Lamin Type A, Prognosis, idiopathic dilated cardiomyopathy, Phenotype, Heart failure, Mutation, Cardiology, cardiovascular system, Female, medicine.symptom, business, Cardiology and Cardiovascular Medicine, Atrioventricular block, Follow-Up Studies
Abstract

Objectives The aim of this study was to analyze the long-term follow-up of dilated cardiolaminopathies. Background Lamin A/C (LMNA) gene mutations cause a variety of phenotypes. In the cardiology setting, patients diagnosed with idiopathic dilated cardiomyopathy (DCM) plus atrioventricular block (AVB) constitute the majority of reported cases. Methods Longitudinal retrospective observational studies were conducted with 27 consecutive families in which LMNA gene defects were identified in the probands, all sharing the DCM phenotype. Results Of the 164 family members, 94 had LMNA gene mutations. Sixty of 94 (64%) were phenotypically affected whereas 34 were only genotypically affected, including 5 with pre-clinical signs. Of the 60 patients, 40 had DCM with AVB, 12 had DCM with ventricular tachycardia/fibrillation, 6 had DCM with AVB and Emery-Dreifuss muscular dystrophy type 2 (EDMD2), and 2 had AVB plus EDMD2. During a median of 57 months (interquartile range 36 to 107 months), we observed 49 events in 43 DCM patients (6 had a later event, excluded from the analysis). The events were related to heart failure (15 heart transplants, 1 death from end-stage heart failure) and ventricular arrhythmias (15 sudden cardiac deaths and 12 appropriate implantable cardioverter-defibrillator interventions). By multivariable analysis, New York Heart Association functional class III to IV and highly dynamic competitive sports for ≥10 years were independent predictors of total events. By a bivariable Cox model, splice site mutations and competitive sport predicted sudden cardiac death. Conclusions Dilated cardiomyopathies caused by LMNA gene defects are highly penetrant, adult onset, malignant diseases characterized by a high rate of heart failure and life-threatening arrhythmias, predicted by New York Heart Association functional class, competitive sport activity, and type of mutation.

Published
2008
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44. Long-term health outcome and quality of life in children with multisystem inflammatory syndrome: findings from multidisciplinary follow-up at an Italian tertiary-care paediatric hospital.

Author

D'Auria E, Bova SM, Dallapiccola AR, De Santis R, Leone A, Calcaterra V, Mannarino S, Garbin M, Olivotto S, Zirpoli S, Ghezzi M, Munari AM, Verduci E, Farolfi A, Bosetti A, Perico V, Capetti P, Gadda A, Gianolio L, Lo Monaco G, Lonoce L, Previtali R, Serafini L, Taranto S, Veggiotti P, and Zuccotti G

Abstract

Multisystem inflammatory syndrome is a severe complication of SARS-CoV-2 infection in children (MIS-C). To date, data on long-term sequelae mainly concern cardiac outcomes. All ≤ 18 year olds consecutively admitted to the Buzzi Children's Hospital with a diagnosis of MIS-C between October 1, 2020, and May 31, 2022, were followed up for up to 12 months by a dedicated multidisciplinary team. They underwent laboratory tests, multi-organ clinical and instrumental assessments, and psychosocial evaluation. 56/62 patients, 40 M, mean age 8.7 years (95% CI 7.7, 9.7), completed the follow-up. Cardiological, gastroenterological, pneumological, and neurological evaluations, including IQ and EEG, were normal. Alterations of HOMA-IR index and/or TyG index, observed in almost all patients during hospitalisation, persisted in about a third of the population at 12 months. At 6 and 12 months respectively, impairment of adaptive functions was observed in 38/56 patients (67.9%) and 25/56 (44.6%), emotional and behavioural problems in 10/56 (17.9%) and 9/56 (16.1%), and decline in QoL in 14/56 (25.0%) and 9/56 (16.1%). Psychosocial well-being impairment was significantly more frequent in the subgroup with persistent glycometabolic dysfunction at 12 months (75% vs. 40.9% p < 0.001)., Conlusion: The mechanisms that might explain the long-term persistence of both metabolic alterations and neuro-behavioural outcomes and their possible relationship are far from being clarified. Our study points out to the potential long-term effects of pandemics and to the importance of a multidisciplinary follow-up to detect potential negative sequelae in different areas of health, both physical and psychosocial., What Is Known: • Multisystem inflammatory syndrome in children (MIS-C) is a severe complication of SARS-CoV-2 infection. • Few data exist on the medium- and long-term outcomes of MIS-C, mostly focused on cardiac involvement. Emerging evidence shows neurological and psychological sequelae at mid- and long-term follow-up., What Is New: • This study reveals that MIS-C may lead to long-term glycometabolic dysfunctions joined to impairment in the realm of general well-being and decline in quality of life, in a subgroup of children. • This study highlights the importance of a long-term multidisciplinary follow-up of children hospitalised with MIS-C, in order to detect the potential long-term sequelae in different areas of health, both physical and psychosocial well-being., (© 2024. The Author(s).)

Published
2024
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45. Long-term cytokine profile in multisystem inflammatory disease among children.

Author

Calcaterra V, Loretelli C, Biganzoli D, Abdelsalam A, Marano G, Carelli S, Fiori L, Mannarino S, D'Auria E, Verduci E, De Santis R, Dilillo D, Fabiano V, Carlucci P, Maghraby E, Messa L, Cereda C, Fiorina P, Biganzoli E, and Zuccotti G

Subjects
Humans, Child, Female, Male, Child, Preschool, Systemic Inflammatory Response Syndrome blood, Systemic Inflammatory Response Syndrome immunology, Adolescent, Follow-Up Studies, SARS-CoV-2, Cytokines blood, COVID-19 immunology, COVID-19 blood, COVID-19 complications
Abstract

Background: Multisystem inflammatory disease in children (MIS-C) is a post-infectious condition following coronavirus disease-19 infection. Long-term follow-up data suggests that initial clinical severity does not necessarily correlate with long-term outcomes. The long-term immunological response in children with MIS-C remains poorly understood. We analyzed cytokine profiles at diagnosis and during follow-up, in pediatric patients with MIS-C, exploring correlations among cytokine expressions and standard biochemical and hormonal test results., Methods: Twenty-five MIS-C patients (mean 9.4 ± 3.9) with complete test results at diagnosis and at 6- and 12-months follow-up were included in the study. Selected cytokines, such as IL-9, eotaxin, IP-10, MIP-1β, RANTES, MCP-1(MCAF), TNF-α, PDGF-B, IL-4, and MIP-1α, were included in the analysis., Results: IP-10, MCP-1 (MCAF), and MIP-1α levels normalized or nearly normalized at 6-12 months, the remaining cytokines, including IL-9, eotaxin, MIP-1β, RANTES, TNF-α, PDGF-B, IL-4, remained higher in MIS-C than in controls at our last follow-up time. At 6 months post-diagnosis, a mild negative correlation between triglycerides and HOMA-IR with MCP-1 (MCAF), IL-4, and Eotaxin was noted. At the 12-month follow-up we found a mild positive correlation of cortisol and ACTH levels with PDGF-B, MIP-1α, and TNF-α. Conversely, a negative correlation between these cytokines with fasting glucose and HOMA-IR was observed., Conclusions: Our study findings highlight a notable cytokine-mediated inflammatory response in pediatric patients with MIS-C, characterized by sustained elevated levels over a 12-month monitoring period compared to the control group. We have identified various interrelationships among different cytokines, as well as correlations between heightened cytokine levels and metabolic and hormonal patterns. The pronounced inflammatory response underscores its involvement in acute organ damage, while its persistence suggests potential implications for long-term metabolic disorders., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 Elsevier Ltd. All rights reserved.)

Published
2024
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46. COVID-19-Associated Multisystem Inflammatory Syndrome in Children and Cardiovascular Autonomic Control: A Prospective Cohort Study Nine Months after SARS-CoV-2 Infection.

Author

Castiglioni P, Rampichini S, Corti CG, Mannarino S, Zuccotti G, Calcaterra V, Formenti D, Moriondo A, Maggioni MA, Esposito F, and Merati G

Abstract

Background: Multisystem Inflammatory Syndrome in Children (MIS-C) has emerged as a severe pediatric complication during the SARS-CoV-2 pandemic, with potential long-term cardiovascular repercussions. We hypothesized that heart rate and blood pressure control at rest and during postural maneuvers in MIS-C patients, months after the remission of the inflammatory syndrome, may reveal long-term autonomic dysfunctions. Methods: We assessed 17 MIS-C patients (13 males; 11.9 ± 2.6 years, m ± SD) 9 months after acute infection and 18 age- (12.5 ± 2.1 years) and sex- (13 males) matched controls. Heart rate and blood pressure variability, baroreflex function, and hemodynamic parameters were analyzed in supine and standing postures. Results: MIS-C patients exhibited reduced heart rate variability, particularly in parasympathetic parameters during standing (pNN50+: 6.1 ± 6.4% in controls, 2.5 ± 3.9% in MIS-C; RMSSD: 34 ± 19 ms in controls, 21 ± 14 ms in MIS-C, p < 0.05), with no interaction between case and posture. Blood pressure variability and baroreflex sensitivity did not differ between groups except for the high-frequency power in systolic blood pressure (3.3 ± 1.2 mmHg 2 in controls, 1.8 ± 1.2 mmHg 2 in MIS-C, p < 0.05). The MIS-C group also showed lower diastolic pressure-time indices (DPTI) and systolic pressure-time indices (SPTI), particularly in standing (DPTI: 36.2 ± 9.4 mmHg·s in controls, 29.4 ± 6.2 mmHg·s in MIS-C; SPTI: 26.5 ± 4.3 mmHg·s in controls, 23.9 ± 2.4 mmHg·s in MIS-C, p < 0.05). Conclusions: Altered cardiovascular autonomic control may persist in MIS-C patients with, however, compensatory mechanisms that may help maintain cardiovascular homeostasis during light autonomic challenges, such as postural maneuvers. These results highlight the importance of assessing long-term cardiovascular autonomic control in children with MIS-C to possibly identify residual cardiovascular risks and inform targeted interventions and rehabilitation protocols.

Published
2024
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47. IL-12 and PD-1 peptide combination gene therapy for the treatment of melanoma.

Author

Heller LC, Shi G, Sales Conniff A, Singh J, Mannarino S, Synowiec J, and Heller R

Abstract

Interleukin-12 (IL-12) gene electrotransfer (GET) delivery is highly effective in inducing long-term, complete regression in mouse and human melanoma and other solid tumors. Therapeutic efficacy is enhanced by immune checkpoint inhibitors, and the combination of IL-12 plasmid GET (pIL-12 GET) and anti-programmed cell death protein 1 (PD-1) monoclonal antibodies has reached clinical trials. In this study, we designed peptides and plasmids encoding the mouse homologs of the pembrolizumab and nivolumab programmed cell death 1 ligand 1 (PD-L1) binding regions. We hypothesized that intratumor autocrine/paracrine peptide expression would block PD-1/PD-L1 binding and provide cancer patients with an effective and cost-efficient treatment alternative. We demonstrated that the mouse homolog to pembrolizumab was effective at blocking PD-1/PD-L1 in vitro . After intratumor plasmid delivery, both peptides bound PD-L1 on tumor cells. We established that plasmid DNA delivery to tumors in vivo or to tumor cells in vitro upregulated several immune modulators and PD-L1 mRNA and protein, potentiating this therapy. Finally, we tested the combination of pIL-12 GET therapy and peptide plasmids. We determined that pIL-12 GET therapeutic efficacy could be enhanced by combination with the plasmid encoding the pembrolizumab mouse homolog., Competing Interests: L.H. and R.H. are inventors on patents that cover the technology that was used in the work reported in this manuscript. In addition, R.H. owns stock and stock options in Inovio Pharmaceutical, Inc., (© 2024 The Author(s).)

Published
2024
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48. Fetal Myocarditis Associated With Maternal SARS-CoV-2 Infection.

Author

Mannarino S, Lanna M, Calcaterra V, Carzaniga T, Casiraghi L, Lai A, Gabrieli A, Bergna A, Fini G, Bianchi S, De Amici M, Zehender G, Bellini T, Buscaglia M, and Zuccotti G

Subjects
Child, Female, Humans, SARS-CoV-2, Antibodies, Mothers, Antibodies, Viral, Myocarditis etiology, COVID-19 complications
Abstract

We report the first case of significant fetal myocardial involvement associated with maternal SARS-CoV-2 infection, in which restoration of cardiac function at birth was noted. The demonstration of previous infection was supported by the quantification of humoral response in child and mother, in particular the presence of anti-N antibodies and through the detection of specific antibodies against the BA.4/5 variant., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2024 Wolters Kluwer Health, Inc. All rights reserved.)

Published
2024
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49. High-Sensitivity Cardiac Troponin and the Management of Congenital Heart Disease in Newborns and Infants.

Author

Ferraro S, Biganzoli E, Mannarino S, Lanzoni M, Zuccotti G, Plebani M, and Kavsak P

Subjects
Female, Humans, Infant, Infant, Newborn, Pregnancy, Heart, Troponin T, Heart Defects, Congenital diagnosis, Heart Injuries
Abstract

Background: Early cardiac interventions in newborns and infants suspected for congenital heart disease (CHD) decrease morbidity and mortality. After updating current evidence on the use of cardiac troponins (cTn) in the context of CHD for risk stratification at early ages, we discuss relevant issues, starting from the evidence that only the measurement of the cTnT form is useful in this population., Content: In newborns/infants with CHD, the cTnT concentration increase is correlated with: (a) cardiac stress and hemodynamic parameters, but not with the type of CHD; (b) volume overload/right ventricular pressure overload; (c) postoperative hypoperfusion injury and mortality; and (d) effects of cardioprotective strategies. For infants with CHD, high-sensitivity cTnT (hs-cTnT) concentrations >25 ng/L are an independent predictor of poor outcomes. Transitioning from cTnT to hs-cTnT in newborns/infants improves the identification of: (a) physiopathological mechanisms and factors that increased hs-cTnT early after birth; (b) myocardial injury, even when subclinical; (c) identification of patients requiring immediate therapeutic interventions; and (d) 99th percentile upper reference limits (URLs). However, no reliable URLs are currently available to allow the detection of myocardial injury associated with CHD in newborns/infants., Summary: Additional data evaluating the clinical value of hs-cTnT in the risk stratification of newborns/infants with CHD who may suffer myocardial injury is needed. Validating the measurement, possibly in amniotic fluid samples, and improving the interpretation of hs-cTnT concentrations in the prenatal period, at birth and within 1 year of age are crucial to change CHD mortality/morbidity trends in the pediatric population., (© Association for Diagnostics & Laboratory Medicine 2024. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

Published
2024
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50. Maternal hemodynamic evaluation in monochorionic twin pregnancy complicated by twin-to-twin transfusion syndrome treated with fetoscopic laser surgery.

Author

Milazzo R, Garbin M, Consonni D, Casati D, Faiola S, Laoreti A, Mannarino S, Cetin I, and Lanna MM

Subjects
Pregnancy, Female, Humans, Infant, Pregnancy, Twin, Placenta, Placenta Growth Factor, Hemodynamics, Lasers, Fetofetal Transfusion diagnosis, Fetofetal Transfusion surgery, Laser Therapy adverse effects
Abstract

Background: Maternal cardiovascular adaptations are amplified in twin pregnancies to support the metabolic request of the feto-placental unit. Few studies have evaluated the maternal hemodynamics changes after routine use of laser surgery in the treatment of twin-twin transfusion syndrome., Objective: The aim of our study was to evaluate hemodynamic changes in monochorionic twin pregnancies complicated by twin-twin transfusion syndrome before and after treatment with fetoscopic laser surgery., Study Design: A prospective observational study from 2020 to 2022, included monochorionic twin pregnancies complicated with twin-twin transfusion syndrome undergoing laser surgery between 16 and 26 weeks of gestation. To assess placental function and perfusion, uterine artery pulsatility index, hemoglobin, hematocrit, and soluble fms-like tyrosine kinase-1/placental growth factor ratio sampling prelaser and 24 hours postlaser were measured. Echocardiography by a single cardiologist evaluated maternal hemodynamics at presurgery, 24 hours, and 1 week postlaser. Those data were crosswise compared with cardiovascular indices of uncomplicated monochorionic pregnancies recruited at the same gestational age using nonparametric tests. Moreover, we fitted random-intercept linear regression models to investigate maternal hemodynamic changes according to the amount of amniotic fluid drained during laser surgery., Results: Forty-two twin-twin transfusion syndrome pregnancies with a median gestational age of 19.1 (17.4-20.9) weeks and 15 uncomplicated monochorionic pregnancies at the same gestational age were enrolled. Overall survival rate after laser was 72% with delivery at a median gestational age of 31.5 (27-34) weeks. Significant changes in blood chemistry and placental function were observed in the twin-twin transfusion syndrome group, along with alterations in arterial pressure, heart rate, cardiac output, and ventricular strain, eventually aligning with the uncomplicated group's values by 1 week postlaser. The amount of amniodrainage, with a 1000 ml cut-off, did not significantly impact hemodynamic parameters. Lastly, we detected a percentage of laser surgery complications in agreement with international literature and we did not record any maternal procedure-related problems., Conclusion: Our analysis highlighted that maternal cardiovascular status in monochorionic twin pregnancy complicated by twin-twin transfusion syndrome was more dynamic and; 1 week after fetoscopic laser ablation of placental anastomosis completed by amniodrainage, maternal hemodynamic parameters restored to values similar to uncomplicated monochorionic twin pregnancies., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Published
2024
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