595 results on '"Montini G"'
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2. Timing and Modality of Kidney Replacement Therapy in Children and Adolescents
- Author
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Cortina, G., Arbeiter, K., Dusek, J., Harambat, J., Ranchin, B., Fischbach, M., Querfeld, U., Habbig, S., Galiano, M., Büscher, R., Gimpel, C., Kemper, M., Melk, A., Thurn, D., Schaefer, F., Doyon, A., Wühl, E., Pohl, M., Wygoda, S., Jeck, N., Kranz, B., Wigger, M., Montini, G., Lugani, F., Testa, S., Vidal, E., Matteucci, C., Picca, S., Jankauskiene, A., Azukaitis, K., Zurowska, A., Drodz, D., Tkaczyk, M., Urasinski, T., Litwin, M., Szczepanska, M., Texeira, A., Peco-Antic, A., Bucher, B., Laube, G., Anarat, A., Bayazit, A.K., Yalcinkaya, F., Basin, E., Cakar, N., Soylemezoglu, O., Duzova, A., Bilginer, Y., Erdogan, H., Donmez, O., Balat, A., Kiyak, A., Caliskan, S., Canpolat, N., Candan, C., Civilibal, M., Emre, S., Ozcelik, G., Mir, S., Sözeri, B., Yavascan, O., Tabel, Y., Ertan, P., Yilmaz, E., Shroff, R., Thumfart, Julia, Wagner, Steffen, Kirchner, Marietta, Azukaitis, Karolis, Bayazit, Aysun K., Obrycki, Lukasz, Canpolat, Nur, Bulut, Ipek Kaplan, Duzova, Ali, Anarat, Ali, Bessenay, Lucie, Shroff, Rukshana, Paripovic, Dusan, Sever, Lale, Candan, Cengiz, Lugani, Francesca, Yilmaz, Alev, Yalcinkaya, Fatos, Arbeiter, Klaus, Kiyak, Aysel, Zurowska, Aleksandra, Galiano, Matthias, Querfeld, Uwe, Melk, Anette, and Schaefer, Franz
- Published
- 2024
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3. Respiratory complications and sleep disorders in children with chronic kidney disease: A correlation often underestimated
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Lelii, M., Senatore, L., Paglialonga, F., Consolo, S., Montini, G., Rocchi, A., Marchisio, P., and Patria, M.F.
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- 2023
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4. Timing and modality of kidney replacement therapy in children and adolescents
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Thumfart, Julia, primary, Wagner, Steffen, additional, Kirchner, Marietta, additional, Azukaitis, Karolis, additional, Bayazit, Aysun K., additional, Obrycki, Lukasz, additional, Canpolat, Nur, additional, Bulut, Ipek Kaplan, additional, Duzova, Ali, additional, Anarat, Ali, additional, Bessenay, Lucie, additional, Shroff, Rukshana, additional, Paripovic, Dusan, additional, Sever, Lale, additional, Candan, Cengiz, additional, Lugani, Francesca, additional, Yilmaz, Alev, additional, Yalcinkaya, Fatos, additional, Arbeiter, Klaus, additional, Kiyak, Aysel, additional, Zurowska, Aleksandra, additional, Galiano, Matthias, additional, Querfeld, Uwe, additional, Melk, Anette, additional, Schaefer, Franz, additional, Cortina, G., additional, Arbeiter, K., additional, Dusek, J., additional, Harambat, J., additional, Ranchin, B., additional, Fischbach, M., additional, Zalosczyk, A., additional, Querfeld, U., additional, Habbig, S., additional, Galiano, M., additional, Büscher, R., additional, Gimpel, C., additional, Kemper, M., additional, Melk, A., additional, Thurn, D., additional, Schaefer, F., additional, Doyon, A., additional, Wühl, E., additional, Pohl, M., additional, Wygoda, S., additional, Jeck, N., additional, Kranz, B., additional, Wigger, M., additional, Montini, G., additional, Lugani, F., additional, Testa, S., additional, Vidal, E., additional, Matteucci, C., additional, Picca, S., additional, Jankauskiene, A., additional, Azukaitis, K., additional, Zurowska, A., additional, Drodz, D., additional, Tkaczyk, M., additional, Urasinski, T., additional, Litwin, M., additional, Niemirska, A., additional, Szczepanska, M., additional, Texeira, A., additional, Peco-Antic, A., additional, Bucher, B., additional, Laube, G., additional, Anarat, A., additional, Bayazit, A.K., additional, Yalcinkaya, F., additional, Basin, E., additional, Cakar, N., additional, Soylemezoglu, O., additional, Duzova, A., additional, Bilginer, Y., additional, Erdogan, H., additional, Donmez, O., additional, Balat, A., additional, Kiyak, A., additional, Caliskan, S., additional, Canpolat, N., additional, Candan, C., additional, Civilibal, M., additional, Emre, S., additional, Alpay, H., additional, Ozcelik, G., additional, Mir, S., additional, Sözeri, B., additional, Yavascan, O., additional, Tabel, Y., additional, Ertan, P., additional, Yilmaz, E., additional, and Shroff, R., additional
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- 2024
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- View/download PDF
5. Adolescent gender dysphoria management: position paper from the Italian Academy of Pediatrics, the Italian Society of Pediatrics, the Italian Society for Pediatric Endocrinology and Diabetes, the Italian Society of Adolescent Medicine and the Italian Society of Child and Adolescent Neuropsychiatry
- Author
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Calcaterra, V, Tornese, G, Zuccotti, G, Staiano, A, Cherubini, V, Gaudino, R, Fazzi, E, Barbi, E, Chiarelli, F, Corsello, G, Esposito, S, Ferrara, P, Iughetti, L, Laforgia, N, Maghnie, M, Marseglia, G, Perilongo, G, Pettoello-Mantovani, M, Ruggieri, M, Russo, G, Salerno, M, Striano, P, Valerio, G, Wasniewska, M, Agosti, M, Agostoni, C, Aiuti, A, Azzari, C, Badolato, R, Balduzzi, A, Baraldi, E, Canani, R, Biffi, A, Biondi, A, Bisogno, G, Pierri, N, Carnielli, V, Cianfarani, S, Cogo, P, Corvaglia, L, Dani, C, Di Salvo, G, Fagioli, F, Fanos, V, Ferrero, G, Francavilla, R, Galli, L, Gazzolo, D, Giaquinto, C, Giordano, P, Gitto, E, Grosso, S, Guarino, A, Indrio, F, Lanari, M, Lionetti, P, Locatelli, F, Lombardo, F, Maffeis, C, Marino, B, Midulla, F, Del Giudice, E, Del Giudice, M, Montini, G, Parenti, G, Parisi, P, Peroni, D, Perrotta, S, Piacentini, G, Pietrobelli, A, Raimondi, F, Ramenghi, U, Ravelli, A, Romano, C, Rossi, F, Rossi, P, Damiano, V, Spalice, A, Suppiej, A, Troncone, R, Verrotti, A, Null, N, Calcaterra, Valeria, Tornese, Gianluca, Zuccotti, Gianvincenzo, Staiano, Annamaria, Cherubini, Valentino, Gaudino, Rossella, Fazzi, Elisa Maria, Barbi, Egidio, Chiarelli, Francesco, Corsello, Giovanni, Esposito, Susanna Maria Roberta, Ferrara, Pietro, Iughetti, Lorenzo, Laforgia, Nicola, Maghnie, Mohamad, Marseglia, Gianluigi, Perilongo, Giorgio, Pettoello-Mantovani, Massimo, Ruggieri, Martino, Russo, Giovanna, Salerno, Mariacarolina, Striano, Pasquale, Valerio, Giuliana, Wasniewska, Malgorzata, Agosti, Massimo, Agostoni, Carlo Virginio, Aiuti, Alessandro, Azzari, Chiara, Badolato, Raffaele, Balduzzi, Adriana, Baraldi, Eugenio, Canani, Roberto Berni, Biffi, Alessandra, Biondi, Andrea, Bisogno, Gianni, Pierri, Nicola Brunetti, Carnielli, Virginio, Cianfarani, Stefano, Cogo, Paola, Corvaglia, Luigi, Dani, Carlo, Di Salvo, Giovanni, Fagioli, Franca, Fanos, Vassilios, Ferrero, Giovanni Battista, Francavilla, Ruggiero, Galli, Luisa, Gazzolo, Diego, Giaquinto, Carlo, Giordano, Paola, Gitto, Eloisa, Grosso, Salvatore, Guarino, Alfredo, Indrio, Flavia, Lanari, Marcello, Lionetti, Paolo, Locatelli, Franco, Lombardo, Fortunato, Maffeis, Claudio, Marino, Bruno, Midulla, Fabio, Del Giudice, Emanuele Miraglia, Del Giudice, Michele Miraglia, Montini, Giovanni, Parenti, Giancarlo, Parisi, Pasquale, Peroni, Diego, Perrotta, Silverio, Piacentini, Giorgio, Pietrobelli, Angelo, Raimondi, Francesco, Ramenghi, Ugo, Ravelli, Angelo, Romano, Claudio, Rossi, Francesca, Rossi, Paolo, Damiano, Vincenzo Salpietro, Spalice, Alberto, Suppiej, Agnese, Troncone, Riccardo, Verrotti, Alberto, null, null, Calcaterra, V, Tornese, G, Zuccotti, G, Staiano, A, Cherubini, V, Gaudino, R, Fazzi, E, Barbi, E, Chiarelli, F, Corsello, G, Esposito, S, Ferrara, P, Iughetti, L, Laforgia, N, Maghnie, M, Marseglia, G, Perilongo, G, Pettoello-Mantovani, M, Ruggieri, M, Russo, G, Salerno, M, Striano, P, Valerio, G, Wasniewska, M, Agosti, M, Agostoni, C, Aiuti, A, Azzari, C, Badolato, R, Balduzzi, A, Baraldi, E, Canani, R, Biffi, A, Biondi, A, Bisogno, G, Pierri, N, Carnielli, V, Cianfarani, S, Cogo, P, Corvaglia, L, Dani, C, Di Salvo, G, Fagioli, F, Fanos, V, Ferrero, G, Francavilla, R, Galli, L, Gazzolo, D, Giaquinto, C, Giordano, P, Gitto, E, Grosso, S, Guarino, A, Indrio, F, Lanari, M, Lionetti, P, Locatelli, F, Lombardo, F, Maffeis, C, Marino, B, Midulla, F, Del Giudice, E, Del Giudice, M, Montini, G, Parenti, G, Parisi, P, Peroni, D, Perrotta, S, Piacentini, G, Pietrobelli, A, Raimondi, F, Ramenghi, U, Ravelli, A, Romano, C, Rossi, F, Rossi, P, Damiano, V, Spalice, A, Suppiej, A, Troncone, R, Verrotti, A, Null, N, Calcaterra, Valeria, Tornese, Gianluca, Zuccotti, Gianvincenzo, Staiano, Annamaria, Cherubini, Valentino, Gaudino, Rossella, Fazzi, Elisa Maria, Barbi, Egidio, Chiarelli, Francesco, Corsello, Giovanni, Esposito, Susanna Maria Roberta, Ferrara, Pietro, Iughetti, Lorenzo, Laforgia, Nicola, Maghnie, Mohamad, Marseglia, Gianluigi, Perilongo, Giorgio, Pettoello-Mantovani, Massimo, Ruggieri, Martino, Russo, Giovanna, Salerno, Mariacarolina, Striano, Pasquale, Valerio, Giuliana, Wasniewska, Malgorzata, Agosti, Massimo, Agostoni, Carlo Virginio, Aiuti, Alessandro, Azzari, Chiara, Badolato, Raffaele, Balduzzi, Adriana, Baraldi, Eugenio, Canani, Roberto Berni, Biffi, Alessandra, Biondi, Andrea, Bisogno, Gianni, Pierri, Nicola Brunetti, Carnielli, Virginio, Cianfarani, Stefano, Cogo, Paola, Corvaglia, Luigi, Dani, Carlo, Di Salvo, Giovanni, Fagioli, Franca, Fanos, Vassilios, Ferrero, Giovanni Battista, Francavilla, Ruggiero, Galli, Luisa, Gazzolo, Diego, Giaquinto, Carlo, Giordano, Paola, Gitto, Eloisa, Grosso, Salvatore, Guarino, Alfredo, Indrio, Flavia, Lanari, Marcello, Lionetti, Paolo, Locatelli, Franco, Lombardo, Fortunato, Maffeis, Claudio, Marino, Bruno, Midulla, Fabio, Del Giudice, Emanuele Miraglia, Del Giudice, Michele Miraglia, Montini, Giovanni, Parenti, Giancarlo, Parisi, Pasquale, Peroni, Diego, Perrotta, Silverio, Piacentini, Giorgio, Pietrobelli, Angelo, Raimondi, Francesco, Ramenghi, Ugo, Ravelli, Angelo, Romano, Claudio, Rossi, Francesca, Rossi, Paolo, Damiano, Vincenzo Salpietro, Spalice, Alberto, Suppiej, Agnese, Troncone, Riccardo, Verrotti, Alberto, and null, null
- Abstract
Background In response to the imperative need for standardized support for adolescent Gender Dysphoria (GD), the Italian Academy of Pediatrics, in collaboration with the Italian Society of Pediatrics, the Italian Society for Pediatric Endocrinology and Diabetes, Italian Society of Adolescent Medicine and Italian Society of Child and Adolescent Neuropsychiatry is drafting a position paper. The purpose of this paper is to convey the author's opinion on the topic, offering foundational information on potential aspects of gender-affirming care and emphasizing the care and protection of children and adolescents with GD.Main body Recognizing that adolescents may choose interventions based on their unique needs and goals and understanding that every individual within this group has a distinct trajectory, it is crucial to ensure that each one is welcomed and supported. The approach to managing individuals with GD is a multi-stage process involving a multidisciplinary team throughout all phases. Decisions regarding treatment should be reached collaboratively by healthcare professionals and the family, while considering the unique needs and circumstances of the individual and be guided by scientific evidence rather than biases or ideologies. Politicians and high court judges should address discrimination based on gender identity in legislation and support service development that aligns with the needs of young people. It is essential to establish accredited multidisciplinary centers equipped with the requisite skills and experience to effectively manage adolescents with GD, thereby ensuring the delivery of high-quality care.Conclusion Maintaining an evidence-based approach is essential to safeguard the well-being of transgender and gender diverse adolescents.
- Published
- 2024
6. New Insight into Idiopathic Nephrotic Syndrome: Strategy Based on Urinary Exosomes
- Author
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Barigazzi, Elisa, primary, Santorelli, Lucia, additional, Morello, W., additional, Raimondo, F., additional, Crapella, B., additional, Ghio, L., additional, Tamburello, C., additional, Montini, G., additional, and Pitto, M., additional
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- 2020
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7. New Insight into Idiopathic Nephrotic Syndrome: Strategy Based on Urinary Exosomes
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Barigazzi, Elisa, Santorelli, Lucia, Morello, W., Raimondo, F., Crapella, B., Ghio, L., Tamburello, C., Montini, G., Pitto, M., Sindona, Giovanni, editor, Banoub, Joseph H., editor, and Di Gioia, Maria Luisa, editor
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- 2020
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8. L’effetto sospensivo dell'impugnazione delle decisioni penali
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Montini, G. Paolo, primary
- Published
- 2023
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9. Blood pressure management in children on dialysis
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Paglialonga, F., Consolo, S., Edefonti, A., and Montini, G.
- Subjects
Hypertension -- Causes of -- Health aspects ,Blood pressure -- Health aspects -- Control ,Cardiovascular diseases -- Complications and side effects ,Health - Abstract
Hypertension is a leading cause of cardiovascular complications in children on dialysis. Volume overload and activation of the renin-angiotensin-aldosterone system play a major role in the pathophysiology of hypertension. The first step in managing blood pressure (BP) is the careful assessment of ambulatory BP monitoring. Volume control is essential and should start with the accurate identification of dry weight, based on a comprehensive assessment, including bioimpedance analysis and intradialytic blood volume monitoring (BVM). Reduction of interdialytic weight gain (IDWG) is critical, as higher IDWG is associated with a worse left ventricular mass index and poorer BP control: it can be obtained by means of salt restriction, reduced fluid intake, and optimized sodium removal in dialysis. Optimization of peritoneal dialysis and intensified hemodialysis or hemodiafiltration have been shown to improve both fluid and sodium management, leading to better BP levels. Studies comparing different antihypertensive agents in children are lacking. The pharmacokinetic properties of each drug should be considered. At present, BP control remains suboptimal in many patients and efforts are needed to improve the long-term outcomes of children on dialysis., Author(s): F. Paglialonga [sup.1] , S. Consolo [sup.1] , A. Edefonti [sup.1] , G. Montini [sup.1] [sup.2] Author Affiliations: (1) Pediatric Nephrology, Dialysis and Transplant Unit, Fondazione IRCCS Ca' Granda [...]
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- 2018
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10. Diagnosis and management of urinary tract infections in children aged 2 months to 3 years in the Italian emergency units: the ItaUTI study
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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
11. WCN23-0888 PEDIATRIC NEPHROLOGY RESOURCES IN LOW AND MIDDLE INCOME (LMI) COUNTRIES: A SURVEY FROM THE IPNA PRIORITIES IN LOW RESOURCE COUNTRIES COMMITTEE (LRCC)
- Author
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ERICKSON, R., primary, Kamath, N., additional, Safouh, H., additional, Montini, G., additional, and Bonilla-Félix, M., additional
- Published
- 2023
- Full Text
- View/download PDF
12. Multi-population genome-wide association study implicates immune and non-immune factors in pediatric steroid-sensitive nephrotic syndrome.
- Author
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Barry, A., McNulty, M.T., Jia, X., Gupta, Y., Debiec, H., Luo, Y, Nagano, C., Horinouchi, T., Jung, S., Colucci, M., Ahram, D.F., Mitrotti, A., Sinha, A., Teeninga, N., Jin, G., Shril, S., Caridi, G., Bodria, M., Lim, T.Y., Westland, R., Zanoni, F., Marasa, M., Turudic, D., Giordano, M., Gesualdo, L., Magistroni, R., Pisani, I., Fiaccadori, E., Reiterova, J., Maringhini, S., Morello, W., Montini, G., Weng, P.L., Scolari, F., Saraga, M., Tasic, V., Santoro, D., Wijk, J.A. van, Milošević, D., Kawai, Y., Kiryluk, K., Pollak, M.R., Gharavi, A., Lin, F., Simœs E Silva, A.C., Loos, R.J., Kenny, E.E., Schreuder, M.F., Zurowska, A., Dossier, C., Ariceta, G., Drozynska-Duklas, M., Hogan, J., Jankauskiene, A., Hildebrandt, F., Prikhodina, L., Song, K., Bagga, A., Cheong H, 2.n.d., Ghiggeri, G.M., Vachvanichsanong, P., Nozu, K., Lee, D., Vivarelli, M., Raychaudhuri, S., Tokunaga, K., Sanna-Cherchi, S., Ronco, P., Iijima, K., Sampson, M.G., Barry, A., McNulty, M.T., Jia, X., Gupta, Y., Debiec, H., Luo, Y, Nagano, C., Horinouchi, T., Jung, S., Colucci, M., Ahram, D.F., Mitrotti, A., Sinha, A., Teeninga, N., Jin, G., Shril, S., Caridi, G., Bodria, M., Lim, T.Y., Westland, R., Zanoni, F., Marasa, M., Turudic, D., Giordano, M., Gesualdo, L., Magistroni, R., Pisani, I., Fiaccadori, E., Reiterova, J., Maringhini, S., Morello, W., Montini, G., Weng, P.L., Scolari, F., Saraga, M., Tasic, V., Santoro, D., Wijk, J.A. van, Milošević, D., Kawai, Y., Kiryluk, K., Pollak, M.R., Gharavi, A., Lin, F., Simœs E Silva, A.C., Loos, R.J., Kenny, E.E., Schreuder, M.F., Zurowska, A., Dossier, C., Ariceta, G., Drozynska-Duklas, M., Hogan, J., Jankauskiene, A., Hildebrandt, F., Prikhodina, L., Song, K., Bagga, A., Cheong H, 2.n.d., Ghiggeri, G.M., Vachvanichsanong, P., Nozu, K., Lee, D., Vivarelli, M., Raychaudhuri, S., Tokunaga, K., Sanna-Cherchi, S., Ronco, P., Iijima, K., and Sampson, M.G.
- Abstract
Item does not contain fulltext, Pediatric steroid-sensitive nephrotic syndrome (pSSNS) is the most common childhood glomerular disease. Previous genome-wide association studies (GWAS) identified a risk locus in the HLA Class II region and three additional independent risk loci. But the genetic architecture of pSSNS, and its genetically driven pathobiology, is largely unknown. Here, we conduct a multi-population GWAS meta-analysis in 38,463 participants (2440 cases). We then conduct conditional analyses and population specific GWAS. We discover twelve significant associations-eight from the multi-population meta-analysis (four novel), two from the multi-population conditional analysis (one novel), and two additional novel loci from the European meta-analysis. Fine-mapping implicates specific amino acid haplotypes in HLA-DQA1 and HLA-DQB1 driving the HLA Class II risk locus. Non-HLA loci colocalize with eQTLs of monocytes and numerous T-cell subsets in independent datasets. Colocalization with kidney eQTLs is lacking but overlap with kidney cell open chromatin suggests an uncharacterized disease mechanism in kidney cells. A polygenic risk score (PRS) associates with earlier disease onset. Altogether, these discoveries expand our knowledge of pSSNS genetic architecture across populations and provide cell-specific insights into its molecular drivers. Evaluating these associations in additional cohorts will refine our understanding of population specificity, heterogeneity, and clinical and molecular associations.
- Published
- 2023
13. PERCUTANEOUS CUTTING BALLOON ANGIOPLASTY FOR TREATMENT OF RESISTANT RENAL ARTERY STENOSIS IN PEDIATRIC PATIENTS
- Author
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Salice, P., Mircoli, L., Burdick, L., Butera, G., Mastrangelo, A., Ardissino, G., Borzani, I., Ughi, L., Colombo, F., Pomidossi, A., Baca, L., Beretta, C., Montini, G., Lombardi, F., and Morganti, A.
- Published
- 2019
- Full Text
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14. Blood pressure management in children on dialysis
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Paglialonga, F., Consolo, S., Edefonti, A., and Montini, G.
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- 2017
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15. Variability of diagnostic criteria and treatment of idiopathic nephrotic syndrome across European countries
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Deschênes, Georges, Vivarelli, Marina, Peruzzi, Licia, Alpay, H., Alvaro Madrid, A., Andersen, R., Bald, M., Benetti, E., Berard, E., Bockenhauer, D., Boyer, O., Brackman, D., Dossier, C., Ekinci, Z., Emma, F., Enneman, B., Espinosa-Roman, L., Fila, M., Ghio, L., Groothoff, J. W., Guigonis, V., Jankauskiene, A., Kagan, M., Kovacevic, M., Kemper, M. J., Levtchenko, E., Maringhini, S., Mir, S., Mitsioni, A., Mizerska-Wasiak, M., Wasiak, K., Moczulska, A., Montini, G., Murer, L., Nuutinen, M., Obukhova, V., Oh, J., Ozkaya, O., Papalia, T., Peco Antic, A., Pecoraro, C., Pena-Carrion, A., Petrossian, E., Pietrement, C., Prikhodina, L., Querfeld, U., Rittig, S., Saleem, M. A., Saraga, M., Savenkova, N., Sever, L., Tullus, K., Ulinski, T., Vande Walle, J., Vara, J., Webb, N., Weber, L. T., Zurowska, A., and On behalf of the ESPN Working Group on Idiopathic Nephrotic Syndrome
- Published
- 2017
- Full Text
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16. Voiding cystourethrography and 99MTC-MAG3 renal scintigraphy in pediatric vesicoureteral reflux: what is the role of indirect cystography?
- Author
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Capone, V., Taroni, F., Pavesi, M.A., Castellani, M., Consonni, D., Berrettini, A., Crapella, B., Marra, G., De Palma, D., Zucchetta, P., Manzoni, G.A., and Montini, G.
- Published
- 2019
- Full Text
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17. COVID-19 in Immunosuppressed Children
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Nicastro, E, Verdoni, L, Bettini, L, Zuin, G, Balduzzi, A, Montini, G, Biondi, A, D'Antiga, L, Nicastro E., Verdoni L., Bettini L. R., Zuin G., Balduzzi A., Montini G., Biondi A., D'Antiga L., Nicastro, E, Verdoni, L, Bettini, L, Zuin, G, Balduzzi, A, Montini, G, Biondi, A, D'Antiga, L, Nicastro E., Verdoni L., Bettini L. R., Zuin G., Balduzzi A., Montini G., Biondi A., and D'Antiga L.
- Abstract
Following the spread of the SARS-CoV-2 infection and coronavirus disease 2019 (COVID-19) to a global pandemic, concerns have arisen for the disease impact in at-risk populations, especially in immunocompromised hosts. On the other hand, clinical studies have clarified that the COVID-19 clinical burden is mostly due to over-inflammation and immune-mediated multiorgan injury. This has led to downsizing the role of immunosuppression as a determinant of outcome, and early reports confirm the hypothesis that patients undergoing immunosuppressive treatments do not have an increased risk of severe COVID-19 with respect to the general population. Intriguingly, SARS-CoV-2 natural reservoirs, such as bats and mice, have evolved mechanisms of tolerance involving selection of genes optimizing viral clearance through interferon type I and III responses and also dampening inflammasome response and cytokine expression. Children exhibit resistance to COVID-19 severe manifestations, and age-related features in innate and adaptive response possibly explaining this difference are discussed. A competent recognition by the innate immune system and controlled pro-inflammatory signaling seem to be the pillars of an effective response and the premise for pathogen clearance in SARS-CoV-2 infection. Immunosuppression—if not associated with other elements of fragility—do not represent per se an obstacle to this competent/tolerant phenotype in children. Several reports confirm that children receiving immunosuppressive medications have similar clinical involvement and outcomes as the pediatric general population, indicating that maintenance treatments should not be interrupted in suspect or confirmed SARS-CoV-2 infection.
- Published
- 2021
18. New Insight into Idiopathic Nephrotic Syndrome: Strategy Based on Urinary Exosomes
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Sindona, Giovanni, Banoub, Joseph H, Di Gioia Maria Luisa, Barigazzi, E, Santorelli, L, Morello, W, Raimondo, F, Crapella, B, Ghio, L, Tamburello, C, Montini, G, Pitto, M, Barigazzi E., Santorelli L., Morello W., Raimondo F., Crapella B., Ghio L., Tamburello C., Montini G., Pitto M., Sindona, Giovanni, Banoub, Joseph H, Di Gioia Maria Luisa, Barigazzi, E, Santorelli, L, Morello, W, Raimondo, F, Crapella, B, Ghio, L, Tamburello, C, Montini, G, Pitto, M, Barigazzi E., Santorelli L., Morello W., Raimondo F., Crapella B., Ghio L., Tamburello C., Montini G., and Pitto M.
- Abstract
This study aims to verify the possibility of using urinary exosomes (UE) of idiopathic nephrotic syndrome (INS) patients as a source of predictive markers of response to the corticosteroid treatment, and/or to clarify the disease etiopathogenesis.
- Published
- 2020
19. Definition, diagnosis and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group
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Capone, V., Persico, N., Berrettini, A., Decramer, S., Marco, E.A. De, Palma, D., Familiari, A., Feitz, W., Herthelius, M., Kazlauskas, V., Liebau, M., Manzoni, G. de, Maternik, M., Mosiello, G., Schanstra, J.P., Walle, J. Vande, Wühl, E., Ylinen, E., Zurowska, A., Schaefer, F., Montini, G., Capone, V., Persico, N., Berrettini, A., Decramer, S., Marco, E.A. De, Palma, D., Familiari, A., Feitz, W., Herthelius, M., Kazlauskas, V., Liebau, M., Manzoni, G. de, Maternik, M., Mosiello, G., Schanstra, J.P., Walle, J. Vande, Wühl, E., Ylinen, E., Zurowska, A., Schaefer, F., and Montini, G.
- Abstract
Item does not contain fulltext, Fetal lower urinary tract obstruction (LUTO) is associated with high mortality and postnatal morbidity caused by lung hypoplasia and impaired kidney function. Specific diagnostic features that can guide clinical approach and decisions are lacking; thus, the European Reference Network for Rare Kidney Diseases established a work group to develop recommendations regarding the clinical definition, diagnosis and management of prenatally detected LUTO. The work group recommends the use of antero-posterior diameter of renal pelvis as the most reliable parameter for suspecting obstructive uropathies and for suspecting prenatal LUTO in the presence of fetal megacystis. Regarding prenatal and postnatal prognosis of fetuses with LUTO, the risk of fetal and neonatal death depends on the presence of oligohydramnios or anhydramnios before 20 weeks' gestation, whereas the risk of kidney replacement therapy cannot be reliably foreseen before birth. Parents of fetuses with LUTO must be referred to a tertiary obstetric centre with multidisciplinary expertise in prenatal and postnatal management of obstructive uropathies, and vesico-amniotic shunt placement should be offered in selected instances, as it increases perinatal survival of fetuses with LUTO.
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- 2022
20. Definition, diagnosis and clinical management of non-obstructive kidney dysplasia: a consensus statement by the ERKNet Working Group on Kidney Malformations
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Kohl, S., Avni, F.E., Boor, P., Capone, V., Clapp, W.L., Palma, D., Harris, T., Heidet, L., Hilger, A.C., Liapis, H., Lilien, M., Manzoni, G. de, Montini, G., Negrisolo, S., Pierrat, M.J., Raes, A., Reutter, H., Schreuder, M.F., Weber, S., Winyard, P.J., Woolf, A.S., Schaefer, F., Liebau, M.C., Kohl, S., Avni, F.E., Boor, P., Capone, V., Clapp, W.L., Palma, D., Harris, T., Heidet, L., Hilger, A.C., Liapis, H., Lilien, M., Manzoni, G. de, Montini, G., Negrisolo, S., Pierrat, M.J., Raes, A., Reutter, H., Schreuder, M.F., Weber, S., Winyard, P.J., Woolf, A.S., Schaefer, F., and Liebau, M.C.
- Abstract
Item does not contain fulltext, Kidney dysplasia is one of the most frequent causes of chronic kidney failure in children. While dysplasia is a histological diagnosis, the term 'kidney dysplasia' is frequently used in daily clinical life without histopathological confirmation. Clinical parameters of kidney dysplasia have not been clearly defined, leading to imprecise communication amongst healthcare professionals and patients. This lack of consensus hampers precise disease understanding and the development of specific therapies. Based on a structured literature search, we here suggest a common basis for clinical, imaging, genetic, pathological and basic science aspects of non-obstructive kidney dysplasia associated with functional kidney impairment. We propose to accept hallmark sonographic findings as surrogate parameters defining a clinical diagnosis of dysplastic kidneys. We suggest differentiated clinical follow-up plans for children with kidney dysplasia and summarize established monogenic causes for non-obstructive kidney dysplasia. Finally, we point out and discuss research gaps in the field.
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- 2022
21. Circulating Permeability Factors in Focal Segmental Glomerulosclerosis: In Vitro Detection
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Veissi, S.T., Smeets, B., Wijk, J.A. van, Classens, R.J.P.C., Velden, T.J.A.M. van der, Jeronimus-Klaasen, A., Veltkamp, F., Mak-Nienhuis, E.M., Morello, W., Montini, G., Bouts, A.H., Heuvel, L.P.W.J. van den, Schreuder, M.F., Veissi, S.T., Smeets, B., Wijk, J.A. van, Classens, R.J.P.C., Velden, T.J.A.M. van der, Jeronimus-Klaasen, A., Veltkamp, F., Mak-Nienhuis, E.M., Morello, W., Montini, G., Bouts, A.H., Heuvel, L.P.W.J. van den, and Schreuder, M.F.
- Abstract
Item does not contain fulltext, INTRODUCTION: The recurrence of proteinuria after kidney transplantation in patients with focal segmental glomerulosclerosis (FSGS) is considered proof of the presence of circulating permeability factors (CPFs). The aim of this study is to demonstrate the presence of plasma CPFs using series of in vitro assays. METHODS: Podocytes and endothelial cells (glomerular microvascular endothelial cells [GMVECs]) were incubated with plasma from FSGS patients with presumed CPFs in relapse and remission and from steroid-resistant nephrotic syndrome (SRNS), steroid-sensitive nephrotic syndrome (SSNS), membranous nephropathy (MN), and healthy controls (hCtrls). Cell viability, podocyte actin cytoskeleton architecture, and reactive oxygen species (ROS) formation with or without ROS scavenger were investigated by Cell Counting Kit-8 assay, immunofluorescence staining, and CM-H2DCFDA probing, respectively. RESULTS: Presumed CPF-containing plasma causes a series of events in podocytes but not in GMVECs. These events include actin cytoskeleton rearrangement and excessive formation of ROS, which results in podocyte loss. These effects were solely observed in response to CPF plasma collected during relapse, but not in response to plasma of hCtrls, or patients with SRNS, SSNS, and MN. The copresence of dimethylthiourea, a scavenger of ROS, abolished the aforementioned effects of CPF plasma. CONCLUSION: We provide a panel of in vitro bioassays to measure podocyte injury and predict the presence of CPFs in plasma of patients with nephrotic syndrome (NS), providing a new framework for monitoring CPF activity that may contribute to future NS diagnostics or used for disease monitoring purposes. Moreover, our findings suggest that the inhibition of ROS formation or facilitating rapid ROS scavenging may exert beneficial effects in patients with CPFs.
- Published
- 2022
22. Circulating Permeability Factors in Focal Segmental Glomerulosclerosis: In V itro Detection
- Author
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Veissi, S.T., Smeets, B., Wijk, J.A. van, Classens, R.J.P.C., Velden, T.J.A.M. van der, Jeronimus-Klaasen, A., Veltkamp, F., Mak-Nienhuis, E.M., Morello, W., Montini, G., Bouts, A.H., Heuvel, L.P.W.J. van den, Schreuder, M.F., Veissi, S.T., Smeets, B., Wijk, J.A. van, Classens, R.J.P.C., Velden, T.J.A.M. van der, Jeronimus-Klaasen, A., Veltkamp, F., Mak-Nienhuis, E.M., Morello, W., Montini, G., Bouts, A.H., Heuvel, L.P.W.J. van den, and Schreuder, M.F.
- Abstract
Item does not contain fulltext, INTRODUCTION: The recurrence of proteinuria after kidney transplantation in patients with focal segmental glomerulosclerosis (FSGS) is considered proof of the presence of circulating permeability factors (CPFs). The aim of this study is to demonstrate the presence of plasma CPFs using series of in vitro assays. METHODS: Podocytes and endothelial cells (glomerular microvascular endothelial cells [GMVECs]) were incubated with plasma from FSGS patients with presumed CPFs in relapse and remission and from steroid-resistant nephrotic syndrome (SRNS), steroid-sensitive nephrotic syndrome (SSNS), membranous nephropathy (MN), and healthy controls (hCtrls). Cell viability, podocyte actin cytoskeleton architecture, and reactive oxygen species (ROS) formation with or without ROS scavenger were investigated by Cell Counting Kit-8 assay, immunofluorescence staining, and CM-H2DCFDA probing, respectively. RESULTS: Presumed CPF-containing plasma causes a series of events in podocytes but not in GMVECs. These events include actin cytoskeleton rearrangement and excessive formation of ROS, which results in podocyte loss. These effects were solely observed in response to CPF plasma collected during relapse, but not in response to plasma of hCtrls, or patients with SRNS, SSNS, and MN. The copresence of dimethylthiourea, a scavenger of ROS, abolished the aforementioned effects of CPF plasma. CONCLUSION: We provide a panel of in vitro bioassays to measure podocyte injury and predict the presence of CPFs in plasma of patients with nephrotic syndrome (NS), providing a new framework for monitoring CPF activity that may contribute to future NS diagnostics or used for disease monitoring purposes. Moreover, our findings suggest that the inhibition of ROS formation or facilitating rapid ROS scavenging may exert beneficial effects in patients with CPFs.
- Published
- 2022
23. LINE1 are spliced in non-canonical transcript variants to regulate T cell quiescence and exhaustion
- Author
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Marasca, F, Sinha, S, Vadalà, R, Polimeni, B, Ranzani, V, Paraboschi, E, Burattin, F, Ghilotti, M, Crosti, M, Negri, M, Campagnoli, S, Notarbartolo, S, Sartore-Bianchi, A, Siena, S, Prati, D, Montini, G, Viale, G, Torre, O, Harari, S, Grifantini, R, Soldà, G, Biffo, S, Abrignani, S, Bodega, B, Marasca, Federica, Sinha, Shruti, Vadalà, Rebecca, Polimeni, Benedetto, Ranzani, Valeria, Paraboschi, Elvezia Maria, Burattin, Filippo Vittorio, Ghilotti, Marco, Crosti, Mariacristina, Negri, Maria Luce, Campagnoli, Susanna, Notarbartolo, Samuele, Sartore-Bianchi, Andrea, Siena, Salvatore, Prati, Daniele, Montini, Giovanni, Viale, Giuseppe, Torre, Olga, Harari, Sergio, Grifantini, Renata, Soldà, Giulia, Biffo, Stefano, Abrignani, Sergio, Bodega, Beatrice, Marasca, F, Sinha, S, Vadalà, R, Polimeni, B, Ranzani, V, Paraboschi, E, Burattin, F, Ghilotti, M, Crosti, M, Negri, M, Campagnoli, S, Notarbartolo, S, Sartore-Bianchi, A, Siena, S, Prati, D, Montini, G, Viale, G, Torre, O, Harari, S, Grifantini, R, Soldà, G, Biffo, S, Abrignani, S, Bodega, B, Marasca, Federica, Sinha, Shruti, Vadalà, Rebecca, Polimeni, Benedetto, Ranzani, Valeria, Paraboschi, Elvezia Maria, Burattin, Filippo Vittorio, Ghilotti, Marco, Crosti, Mariacristina, Negri, Maria Luce, Campagnoli, Susanna, Notarbartolo, Samuele, Sartore-Bianchi, Andrea, Siena, Salvatore, Prati, Daniele, Montini, Giovanni, Viale, Giuseppe, Torre, Olga, Harari, Sergio, Grifantini, Renata, Soldà, Giulia, Biffo, Stefano, Abrignani, Sergio, and Bodega, Beatrice
- Abstract
How gene expression is controlled to preserve human T cell quiescence is poorly understood. Here we show that non-canonical splicing variants containing long interspersed nuclear element 1 (LINE1) enforce naive CD4+ T cell quiescence. LINE1-containing transcripts are derived from CD4+ T cell-specific genes upregulated during T cell activation. In naive CD4+ T cells, LINE1-containing transcripts are regulated by the transcription factor IRF4 and kept at chromatin by nucleolin; these transcripts act in cis, hampering levels of histone 3 (H3) lysine 36 trimethyl (H3K36me3) and stalling gene expression. T cell activation induces LINE1-containing transcript downregulation by the splicing suppressor PTBP1 and promotes expression of the corresponding protein-coding genes by the elongating factor GTF2F1 through mTORC1. Dysfunctional T cells, exhausted in vitro or tumor-infiltrating lymphocytes (TILs), accumulate LINE1-containing transcripts at chromatin. Remarkably, depletion of LINE1-containing transcripts restores TIL effector function. Our study identifies a role for LINE1 elements in maintaining T cell quiescence and suggests that an abundance of LINE1-containing transcripts is critical for T cell effector function and exhaustion.
- Published
- 2022
24. How Covid-19 changed the epidemiology of febrile urinary tract infections in children in the emergency department during the first outbreak
- Author
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Cesca, L., Conversano, E., Vianello, F. A., Martelli, L., Gualeni, C., Bassani, F., Brugnara, M., Rubin, G., Parolin, M., Anselmi, M., Marchiori, M., Vergine, G., Miorin, E., Vidal, E., Milocco, C., Orsi, C., Puccio, G., Peruzzi, L., Montini, G., and Dall'Amico, R.
- Subjects
Adult ,Emergency Service ,Urinary tract infection ,Fever ,Adolescent ,SARS-CoV-2 ,COVID-19 ,Infant ,Covid19 ,Newborn ,Disease Outbreaks ,Anti-Bacterial Agents ,Hospital ,C-Reactive Protein ,Diagnosis delay ,Child ,Child, Preschool ,Emergency Service, Hospital ,Escherichia coli ,Humans ,Infant, Newborn ,Pandemics ,Retrospective Studies ,Urinary Tract Infections ,Preschool - Published
- 2022
25. MIF plasma level as a possible tool to predict steroid responsiveness in children with idiopathic nephrotic syndrome
- Author
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Cuzzoni, E, Colturi, F, De Iudicibus, S, Marcuzzi, A, Lucafo, M, Pelin, M, Favretto, D, Monti, E, Morello, W, Ghio, L, La Scola, C, Mencarelli, F, Pasini, A, Montini, G, Decorti, G, Stocco, G, Cuzzoni E., COLTURI, FRANCA RENZA, De Iudicibus S., Marcuzzi A., Lucafo M., Pelin M., Favretto D., Monti E., Morello W., Ghio L., La Scola C., Mencarelli F., Pasini A., Montini G., Decorti G., Stocco G., Cuzzoni, E, Colturi, F, De Iudicibus, S, Marcuzzi, A, Lucafo, M, Pelin, M, Favretto, D, Monti, E, Morello, W, Ghio, L, La Scola, C, Mencarelli, F, Pasini, A, Montini, G, Decorti, G, Stocco, G, Cuzzoni E., COLTURI, FRANCA RENZA, De Iudicibus S., Marcuzzi A., Lucafo M., Pelin M., Favretto D., Monti E., Morello W., Ghio L., La Scola C., Mencarelli F., Pasini A., Montini G., Decorti G., and Stocco G.
- Abstract
Purpose: Idiopathic nephrotic syndrome (INS) is the most frequent form of childhood nephrotic syndrome. Steroids represent the best therapeutic option; however, inter-individual differences in their efficacy and side effects have been reported. To date, there is no way to predict patients’ resistance and/or dependence. Alterations in the cytokine profile of INS patients might contribute to proteinuria and glomerular damage and affect drug sensitivity. Methods: The cytokine plasma levels were measured in 21 INS children at diagnosis to investigate the association among cytokines pattern and clinical response. Patients were selected on the basis of their clinical response: 7 steroid sensitive (SS), 7 dependent (SD), and 7 resistant (SR). Significant results were then analyzed in 41 additional pediatric INS patients. Results: Within the 48 cytokines analyzed, macrophage migration inhibitory factor (MIF) was a good predictor of steroid response. Indeed, SR patients showed significantly higher MIF plasma levels compared with all others (p = 0.022; OR = 4.3, 95%CI = 1.2–25.4): a cutoff concentration of MIF > 501 pg/ml significantly discriminated SR patients (sensitivity = 85.7%, specificity = 71.4%). On the contrary, SD patients showed lower MIF plasma levels compared with others (p = 0.010; OR = 0.12, 95%CI = 9.2 × 10−3–6.7 × 10−1). Significant results were confirmed in the entire cohort. Conclusions: Our comprehensive cytokine analysis indicates that assessing MIF plasma levels at diagnosis could predict response to glucocorticoids in children with INS.
- Published
- 2019
26. Childhood Idiopathic Nephrotic Syndrome: Does the Initial Steroid Treatment Modify the Outcome? A Multicentre, Prospective Cohort Study
- Author
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Pasini, A., Bertulli, C., Casadio, L., Corrado, C., Edefonti, A., Ghiggeri, G., Ghio, L., Giordano, M., La Scola, C., Malaventura, C., Maringhini, S., Mastrangelo, A. P., Materassi, M., Mencarelli, F., Messina, G., Monti, E., Morello, W., Puccio, G., Romagnani, P., and Montini, G.
- Subjects
childhood idiopathic nephrotic syndrome ,frequent relapsers ,total serum protein ,Pediatrics, Perinatology and Child Health ,steroid dependency ,prognostic factors ,Pediatrics ,age at onset ,steroid treatment ,NO ,childhood idiopathic nephrotic syndrome, steroid treatment, frequent relapsers, steroid dependency, prognostic factors, age at onset, total serum protein ,Original Research - Abstract
Background: A great majority of children with idiopathic nephrotic syndrome will relapse after successful treatment of the initial episode. The possibility that different steroid dosing regimens at onset, adjusted for risk factors, can reduce the rate of relapse represents an interesting option to investigate. Objectives: To evaluate the effect of the initial steroid regimen, adjusted for time to remission (TTR), on the frequency of relapses and steroid dependence, and to verify the influence of prognostic factors on disease course. Methods: A multicentre, prospective, cohort study. Children with nephrotic syndrome, with TTR ≤ 10 days (Group A), were given a 20-week prednisone regimen (2,828 mg/m2) and those with a TTR >10 days, a 22-week regimen (3,668 mg/m2) (Group B). Previously published retrospective data from the same centers were also evaluated. Main outcomes were: relapse rate, number of frequent relapsers + steroid dependent children and total prednisone dose after induction. Results: 143 children were enrolled. Rate of relapsed subjects (77 vs. 79%) and frequent relapsers + steroid dependent subjects (40 vs. 53%) did not differ between Groups A and B, or between the retrospective and prospective cohorts. The cumulative prednisone dose taken after the induction treatment was similar in both groups and in the retrospective and prospective cohorts. TTR was not associated with relapse risk. Age at onset and total serum protein were significantly lower in relapsing patients. At ROC analysis, the best cut-off was 5.3 years for age at onset and 4.2 g/dL for total serum protein. According to these cut-offs, older children with higher total serum protein had a higher relapse free survival rate (58%) than younger children with lower total serum protein (17%). Conclusions: TTR was not found to be a prognostic factor of relapse; because of this, different steroid regimens, adjusted for TTR, did not modify the relapse rate in any relevant measure. Conversely, younger age and low total serum protein were independent predictors of relapse risk, however this outcome was not modified by higher prednisone regimens. Clinical Trial Registration:https://www.ClinicalTrials.gov/, identifier: NCT01386957 (www.nefrokid.it).
- Published
- 2021
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- View/download PDF
27. A Community Outbreak of Haemolytic-Uraemic Syndrome in Children Occurring in a Large Area of Northern Italy over a Period of Several Months
- Author
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Tozzi, A. E., Niccolini, A., Caprioli, A., Luzzi, I., Montini, G., Zacchello, G., Gianviti, A., Principato, F., and Rizzoni, G.
- Published
- 1994
28. [Management of Primary Hyperoxaluria Type 1 in Italy]
- Author
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Ferraro, P. M., Giovanni Gambaro, Mandrile, G., Montini, G., Pasini, A., and Peruzzi, L.
- Subjects
hyperoxalemia ,hyperoxaluria ,lumasiran ,primary hyperoxaluria type 1 ,Italy ,Hyperoxaluria, Primary ,Humans ,Settore MED/14 - NEFROLOGIA ,Child ,Kidney - Abstract
Primary hyperoxaluria type 1 is a rare genetic disease; the onset of symptoms ranges from childhood to the sixth decade of life and the disease may go unrecognized for several years. There is an urgent need for drugs able to inhibit the liver production of oxalate and to prevent the disease progression; lumasiran, an innovative molecule based on RNAi interference, is one of the most promising drugs. A group of leading Italian experts on this disease met to respond to some unmet medical needs (early diagnosis, availability of genetic tests and dosage of plasma oxalate, timing of liver transplantation, need for etiologic treatment), based on the analysis of the main scientific evidence and their personal experience. Children showing the characteristic symptoms of the disease usually undergo a metabolic screening and obtain an early diagnosis, while the experience is very limited in adults and the diagnosis difficult. It is therefore essential to increase the knowledge around this disease and the importance of metabolic and genetic screening to define a checklist of shared clinical and laboratory criteria and to establish a multidisciplinary management of potential patients. Oxalate is the cause of the disease: it is crucial to reduce both oxaluria and oxalemia through appropriate therapeutic strategies, able to prevent and/or reduce renal and systemic complications of primary type 1 hyperoxaluria. Lumasiran allows to significantly reduce the levels of oxalate both in blood and in urine, halting the course of the disease and preventing serious renal and systemic complications, if the therapy is started at an early stage of the disease.
- Published
- 2021
29. The European Rare Kidney Disease Registry (ERKReg): objectives, design and initial results
- Author
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Bassanese, G., Wlodkowski, T., Servais, A., Heidet, L., Roccatello, D., Emma, F., Levtchenko, E.N., Ariceta, G., Bacchetta, J., Capasso, G., Jankauskiene, A., Miglinas, M., Ferraro, P.M., Montini, G., Oh, J., Decramer, S., Levart, T.K., Wetzels, J., Cornelissen, E.A., Devuyst, O., Zurowska, A., Pape, L., Buescher, A., Haffner, D., Varda, N. Marcun, Ghiggeri, G.M., Remuzzi, G., Konrad, M., Longo, G., Bockenhauer, D., Awan, A., Andersone, I., Groothoff, J.W., Schaefer, F., Bassanese, G., Wlodkowski, T., Servais, A., Heidet, L., Roccatello, D., Emma, F., Levtchenko, E.N., Ariceta, G., Bacchetta, J., Capasso, G., Jankauskiene, A., Miglinas, M., Ferraro, P.M., Montini, G., Oh, J., Decramer, S., Levart, T.K., Wetzels, J., Cornelissen, E.A., Devuyst, O., Zurowska, A., Pape, L., Buescher, A., Haffner, D., Varda, N. Marcun, Ghiggeri, G.M., Remuzzi, G., Konrad, M., Longo, G., Bockenhauer, D., Awan, A., Andersone, I., Groothoff, J.W., and Schaefer, F.
- Abstract
Contains fulltext : 235691.pdf (Publisher’s version ) (Open Access), BACKGROUND: The European Rare Kidney Disease Reference Network (ERKNet) recently established ERKReg, a Web-based registry for all patients with rare kidney diseases. The main objectives of this core registry are to generate epidemiological information, identify current patient cohort for clinical research, explore diagnostic and therapeutic management practices, and monitor treatment performance and patient's outcomes. The registry has a modular design that allows to integrate comprehensive disease-specific registries as extensions to the core database. The diagnosis (Orphacode) and diagnostic information (clinical, imaging, histopathological, biochemical, immunological and genetic) are recorded. Anthropometric, kidney function, and disease-specific management and outcome items informing a set of 61 key performance indicators (KPIs) are obtained annually. Data quality is ensured by automated plausibility checks upon data entry and regular offline database checks prompting queries. Centre KPI statistics and benchmarking are calculated automatically. RESULTS: Within the first 24 months since its launch, 7607 patients were enrolled to the registry at 45 pediatric and 12 specialized adult nephrology units from 21 countries. A kidney disease diagnosis had been established in 97.1% of these patients at time of enrolment. While 199 individual disease entities were reported by Orphacode, 50% of the cohort could be classified with 11, 80% with 43 and 95% with 92 codes. Two kidney diagnoses were assigned in 6.5% of patients; 5.9% suffered from syndromic disease. Whereas glomerulopathies (54.8%) and ciliopathies including autosomal dominant polycystic kidney disease (ADPKD) (31.5%) were the predominant disease groups among adults, the pediatric disease spectrum encompassed congenital anomalies of the kidney and urinary tract (CAKUT) (33.7%), glomerulopathies (30.7%), ciliopathies (14.0%), tubulopathies (9.2%), thrombotic microangiopathies (5.6%), and metabolic nephropathies (4
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- 2021
30. Urinary Extracellular Vesicle Protein Profiles Discriminate Different Clinical Subgroups of Children with Idiopathic Nephrotic Syndrome
- Author
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Santorelli, L, Morello, W, Barigazzi, E, Capitoli, G, Tamburello, C, Ghio, L, Crapella, B, Galimberti, S, Montini, G, Pitto, M, Raimondo, F, Santorelli, L, Morello, W, Barigazzi, E, Capitoli, G, Tamburello, C, Ghio, L, Crapella, B, Galimberti, S, Montini, G, Pitto, M, and Raimondo, F
- Abstract
Idiopathic nephrotic syndrome (INS) is the most frequent primary glomerular disease in children, displaying high grade proteinuria and oedema. The mainstay of therapy are steroids, and patients are usually classified according to the treatment response (sensitive vs. resistant). The mechanisms involved in INS pathogenesis and treatment responsiveness have not yet been identified. In this context, the analysis of urinary extracellular vesicles (UEv) is interesting, since they represent a molecular snapshot of the parental cells, offering a “fingerprint” for monitoring their status. Therefore, the aim of this study is to verify the feasibility of using UEv of INS patients as indicators of therapy response and its prediction. UEv were isolated from the urine of pediatric patients in remission after therapy; they showed characteristic electrophoresis profiles that matched specific patient subgroups. We then built a statistical model to interpret objectively each patient UEv protein profile: in particular, steroid-resistant patients cluster together with a very distinct pattern from other INS patients and controls. In conclusion, the evaluation of the UEv protein profile looks promising in the investigation of INS, showing a disease signature that might predict clinical evolution.
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- 2021
31. Prognostic Factors and Long-Term Outcome with ANCA-Associated Kidney Vasculitis in Childhood
- Author
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Calatroni, M, Consonni, F, Allinovi, M, Bettiol, A, Jawa, N, Fiasella, S, Curi, D, Abu-Rumeileh, S, Tomei, L, Fortunato, L, Gelain, E, Gianfreda, D, Oliva, E, Jeannin, G, Salviani, C, Emmi, G, Bodria, M, Sinico, R, Moroni, G, Ramirez, G, Bozzolo, E, Tombetti, E, Monti, S, Bracaglia, C, Marucci, G, Pastore, S, Esposito, P, Catanoso, M, Crapella, B, Montini, G, Roperto, R, Materassi, M, Rossi, G, Badalamenti, S, Yeung, R, Romagnani, P, Ghiggeri, G, Noone, D, Vaglio, A, Calatroni, Marta, Consonni, Filippo, Allinovi, Marco, Bettiol, Alessandra, Jawa, Natasha, Fiasella, Susanna, Curi, Dritan, Abu-Rumeileh, Sarah, Tomei, Leonardo, Fortunato, Laura, Gelain, Elena, Gianfreda, Davide, Oliva, Elena, Jeannin, Guido, Salviani, Chiara, Emmi, Giacomo, Bodria, Monica, Sinico, Renato, Moroni, Gabriella, Ramirez, Giuseppe, Bozzolo, Enrica, Tombetti, Enrico, Monti, Sara, Bracaglia, Claudia, Marucci, Giulia, Pastore, Serena, Esposito, Pasquale, Catanoso, Maria, Crapella, Barbara, Montini, Giovanni, Roperto, Rosa, Materassi, Marco, Rossi, Giovanni, Badalamenti, Salvatore, Yeung, Rae, Romagnani, Paola, Ghiggeri, Gian Marco, Noone, Damien, Vaglio, Augusto, Calatroni, M, Consonni, F, Allinovi, M, Bettiol, A, Jawa, N, Fiasella, S, Curi, D, Abu-Rumeileh, S, Tomei, L, Fortunato, L, Gelain, E, Gianfreda, D, Oliva, E, Jeannin, G, Salviani, C, Emmi, G, Bodria, M, Sinico, R, Moroni, G, Ramirez, G, Bozzolo, E, Tombetti, E, Monti, S, Bracaglia, C, Marucci, G, Pastore, S, Esposito, P, Catanoso, M, Crapella, B, Montini, G, Roperto, R, Materassi, M, Rossi, G, Badalamenti, S, Yeung, R, Romagnani, P, Ghiggeri, G, Noone, D, Vaglio, A, Calatroni, Marta, Consonni, Filippo, Allinovi, Marco, Bettiol, Alessandra, Jawa, Natasha, Fiasella, Susanna, Curi, Dritan, Abu-Rumeileh, Sarah, Tomei, Leonardo, Fortunato, Laura, Gelain, Elena, Gianfreda, Davide, Oliva, Elena, Jeannin, Guido, Salviani, Chiara, Emmi, Giacomo, Bodria, Monica, Sinico, Renato, Moroni, Gabriella, Ramirez, Giuseppe, Bozzolo, Enrica, Tombetti, Enrico, Monti, Sara, Bracaglia, Claudia, Marucci, Giulia, Pastore, Serena, Esposito, Pasquale, Catanoso, Maria, Crapella, Barbara, Montini, Giovanni, Roperto, Rosa, Materassi, Marco, Rossi, Giovanni, Badalamenti, Salvatore, Yeung, Rae, Romagnani, Paola, Ghiggeri, Gian Marco, Noone, Damien, and Vaglio, Augusto
- Abstract
Background and objectives. Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis is extremely rare in children. We report the clinico-pathological features, long-term outcomes, and prognostic factors of a large paediatric cohort of patients with ANCA-associated kidney vasculitis. Design, setting, participants, and measurements. This retrospective study included 85 consecutive patients with kidney biopsy-proven ANCA-associated vasculitis followed at tertiary referral centres in Italy and Canada. Kidney biopsies were categorised as focal, crescentic, sclerotic or mixed following Berden's classification. The prognostic significance of baseline clinical, laboratory and histological findings was analysed with respect to kidney failure or chronic kidney disease (CKD) 3-5/kidney failure. Results. Fifty-three patients had microscopic polyangiitis (62%) and 32 granulomatosis with polyangiitis (38%). Rapidly progressive glomerulonephritis was the most frequent presentation (39%); one third of the patients also had nephrotic-range proteinuria. Kidney biopsies were classified as focal in 21% of the patients, crescentic in 51%, sclerotic in 15% and mixed in 13%. Remission-induction therapies included cyclophosphamide in 78% of cases. Twenty-five patients (29%) reached kidney failure. The median time to kidney failure or last follow-up was 35 months (6-89) in the whole cohort, and 73 months (24-109) among the patients who did not reach this outcome. Cases with sclerotic histology showed significantly shorter kidney survival [HR 11.80 (95% CI 2.49-55.99)] and CKD 3-5-free survival [HR 8.88 (95% CI 2.43-32.48)] as compared with focal/mixed ones. Baseline eGFR, low serum albumin, hypertension, central nervous system complications and sclerotic histology, which reflected severe kidney involvement, were associated with both kidney failure or CKD stage 3-5/kidney failure at unadjusted analysis; no independent prognostic factors emerged at multivariate analysis. Conclusions.
- Published
- 2021
32. Management of Primary Hyperoxaluria Type 1 in Italy
- Author
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Ferraro, Pietro Manuel, Gambaro, G., Mandrile, G., Montini, G., Pasini, A., Peruzzi, L., Ferraro P. M. (ORCID:0000-0002-1379-022X), Ferraro, Pietro Manuel, Gambaro, G., Mandrile, G., Montini, G., Pasini, A., Peruzzi, L., and Ferraro P. M. (ORCID:0000-0002-1379-022X)
- Abstract
Primary hyperoxaluria type 1 is a rare genetic disease; the onset of symptoms ranges from childhood to the sixth decade of life and the disease may go unrecognized for several years. There is an urgent need for drugs able to inhibit the liver production of oxalate and to prevent the disease progression; lumasiran, an innovative molecule based on RNAi interference, is one of the most promising drugs. A group of leading Italian experts on this disease met to respond to some unmet medical needs (early diagnosis, availability of genetic tests and dosage of plasma oxalate, timing of liver transplantation, need for etiologic treatment), based on the analysis of the main scientific evidence and their personal experience. Children showing the characteristic symptoms of the disease usually undergo a metabolic screening and obtain an early diagnosis, while the experience is very limited in adults and the diagnosis difficult. It is therefore essential to increase the knowledge around this disease and the importance of metabolic and genetic screening to define a checklist of shared clinical and laboratory criteria and to establish a multidisciplinary management of potential patients. Oxalate is the cause of the disease: it is crucial to reduce both oxaluria and oxalemia through appropriate therapeutic strategies, able to prevent and/or reduce renal and systemic complications of primary type 1 hyperoxaluria. Lumasiran allows to significantly reduce the levels of oxalate both in blood and in urine, halting the course of the disease and preventing serious renal and systemic complications, if the therapy is started at an early stage of the disease.
- Published
- 2021
33. 68Ga-DOTANOC: biodistribution and dosimetry in patients affected by neuroendocrine tumors
- Author
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Pettinato, C., Sarnelli, A., Di Donna, M., Civollani, S., Nanni, C., Montini, G., Di Pierro, D., Ferrari, M., Marengo, M., and Bergamini, C.
- Published
- 2008
- Full Text
- View/download PDF
34. Cluster analysis identifies distinct pathogenetic patterns in c3 glomerulopathies/immune complex–Mediated membranoproliferative GN
- Author
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Iatropoulos, P, Daina, E, Curreri, M, Piras, R, Valoti, E, Mele, C, Bresin, E, Gamba, S, Alberti, M, Breno, M, Perna, A, Bettoni, S, Sabadini, E, Murer, L, Vivarelli, M, Noris, M, Remuzzi, G, Bottanelli, L, Donadelli, R, Cuccarolo, P, Abbate, M, Carrara, C, Cannata, A, Ferrari, S, Gaspari, F, Stucchi, N, Bassani, C, Lena, M, Omati, G, Taruscia, D, Bellantuono, R, Giordano, M, Messina, G, Caruso, M, Gotti, E, Mescia, F, Perticucci, E, Schieppati, A, Verdoni, L, Berto, M, Baraldi, O, Montini, G, Pasini, A, Passler, W, Degasperi, T, Gaggiotti, M, Gregorini, G, Miglietti, N, Guarnieri, A, Cirami, L, Roperto, R, Di Giorgio, G, Barbano, G, Innocenti, M, Ghiggeri, G, Magnasco, A, Rolla, D, Casartelli, D, Lambertini, D, Maggio, M, Cosci, P, Conti, G, Amar, K, Ardissino, G, Marinosci, A, Sinico, R, Montoli, A, Bonucchi, D, Facchini, F, Furci, L, Ferretti, A, Nuzzi, F, Pecoraro, C, Visciano, B, Canavese, C, Radin, E, Stratta, P, Nordio, M, Benetti, E, Parolin, M, Alberici, F, Manenti, L, Brugnano, R, Manenti, F, Capitanini, A, Emma, F, Massella, L, Rosa, M, Mazzon, M, Basso, E, Besso, L, Lavacca, A, Mella, A, Bertero, M, Coppo, R, Peruzzi, L, Porcellini, M, Piccoli, G, Clari, R, Pasi, A, Gangemi, C, Alfandary, H, Dagan, A, Conceiçao, M, Sameiro, F, Croze, L, Malvezzi, P, Tsygin, A, Zelan, B, Nastasi, N, Iatropoulos, Paraskevas, Daina, Erica, Curreri, Manuela, Piras, Rossella, Valoti, Elisabetta, Mele, Caterina, Bresin, Elena, Gamba, Sara, Alberti, Marta, Breno, Matteo, Perna, Annalisa, Bettoni, Serena, Sabadini, Ettore, Murer, Luisa, Vivarelli, Marina, Noris, Marina, Remuzzi, Giuseppe, Bottanelli, L., Donadelli, R., Cuccarolo, P., Abbate, M., Carrara, C., Cannata, A., Ferrari, S., Gaspari, F., Stucchi, N., Bassani, C., Lena, M., Omati, G., Taruscia, D., Bellantuono, R., Giordano, M., Messina, G., Caruso, M., Gotti, E., Mescia, F., Perticucci, E., Schieppati, A., Verdoni, L., Berto, M., Baraldi, O., Montini, G., Pasini, A., Passler, W., Degasperi, T., Gaggiotti, M., Gregorini, G., Miglietti, N., Guarnieri, A., Cirami, L., Roperto, R. M., Di Giorgio, G., Barbano, G., Innocenti, M. L. D., Ghiggeri, G. M., Magnasco, A., Rolla, D., Casartelli, D., Lambertini, D., Maggio, M., Cosci, P. M., Conti, G., Amar, K., Ardissino, G., Marinosci, A., Sinico, R. A., Montoli, A., Bonucchi, D., Facchini, F., Furci, L., Ferretti, A., Nuzzi, F., Pecoraro, C., Visciano, B., Canavese, C., Radin, E., Stratta, P., Nordio, M., Benetti, E., Parolin, M., Alberici, F., Manenti, L., Brugnano, R., Manenti, F., Capitanini, A., Emma, F., Massella, L., Rosa, M., Mazzon, M., Basso, E., Besso, L., Lavacca, A., Mella, A., Bertero, M., Coppo, R., Peruzzi, L., Porcellini, M. G., Piccoli, G. B., Clari, R., Pasi, A., Gangemi, C., Alfandary, H., Dagan, A., Conceiçao, M., Sameiro, F. M., Croze, L., Malvezzi, P., Tsygin, A., Zelan, B., Nastasi, null, Iatropoulos, P, Daina, E, Curreri, M, Piras, R, Valoti, E, Mele, C, Bresin, E, Gamba, S, Alberti, M, Breno, M, Perna, A, Bettoni, S, Sabadini, E, Murer, L, Vivarelli, M, Noris, M, Remuzzi, G, Bottanelli, L, Donadelli, R, Cuccarolo, P, Abbate, M, Carrara, C, Cannata, A, Ferrari, S, Gaspari, F, Stucchi, N, Bassani, C, Lena, M, Omati, G, Taruscia, D, Bellantuono, R, Giordano, M, Messina, G, Caruso, M, Gotti, E, Mescia, F, Perticucci, E, Schieppati, A, Verdoni, L, Berto, M, Baraldi, O, Montini, G, Pasini, A, Passler, W, Degasperi, T, Gaggiotti, M, Gregorini, G, Miglietti, N, Guarnieri, A, Cirami, L, Roperto, R, Di Giorgio, G, Barbano, G, Innocenti, M, Ghiggeri, G, Magnasco, A, Rolla, D, Casartelli, D, Lambertini, D, Maggio, M, Cosci, P, Conti, G, Amar, K, Ardissino, G, Marinosci, A, Sinico, R, Montoli, A, Bonucchi, D, Facchini, F, Furci, L, Ferretti, A, Nuzzi, F, Pecoraro, C, Visciano, B, Canavese, C, Radin, E, Stratta, P, Nordio, M, Benetti, E, Parolin, M, Alberici, F, Manenti, L, Brugnano, R, Manenti, F, Capitanini, A, Emma, F, Massella, L, Rosa, M, Mazzon, M, Basso, E, Besso, L, Lavacca, A, Mella, A, Bertero, M, Coppo, R, Peruzzi, L, Porcellini, M, Piccoli, G, Clari, R, Pasi, A, Gangemi, C, Alfandary, H, Dagan, A, Conceiçao, M, Sameiro, F, Croze, L, Malvezzi, P, Tsygin, A, Zelan, B, Nastasi, N, Iatropoulos, Paraskevas, Daina, Erica, Curreri, Manuela, Piras, Rossella, Valoti, Elisabetta, Mele, Caterina, Bresin, Elena, Gamba, Sara, Alberti, Marta, Breno, Matteo, Perna, Annalisa, Bettoni, Serena, Sabadini, Ettore, Murer, Luisa, Vivarelli, Marina, Noris, Marina, Remuzzi, Giuseppe, Bottanelli, L., Donadelli, R., Cuccarolo, P., Abbate, M., Carrara, C., Cannata, A., Ferrari, S., Gaspari, F., Stucchi, N., Bassani, C., Lena, M., Omati, G., Taruscia, D., Bellantuono, R., Giordano, M., Messina, G., Caruso, M., Gotti, E., Mescia, F., Perticucci, E., Schieppati, A., Verdoni, L., Berto, M., Baraldi, O., Montini, G., Pasini, A., Passler, W., Degasperi, T., Gaggiotti, M., Gregorini, G., Miglietti, N., Guarnieri, A., Cirami, L., Roperto, R. M., Di Giorgio, G., Barbano, G., Innocenti, M. L. D., Ghiggeri, G. M., Magnasco, A., Rolla, D., Casartelli, D., Lambertini, D., Maggio, M., Cosci, P. M., Conti, G., Amar, K., Ardissino, G., Marinosci, A., Sinico, R. A., Montoli, A., Bonucchi, D., Facchini, F., Furci, L., Ferretti, A., Nuzzi, F., Pecoraro, C., Visciano, B., Canavese, C., Radin, E., Stratta, P., Nordio, M., Benetti, E., Parolin, M., Alberici, F., Manenti, L., Brugnano, R., Manenti, F., Capitanini, A., Emma, F., Massella, L., Rosa, M., Mazzon, M., Basso, E., Besso, L., Lavacca, A., Mella, A., Bertero, M., Coppo, R., Peruzzi, L., Porcellini, M. G., Piccoli, G. B., Clari, R., Pasi, A., Gangemi, C., Alfandary, H., Dagan, A., Conceiçao, M., Sameiro, F. M., Croze, L., Malvezzi, P., Tsygin, A., Zelan, B., and Nastasi, null
- Abstract
Membranoproliferative GN (MPGN) was recently reclassified as alternative pathway complement–mediated C3 glomerulopathy (C3G) and immune complex–mediated membranoproliferative GN (IC-MPGN). However, genetic and acquired alternative pathway abnormalities are also observed in IC-MPGN. Here, we explored the presence of distinct disease entities characterized by specific pathophysiologic mechanisms. We performed unsupervised hierarchical clustering, a data-driven statistical approach, on histologic, genetic, and clinical data and data regarding serum/plasma complement parameters from 173 patients with C3G/IC-MPGN. This approach divided patients into four clusters, indicating the existence of four different pathogenetic patterns. Specifically, this analysis separated patients with fluid-phase complement activation (clusters 1–3) who had low serum C3 levels and a high prevalence of genetic and acquired alternative pathway abnormalities from patients with solid-phase complement activation (cluster 4) who had normal or mildly altered serum C3, late disease onset, and poor renal survival. In patients with fluid-phase complement activation, those in clusters 1 and 2 had massive activation of the alternative pathway, including activation of the terminal pathway, and the highest prevalence of subendothelial deposits, but those in cluster 2 had additional activation of the classic pathway and the highest prevalence of nephrotic syndrome at disease onset. Patients in cluster 3 had prevalent activation of C3 convertase and highly electron-dense intramembranous deposits. In addition, we provide a simple algorithm to assign patients with C3G/IC-MPGN to specific clusters. These distinct clusters may facilitate clarification of disease etiology, improve risk assessment for ESRD, and pave the way for personalized treatment.
- Published
- 2018
35. Post-transplant recurrence of steroid resistant nephrotic syndrome in children: the Italian experience
- Author
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Morello, W, Puvinathan, S, Puccio, G, Ghiggeri, G, Dello Strologo, L, Peruzzi, L, Murer, L, Cioni, M, Guzzo, I, Cocchi, E, Benetti, E, Testa, S, Ghio, L, Caridi, G, Cardillo, M, Torelli, R, Montini, G, Morello, William, Puvinathan, Sairaj, Puccio, Giuseppe, Ghiggeri, Gian Marco, Dello Strologo, Luca, Peruzzi, Licia, Murer, Luisa, Cioni, Michela, Guzzo, Isabella, Cocchi, Enrico, Benetti, Elisa, Testa, Sara, Ghio, Luciana, Caridi, Gianluca, Cardillo, Massimo, Torelli, Rosanna, Montini, Giovanni, Morello, W, Puvinathan, S, Puccio, G, Ghiggeri, G, Dello Strologo, L, Peruzzi, L, Murer, L, Cioni, M, Guzzo, I, Cocchi, E, Benetti, E, Testa, S, Ghio, L, Caridi, G, Cardillo, M, Torelli, R, Montini, G, Morello, William, Puvinathan, Sairaj, Puccio, Giuseppe, Ghiggeri, Gian Marco, Dello Strologo, Luca, Peruzzi, Licia, Murer, Luisa, Cioni, Michela, Guzzo, Isabella, Cocchi, Enrico, Benetti, Elisa, Testa, Sara, Ghio, Luciana, Caridi, Gianluca, Cardillo, Massimo, Torelli, Rosanna, and Montini, Giovanni
- Abstract
Background: Steroid resistant nephrotic syndrome (SRNS) is a frequent cause of end stage renal disease in children and post-transplant disease recurrence is a major cause of graft loss. Methods: We identified all children with SRNS who underwent renal transplantation in Italy, between 2005 and 2017. Data were retrospectively collected for the presence of a causative gene mutation, sex, histology, duration of pre-transplant dialysis, age at onset and transplant, HLA matching, recurrence, therapy for recurrence, and graft survival. Results: 101 patients underwent a first and 22 a second renal transplant. After a median follow-up of 58.5 months, the disease recurred on the first renal transplant in 53.3% of patients with a non-genetic and none with a genetic SRNS. Age at transplant > 9 years and the presence of at least one HLA-AB match were independent risk factors for recurrence. Duration of dialysis was longer in children with relapse, but did not reach statistical significance. Overall, 24% of patients lost the first graft, with recurrence representing the commonest cause. Among 22 patients who underwent a second transplant, 5 suffered of SRNS recurrence. SRNS relapsed in 5/9 (55%) patients with disease recurrence in their first transplant and 2 of them lost the second graft. Conclusions: Absence of a causative mutation represents the major risk factor for post-transplant recurrence in children with SRNS, while transplant can be curative in genetic SRNS. A prolonged time spent on dialysis before transplantation has no protective effect on the risk of relapse and should not be encouraged. Retransplantation represents a second chance after graft loss for recurrence.
- Published
- 2020
36. Renal hemodynamic effect of tacrolimus in renal transplanted children
- Author
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Dello Strologo, L., Pontesilli, C., Montini, G., Ginevri, F., Ardissino, G., Campagnano, P., Pastore, A., Federici, G., and Rizzoni, G.
- Published
- 2001
- Full Text
- View/download PDF
37. Clinical and Molecular Markers of Chronic Interstitial Nephropathy in Congenital Unilateral Ureteropelvic Junction Obstruction
- Author
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Murer, L., Benetti, E., Centi, S., Della Vella, M., Artifoni, L., Capizzi, A., Zucchetta, P., Del Prete, D., Carasi, C., Montini, G., Rigamonti, W., and Zaccello, G.
- Published
- 2006
- Full Text
- View/download PDF
38. I processi di nullità matrimoniale nella riforma di Papa Francesco, Documenti e Studi di Synaxis 30 Gianluca Belfiore
- Author
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Montini, G. Paolo
- Published
- 2018
39. The Art of Good Governance. A Guide to the Administrative Procedure for Just Decision-Making in the Catholic Church William L. Daniel
- Author
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Montini, G. Paolo
- Published
- 2018
40. A phase II trial of short course fludarabine, mitoxantrone, rituximab followed by 90Y-ibritumomab tiuxetan in untreated intermediate/high-risk follicular lymphoma
- Author
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Zinzani, P. L., Tani, M., Pulsoni, A., De Renzo, A., Stefoni, V., Broccoli, A., Montini, G. C., Fina, M., Pellegrini, C., Gandolfi, L., Cavalieri, E., Torelli, F., Scopinaro, F., Argnani, L., Quirini, F., Derenzini, E., Rossi, M., Pileri, S., Fanti, S., and Baccarani, M.
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- 2012
- Full Text
- View/download PDF
41. Rapid determination of creatinine in serum and urine by ion-pair high-performance liquid chromatography
- Author
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Marsilio, R., Dall’Amico, R., Giordano, D., Murer, L., Montini, G., Ros, M., Dussini, N., Zacchello, G., Bacelle, L., and Plebani, M.
- Published
- 1999
- Full Text
- View/download PDF
42. P060 - Semi-closed-circuit vacuum-assisted mini percutaneous nephrolithotomy in pediatric patients: The initial experience by two tertiary referral centers
- Author
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Sampogna, G., Gallioli, A., Berrettini, A., Zanetti, S.P., Llorens, E., Quiroz, Y., Gnech, M., De Marco, E.A., Minoli, D.G., Taroni, F., De Lorenzis, E., Montini, G., Manzoni, G., Bujons, A., and Montanari, E.
- Published
- 2019
- Full Text
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43. Surgical and medical management of stone disease in children under 2 years
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Gnech, M., primary, Taroni, F., additional, De Marco, E.A., additional, Mancuso, M.C., additional, Minoli, D., additional, Capone, V., additional, Montini, G., additional, Montanari, E., additional, Manzoni, G., additional, and Berrettini, A., additional
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- 2020
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44. Mini-PCNL with a semi-closed-circuit vacuum-assisted system in pediatric patients treated by two European tertiary referral centers
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Sampogna, G., primary, Gallioli, A., additional, Berrettini, A., additional, Zanetti, S.P., additional, Llorens, E., additional, Quiróz, Y., additional, Gnech, M., additional, De Marco, E.A., additional, Minoli, D.G., additional, Taroni, F., additional, De Lorenzis, E., additional, Montini, G., additional, Manzoni, G., additional, Bujons Tur, A., additional, and Montanari, E., additional
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- 2020
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45. Genome-wide association study identifies CDH12 as candidate gene for renal injury in patients with posterior urethral valves
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Van Der Zanden, L.F.M., primary, Van Rooij, I.A.L.M., additional, Quaedackers, J.S.L.T., additional, Nijman, R., additional, Steffens, M., additional, De Wall, L.L.L., additional, Bongers, E.M.H.F., additional, Franz, S., additional, Kirchner, M., additional, Behnisch, R., additional, Bayazit, A.K., additional, Caliskan, S., additional, Obrycki, L., additional, Montini, G., additional, Duzova, A., additional, Wuttke, M., additional, Jennings, R., additional, Hanley, N.A., additional, Milmoe, N.J., additional, Winyard, P.J.D., additional, Renkema, K.Y., additional, Schreuder, M.F., additional, Roeleveld, N., additional, and Feitz, W.F.J., additional
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- 2020
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46. Prevention of ill-health: new guidelines to promote and maintain well-being
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Chiodelli, G., Uberti, M., Galli, M. L., Capellini, R., Montini, G., Croce, L., Fioriti, F., Leoni, M., and Corti, S.
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- 2010
47. Effects of nutritional Vitamin D supplementation on markers of bone and mineral metabolism in children with chronic kidney disease
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Cakar, N., Basin, E., Yalcinkaya, F., KARABAY BAYAZIT, AYSUN, Anarat, A., Laube, G., Bucher, B., Peco-Antic, A., Texeira, A., Donmez, O., Balat, A., Szczepanska, M., Niemirska, A., Litwin, M., Urasinski, T., Tkaczyk, M., Kiyak, A., Drodz, D., Zurowska, A., Azukaitis, K., Jankauskiene, A., Picca, S., Matteucci, C., Vidal, E., Testa, S., Lugani, F., Montini, G., Wigger, M., Kranz, B., Jeck, N., Wygoda, S., Pohl, M., Wuehl, E., Doyon, A., Schaefer, F., Thurn, D., Melk, A., Kemper, M., ÇALIŞKAN, Salim, Gimpel, C., Buescher, R., Galiano, M., Habbig, S., Querfeld, U., Zalosczyk, A., Fischbach, M., Ranchin, B., Harambat, J., Canpolat, N., Dusek, J., Arbeiter, K., Cortina, G., Haffner, Dieter, Candan, C., Schaefer, Franz, Sander, Anja, Leifheit-Nestler, Maren, Civilibal, M., Querfeld, Uwe, Melk, Anette, Rosales, Alejandra, Candan, Cengiz, Soylemezoglu, Oguz, Zaloszyc, Ariane, Habbig, Sandra, ALPAY, HARİKA, Emre, S., Alpay, H., Ozcelik, G., Kiyak, Aysel, Harambat, Jerome, Mir, S., Sozeri, B., Yalcinkaya, Fatos, Yilmaz, Ebru, Azukaitis, Karolis, Yavascan, O., DÜZOVA, ALİ, CANPOLAT, Nur, Niemirska, Anna, Tabel, Y., Ertan, P., Thurn, Daniela, KAPLAN BULUT, İPEK, Lerch, Christian, Yilmaz, E., Prytula, A., Bachetta, J., Haffner, D., Klaus, G., Gessner, M., Schmitt, C. P., Stabouli, S., Reusz, G., Verrina, E., Groothoff, J., Tondel, C., Gamero, M. A., Petrosyan, E., Dursun, I., Erdogan, H., Bilginer, Y., Soylemezoglu, O., Shroff, Rukshana, Shroff, R., Wan, Mandy, Bakkaloglu, SEVCAN AZİME, Aitkenhead, Helen, Rees, Lesley, Çukurova Üniversitesi, AGEM - Amsterdam Gastroenterology Endocrinology Metabolism, ARD - Amsterdam Reproduction and Development, Paediatric Nephrology, AGEM - Inborn errors of metabolism, APH - Quality of Care, APH - Methodology, Department of Pediatric Kidney, Liver and Metabolic Diseases, Hannover Medical School Children's Hospital, Hannover, Germany, Renal Unit, Great Ormond Street Hospital for Children, London, United Kingdom, Department of Chemical Pathology, Great Ormond Street Hospital for Children, London, United Kingdom, Department of Pediatric Nephrology, Ege University, Bornova, Izmir, Turkey, Department of Pediatric Nephrology, Faculty of Medicine, Cukurova University, Adana, Turkey, Department of Nephrology, Kidney Transplantation and Arterial Hypertension, Children's Memorial Health Institute, Warsaw, Poland, Department of Pediatrics, Istanbul University Cerrahpasa, Faculty of Medicine, Istanbul, Turkey, Division of Pediatric Nephrology, Hacettepe University, Faculty of Medicine, Ankara, Turkey, Clinic of Pediatrics, Faculty of Medicine, Vilnius University, Vilnius, Lithuania, Department of Pediatric Nephrology, Sanliurfa Children's Hospital, Sanliurfa, Turkey, Department of Pediatric Nephrology, School of Medicine, Ankara University, Ankara, Turkey, Department of Pediatrics, Bordeaux University Hospital, Bordeaux, France, Department of Pediatric Nephrology, Yenimahalle Egitim ve Arastirma Hastanesi Bakirkoy, Istanbul, Turkey, Department of Pediatric Nephrology, Marmara University School of Medicine, Istanbul, Turkey, Division of Pediatric Nephrology, University Children's, Adolescent's Hospital, Cologne, Germany, Pole Médico-Chirurgical de Pédiatrie, Service de Pédiatrie I, Hôpitaux Universitaires de Strasbourg, Strasbourg, France, Department of Pediatric Nephrology, Gazi University Hospital, Ankara, Turkey, Department of Pediatric Nephrology, Göztepe Egitim ve Arastirma Hastanesi, Cocuk Klinigi, Göztepe, Istanbul, Turkey, Department of Pediatrics, Innsbruck Medical University, Innsbruck, Austria, Clinic of Pediatric Nephrology, Charite Children's Hospital, Berlin, Germany, Institute of Medical Biometry and Informatics, University Heidelberg, Heidelberg, Germany, Division of Pediatric Nephrology, Heidelberg, Germany, Children's Hospital, Innsbruck, Austria, University Children's Hospital, Vienna, Austria, University Hospital Motol, Prague, Czech Republic, Hôpital des Enfants, Bordeaux, France, Hôpital Femme Mère Enfant, Université de Lyon, France, Hôpital de Hautepierre, Strasbourg, France, Charité Children's Hospital, Berlin, Germany, University Children's Hospital, Cologne, Germany, University Children's Hospital, Erlangen, Germany, University Children's Hospital, Essen, Germany, Center for Pediatrics and Adolescent Medicine, Freiburg, Germany, UKE University Children's Hospital, Hamburg, Germany, Hannover Medical School, Hannover, Germany, Center for Pediatrics and Adolescent Medicine, Heidelberg, Germany, Center for Pediatrics and Adolescent Medicine, Jena, Germany, City Hospital St. Georg, Leipzig, Germany, KfH Kidney Center for Children, Marburg, Germany, University Children's Hospital, Münster, Germany, Children's Hospital, Rostock, Germany, S. Orsola-Malpighi Hospital, Bologna, Italy, Istituto Giannina Gaslini, Genova, Italy, Fondazione Ospedale Maggiore Policlinico, Milano, Italy, Pediatric Nephrology, Dialysis and Transplant Unit, Padova, Italy, Ospedale Bambino Gesú, Rome, Italy, University Children's Hospital, Vilnius, Lithuania, Pediatric and Adolescent Nephrology, Gdansk, Poland, University Children's Hospital, Krakow, Poland, Polish Mothers Memorial Hospital Research Institute, Lodz, Poland, Clinic of Pediatrics, Szczecin, Poland, Children's Memorial Health Institute, Warsaw, Poland, Zabrze, Poland, Hospital Sao Joao, Porto, Portugal, University Children's Hospital, Belgrade, Serbia, Inselspital, Bern, Switzerland, University Children's Hospital, Zurich, Switzerland, Cukurova University, Adana, Turkey, University Faculty of Medicine, Ankara, Turkey, Baskent University, Faculty of Medicine, Ankara, Turkey, Diskapi Children's Hospital, Ankara, Turkey, Gazi University Hospital, Ankara, Turkey, Hacettepe Medical Faculty, Ankara, Turkey, Dortcelik Children's Hospital, Bursa, Turkey, Uludag University, Bursa, Turkey, University of Gaziantep, Turkey, Bakirkoy Children's Hospital, Istanbul, Turkey, Istanbul University Cerrahpasa, Faculty of Medicine, Istanbul, Turkey, Goztepe Educational and Research Hospital, Istanbul, Turkey, Haseki Educational and Research Hospital, Istanbul, Turkey, Istanbul Medical Faculty, Istanbul, Turkey, Marmara University Medical Faculty, Istanbul, Turkey, Sisli Educational and Research Hospital, Istanbul, Turkey, Ege University Medical Faculty, Izmir, Turkey, Tepecik Training and Research Hospital, Izmir, Turkey, Inonu University, Malatya, Turkey, Celal Bayar University, Manisa, Turkey, Ghent University, Utopaed, Belgium, University Children's Hospital, Lyon, France, University Children's Hospital, Marburg, Germany, University Children's Hospital, Tübingen, Germany, University Children's Hospital, Thessaloniki, Greece, Semmelweis University, Budapest, Hungary, G. Gaslini Institute, Genoa, Italy, Academic Medical Center, Amsterdam, Netherlands, University Children's Hospital, Bergen, Norway, Reina Sofia Universitary Hospital, Cordoba, Spain, Russian National Research Medical University, Moscow, Russian Federation, Erciyes University, Faculty of Medicine, Kayseri, Turkey, Uludağ Üniversitesi/Tıp Fakültesi/Çocuk Sağlığı ve Hastalıkları Anabilim Dalı., Dönmez, Osman, AAA-8778-2021, Lerch, Christian, Shroff, Rukshana, Wan, Mandy, Rees, Lesley, Aitkenhead, Helen, Bulut, Ipek Kaplan, Thurn, Daniela, Bayazit, Aysun Karabay, Niemirska, Anna, Canpolat, Nur, Duzova, Ali, Azukaitis, Karolis, Yilmaz, Ebru, Yalcinkaya, Fatos, Harambat, Jerome, Kiyak, Aysel, Alpay, Harika, Habbig, Sandra, Zaloszyc, Ariane, Soylemezoglu, Oguz, Candan, Cengiz, Rosales, Alejandra, Melk, Anette, Querfeld, Uwe, Leifheit-Nestler, Maren, Sander, Anja, Schaefer, Franz, Haffner, Dieter, Cortina, G., Arbeiter, K., Dusek, J., Harambat, J., Ranchin, B., Fischbach, M., Zalosczyk, A., Querfeld, U., Habbig, S., Galiano, M., Buescher, R., Gimpel, C., Kemper, M., Melk, A., Thurn, D., Schaefer, F., Doyon, A., Wuehl, E., Pohl, M., Wygoda, S., Jeck, N., Kranz, B., Wigger, M., Montini, G., Lugani, F., Testa, S., Vidal, E., Matteucci, C., Picca, S., Jankauskiene, A., Azukaitis, K., Zurowska, A., Drodz, D., Tkaczyk, M., Urasinski, T., Litwin, M., Niemirska, A., Szczepanska, M., Texeira, A., Peco-Antic, A., Bucher, B., Laube, G., Anarat, A., Bayazit, A. K., Yalcinkaya, F., Basin, E., Cakar, N., Soylemezoglu, O., Duzova, A., Bilginer, Y., Erdogan, H., Donmez, O., Balat, A., Kiyak, A., Caliskan, S., Canpolat, N., Candan, C., Civilibal, M., Emre, S., Alpay, H., Ozcelik, G., Mir, S., Sozeri, B., Yavascan, O., Tabel, Y., Ertan, P., Yilmaz, E., Shroff, R., Prytula, A., Bachetta, J., Haffner, D., Klaus, G., Gessner, M., Schmitt, C. P., Stabouli, S., Reusz, G., Verrina, E., Groothoff, J., Tondel, C., Gamero, M. A., Petrosyan, E., Bakkaloglu, S. A., and Dursun, I.
- Subjects
Male ,Fibroblast growth factor 23 ,Comorbidity ,urologic and male genital diseases ,Dietary supplement ,0302 clinical medicine ,Chronic kidney disease ,Chronic ,Child ,Klotho ,Children ,Clinical outcome ,Serum sclerostin ,Double blind procedure ,Vitamins ,Multicenter study ,female genital diseases and pregnancy complications ,3. Good health ,Clinical trial ,Bone and mineral metabolism ,Vitamin D deficiency ,Vitamin D supplementation ,Adolescent ,Alkaline Phosphatase ,Biomarkers ,Bone Density ,Double-Blind Method ,Female ,Fibroblast Growth Factors ,Follow-Up Studies ,Glomerular Filtration Rate ,Humans ,Renal Insufficiency, Chronic ,Vitamin D ,Dietary Supplements ,Blood ,Randomized controlled trial ,Nephrology ,Cohort analysis ,Human ,medicine.medical_specialty ,Mineral metabolism ,Sclerostin ,Clinical article ,Diet supplementation ,Article ,vitamin D deficiency ,Ergocalciferol ,03 medical and health sciences ,Cholecalciferol supplementation ,CKD ,Vitamin D and neurology ,Follow up ,medicine.disease ,Renal-failure ,Clinical effectiveness ,Endocrinology ,chemistry ,School child ,Chronic kidney failure ,Physiology ,Beta Glucuronidase ,Klotho Protein ,Chronic Kidney Disease-Mineral and Bone Disorder ,Fibroblast growth factor ,030232 urology & nephrology ,Medizin ,030204 cardiovascular system & hematology ,Growth-factor 23 ,chemistry.chemical_compound ,Randomized controlled trial (topic) ,Urology & nephrology ,Estimated glomerular filtration rate ,Renal Insufficiency ,Alpha-klotho ,Protein expression level ,Vitamin supplementation ,Priority journal ,Double-blind ,Klotho protein ,Glomerulus filtration rate ,medicine.drug ,Vitamin ,Bone metabolism ,Pathophysiology ,Hemodialysis-patients ,Internal medicine ,medicine ,Renal insufficiency, chronic ,FGF-23 ,Transplantation ,business.industry ,Alkaline phosphatase bone isoenzyme ,Fibroblast Growth Factor-23 ,Biological marker ,Metabolism ,business ,Controlled study ,Kidney disease - Abstract
PubMedID: 29481636 Background: We investigated the effects of nutritional vitamin D supplementation on markers of bone and mineral metabolism, i.e. serum levels of fibroblast growth factor 23(FGF23), Klotho, bone alkaline phosphatase (BAP) and sclerostin, in two cohorts with chronic kidney disease (CKD). Methods: In all, 80 vitamin D-deficient children were selected: 40 with mild to moderate CKD from the ERGO study, a randomized trial of ergocalciferol supplementation [estimated glomerular filtration rate (eGFR) 55 mL/min/1.73 m 2 ], and 40 with advanced CKD from the observational Cardiovascular Comorbidity in Children with Chronic Kidney Disease (4C) study (eGFR 24 mL/min/1.73 m 2 ). In each study, vitamin D supplementation was started in 20 children and 20 matched children not receiving vitamin D served as controls. Measures were taken at baseline and after a median period of 8 months. Age- and gender-related standard deviation scores (SDSs) were calculated. Results: Before vitamin D supplementation, children in the ERGO study had normal FGF23 (median 0.31 SDS) and BAP (-0.10 SDS) but decreased Klotho and sclerostin (-0.77 and - 1.04 SDS, respectively), whereas 4C patients had increased FGF23 (3.87 SDS), BAP (0.78 SDS) and sclerostin (0.76 SDS) but normal Klotho (-0.27 SDS) levels. Vitamin D supplementation further increased FGF23 in 4C but not in ERGO patients. Serum Klotho and sclerostin normalized with vitamin D supplementation in ERGO but remained unchanged in 4C patients. BAP levels were unchanged in all patients. In the total cohort, significant effects of vitamin D supplementation were noted for Klotho at eGFR 40-70mL/min/1.73m 2 . Conclusions: Vitamin D supplementation normalized Klotho and sclerostin in children with mild to moderate CKD but further increased FGF23 in advanced CKD. © The Author(s) 2018. Published by Oxford University Press on behalf of ERA-EDTA. All rights reserved. American Society of Pediatric Nephrology: ESPN 2014.3 This work was supported by the European Society for Pediatric Nephrology (reference number ESPN 2014.3), KfH Foundation for Preventive Medicine and ERA-EDTA (to D.H.).
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- 2018
48. 11C-Methionine PET/CT in Central Nervous System Tumours: A Review
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Maffione, A. M., Nanni, C., Ambrosini, V., Trespidi, S., Lopci, E., Allegri, V., Castellucci, P., Montini, G., Boschi, S., and Fanti, S.
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- 2009
49. ADPedKD: A Global Online Platform on the Management of Children With
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De Rechter, S, Bockenhauer, D, Guay-Woodford, LM, Liu, I, Mallett, AJ, Soliman, NA, Sylvestre, LC, Schaefer, F, Liebau, MC, Mekahli, D, Adamczyk, P, Akinci, N, Alpay, H, Ardelean, C, Ayasreh, N, Aydin, Z, Bael, A, Baudouin, V, Bayrakci, US, Bensman, A, Bialkevich, H, Biebuyck, A, Boyer, O, Bjanid, O, Brylka, A, Caliskan, S, Cambier, A, Camelio, A, Carbone, V, Charbit, M, Chiodini, B, Chirita, A, Cicek, N, Cerkauskiene, R, Collard, L, Conceicao, M, Constantinescu, I, Couderc, A, Crapella, B, Cvetkovic, M, Dima, B, Diomeda, F, Docx, M, Dolan, N, Dossier, C, Drozdz, D, Drube, J, Dunand, O, Dusan, P, Eid, LA, Emma, F, Hernandez, ME, Fila, M, Furlano, M, Gafencu, M, Ghuysen, M, Giani, M, Giordano, M, Girisgen, I, Godefroid, N, Godron-Dubrasquet, A, Gojkovic, I, Gonzalez, E, Gokce, I, Groothoff, JW, Guarino, S, Guffens, A, Hansen, P, Harambat, J, Haumann, S, He, G, Heidet, L, Helmy, R, Hemery, F, Hooman, N, Ilanas, B, Jankauskiene, A, Janssens, P, Karamaria, S, Kazyra, I, Koenig, J, Krid, S, Krug, P, Kwon, V, La Manna, A, Leroy, V, Litwin, M, Lombet, J, Longo, G, Lungu, AC, Mallawaarachchi, A, Marin, A, Marzuillo, P, Massella, L, Mastrangelo, A, McCarthy, H, Miklaszewska, M, Moczulska, A, Montini, G, Morawiec-Knysak, A, Morin, D, Murer, L, Negru, I, Nobili, F, Obrycki, L, Otoukesh, H, Ozcan, S, Pape, L, Papizh, S, Parvex, P, Pawlak-Bratkowska, M, Prikhodina, L, Prytula, A, Quinlan, C, Raes, A, Ranchin, B, Ranguelov, N, Repeckiene, R, Ronit, C, Salomon, R, Santagelo, R, Saygili, SK, Schaefer, S, Schreuder, M, Schurmans, T, Seeman, T, Segers, N, Sinha, M, Snauwaert, E, Spasojevic, B, Stabouli, S, Stoica, C, Stroescu, R, Szczepanik, E, Szczepanska, M, Taranta-Janusz, K, Teixeira, A, Thumfart, J, Tkaczyk, M, Torra, R, Torres, D, Tram, N, Utsch, B, Vande Walle, J, Vieux, R, Vitkevic, R, Wilhelm-Bals, A, Wuhl, E, Yildirim, ZY, Yuksel, S, and Zachwieja, K
- Abstract
Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. Methods: Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. Discussion: The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease. C1 [De Rechter, Stephanie; Mekahli, Djalila] Univ Hosp Leuven, Dept Pediat Nephrol, Herestr 49, B-3000 Leuven, Belgium. [De Rechter, Stephanie; Mekahli, Djalila] Katholieke Univ Leuven, Dept Dev & Regenerat, PKD Res Grp, Leuven, Belgium. [Bockenhauer, Detlef] UCL Ctr Nephrol, London, England. [Bockenhauer, Detlef] Great Ormond St Hosp NHS Fdn Trust, London, England. [Guay-Woodford, Lisa M.] Childrens Natl Hlth Syst, Ctr Translat Sci, Washington, DC USA. [Liu, Isaac] Natl Univ Hlth Syst, Khoo Teck Puat Natl Univ, Childrens Med Inst, Singapore, Singapore. [Mallett, Andrew J.] Royal Brisbane & Womens Hosp, Kidney Hlth Serv, Brisbane, Qld, Australia. [Mallett, Andrew J.] Royal Brisbane & Womens Hosp, Conjoint Renal Res Lab, Brisbane, Qld, Australia. [Mallett, Andrew J.] Univ Queensland, Fac Med, Brisbane, Qld, Australia. [Mallett, Andrew J.] Univ Queensland, Inst Mol Biosci, Brisbane, Qld, Australia. [Mallett, Andrew J.] KidGen Collaborat & Australian Genom Hlth Allianc, Melbourne, Vic, Australia. [Soliman, Neveen A.] Cairo Univ, Ctr Pediat Nephrol & Transplantat, Kasr Al Ainy Sch Med, Dept Pediat, Cairo, Egypt. [Sylvestre, Lucimary C.] Hosp Pequeno Principe, Curitiba, Parana, Brazil. [Schaefer, Franz] Heidelberg Univ, Ctr Pediat & Adolescent Med, Div Pediat Nephrol, Med Ctr, Heidelberg, Germany. [Liebau, Max C.] Univ Hosp Cologne, Dept Pediat, Cologne, Germany. [Liebau, Max C.] Univ Hosp Cologne, Ctr Mol Med, Cologne, Germany. [Adamczyk, P.; Bjanid, O.; Brylka, A.; Morawiec-Knysak, A.; Szczepanska, M.] Dept Pediat, Zabrze, Poland. [Akinci, N.] Sariyer SISLI Hamidiye Etfal Res & Educ Hosp, Istanbul, Turkey. [Alpay, H.; Cicek, N.; Gokce, I] Marmara Univ, Sch Med, Div Pediat Nephrol, Istanbul, Turkey. [Ardelean, C.; Chirita, A.; Gafencu, M.; Stroescu, R.] Timisoara Children Hosp, Timisoara, Romania. [Ayasreh, N.; Furlano, M.; Torra, R.] Fundacio Puigvert, Barcelona, Spain. [Aydin, Z.; Bayrakci, U. S.] Ankara Univ Hlth Sci, Child Hlth & Dis, Ankara, Turkey. [Bael, A.; Docx, M.; Segers, N.] Koningin Paola Kinderziekenhuis Antwerpen, Antwerp, Belgium. [Baudouin, V; Cambier, A.; Couderc, A.; Dossier, C.; Kwon, V] Hop Robert Debre, AP HP, Paris, France. [Bensman, A.; Biebuyck, A.; Boyer, O.; Charbit, M.; Heidet, L.; Krid, S.; Krug, P.; Salomon, R.] Pediat Nephrol Necker Hosp, Paris, France. [Bialkevich, H.; Kazyra, I] 2nd City Childrens Clin Hosp, Natl Ctr Pediat Nephrol & RRT, Minsk, BELARUS. [Caliskan, S.; Ozcan, S.; Saygili, S. K.] Istanbul Cerrahpasa Fac Med, Istanbul, Turkey. [Camelio, A.; Nobili, F.; Vieux, R.] CHU Besancon, Besancon, France. [Carbone, V; Diomeda, F.; Torres, D.] Pediat Nephrol Unit Bari, Bari, Italy. [Chiodini, B.] HUDERF, Brussels, Belgium. [Collard, L.] CHR La Citadelle, Liege, Belgium. [Conceicao, M.; Teixeira, A.] Ctr Hosp Porto, Ctr Materno Infantil Norte, Porto, Portugal. [Constantinescu, I; Lungu, A. C.; Marin, A.; Negru, I; Stroescu, R.] Fundeni Clin Inst, Bucharest, Romania. [Crapella, B.; Giani, M.; Mastrangelo, A.; Montini, G.] Fdn IRCCS Ca Granda, Pediat Nephrol Dialysis & Transplant Unit, Milan, Italy. [Cvetkovic, M.; Gojkovic, I] Univ Childrens Hosp, Belgrade, Serbia. [Dima, B.] Clin Europe Hop St Elisabeth, Brussels, Belgium. [Dolan, N.] Our Ladys Childrens Hosp, Dublin, Ireland. [Drozdz, D.; Miklaszewska, M.; Zachwieja, K.] Jagiellonian Univ, Med Coll Cracow, Pediat Nephrol & Hypertens, Krakow, Poland. [Drube, J.; Pape, L.] Hannover Med Sch, Hannover, Germany. [Dunand, O.; Leroy, V] Pediat Nephrol Unit St Denis, St Denis, Reunion, France. [Dusan, P.; Spasojevic, B.; Stabouli, S.] Aristotle Univ Thessaloniki, Dept Pediat, Thessaloniki, Greece. [Eid, L. A.] Dubai Hosp, Pediat Nephrol Dept, Dubai, U Arab Emirates. [Emma, F.; Massella, L.] Bambino Gesu Pediat Hosp, Rome, Italy. [Espino Hernandez, M.] Hosp Infantil 12 Octubre Madrid, Madrid, Spain. [Fila, M.; Hemery, F.; Morin, D.] CHU Arnaud Villeneuve, Montpellier, France. [Ghuysen, Ms] CHU Liege, Liege, Belgium. [Giordano, M.] Pediat Nephrol Unit, Bari, Italy. [Girisgen, I; Yuksel, S.] Pamukkale Univ, Med Fac, Dept Pediat Nephrol, Denizli, Turkey. [Godefroid, N.; Ranguelov, N.] Clin Univ St Luc, Brussels, Belgium. [Godron-Dubrasquet, A.; Harambat, J.; Ilanas, B.] Bordeaux Univ Childrens Hosp, Bordeaux, France. [Gonzalez, E.] Childrens Univ Hosp, Geneva, Switzerland. [Groothoff, J. W.] Emma Childrens Hosp, Amsterdam, Netherlands. [Guarino, S.; La Manna, A.; Marzuillo, P.] Univ Campania Luigi Vanvitelli, Caserta, Italy. [Guffens, A.] CHC Clin Esperence, Montegnee, Belgium. [Hansen, P.] CHU Tivoli, La Louviere, Belgium. [Haumann, S.] Univ Klinikum Koln, Cologne, Germany. [He, G.] Foshan Women & Children Hosp, Foshan, Peoples R China. [Helmy, R.] Cairo Univ, Kasr Al Ainy Sch Med, Cairo, Egypt. [Hooman, N.; Otoukesh, H.] Iran Univ Med Sci, Aliasghar Clin Res Dev Unit, Tehran, Iran. [Janssens, P.] Univ Hosp Brussels, Brussels, Belgium. [Karamaria, S.; Prytula, A.; Raes, A.; Snauwaert, E.; Vande Walle, J.] UZ Gent, Ghent, Belgium. [Koenig, J.] Univ Hosp Muenster, Munster, Germany. [Litwin, M.; Obrycki, L.] Childrens Mem Hlth Inst, Warsaw, Poland. [Lombet, J.] CHR Citadelle, Liege, Belgium. [Longo, G.; Murer, L.] Hosp Univ Padova, Pediat Nephrol Dialysis & Transplant Unit, Padua, Italy. [Mallawaarachchi, A.] Garvan Inst, Darlinghurst, NSW, Australia. [Mallawaarachchi, A.] Royal Prince Alfred Hosp, Camperdown, NSW, Australia. [Mallawaarachchi, A.; McCarthy, H.; Quinlan, C.] KidGen, Sydney, NSW, Australia. [McCarthy, H.] Childrens Hosp Westmead, Westmead, NSW, Australia. [McCarthy, H.] Sydney Childrens Hosp, Sydney, NSW, Australia. [Papizh, S.; Prikhodina, L.] Pirogov Russian Nat Res Med Uni, Res & Clin Inst Pediat, Moscow, Russia. [Parvex, P.; Wilhelm-Bals, A.] Childrens Univ Hosp Geneva, Geneva, Switzerland. [Pawlak-Bratkowska, M.; Szczepanik, E.; Tkaczyk, M.] Polish Mothers Mem Hosp, Res Inst, Lodz, Poland. [Quinlan, C.] RCH Melbourne, Melbourne, Vic, Australia. [Ranchin, B.] Hop Femme Mere Enfant, Bron, France. [Ronit, C.] Ctr Hosp Luxembourg, Clin Pediat, Luxembourg, Luxembourg. [Schaefer, S.; Wuehl, E.] Ctr Pediat & Adolescent, Div Pediat Nephrol, Heidelberg, Germany. [Schreuder, M.] Radboudumc Amalia Childrens Hosp, Nijmegen, Netherlands. [Schurmans, T.; Tram, N.] CHU Charleroi, Charleroi, Belgium. [Seeman, T.] Charles Univ Prague, Prague, Czech Republic. [Seeman, T.] Motol Univ Hosp, Prague, Czech Republic. [Sinha, M.] Evelina London Childrens Hosp, London, England. [Taranta-Janusz, K.] Dept Pediat & Nephrol, Bialystok, Poland. [Thumfart, J.] Berlin Charite Univ Med, Berlin, Germany. [Utsch, B.] Herford Hosp, Dept Paediat, Herford, Germany. [Yildirim, Z. Y.] Istanbul Univ, Fac Med, Pediat Nephrol Dept, Istanbul, Turkey.
- Published
- 2019
50. ADPedKD: A Global Online Platform on the Management of Children With ADPKD
- Author
-
De Rechter, Stephanie, Bockenhauer, Detlef, Guay-Woodford, Lisa M., Liu, Isaac, Mallett, Andrew J., Soliman, Neveen A., Sylvestre, Lucimary C., Schaefer, Franz, Liebau, Max C., Mekahli, Djalila, Adamczyk, P., Akinci, N., Alpay, H., Ardelean, C., Ayasreh, N., Aydin, Z., Bael, A., Baudouin, V, Bayrakci, U. S., Bensman, A., Bialkevich, H., Biebuyck, A., Boyer, O., Bjanid, O., Brylka, A., Caliskan, S., Cambier, A., Camelio, A., Carbone, V, Charbit, M., Chiodini, B., Chirita, A., Cicek, N., Cerkauskiene, R., Collard, L., Conceicao, M., Constantinescu, I, Couderc, A., Crapella, B., Cvetkovic, M., Dima, B., Diomeda, F., Docx, M., Dolan, N., Dossier, C., Drozdz, D., Drube, J., Dunand, O., Dusan, P., Eid, L. A., Emma, F., Espino Hernandez, M., Fila, M., Furlano, M., Gafencu, M., Ghuysen, Ms, Giani, M., Giordano, M., Girisgen, I, Godefroid, N., Godron-Dubrasquet, A., Gojkovic, I, Gonzalez, E., Gokce, I, Groothoff, J. W., Guarino, S., Guffens, A., Hansen, P., Harambat, J., Haumann, S., He, G., Heidet, L., Helmy, R., Hemery, F., Hooman, N., Ilanas, B., Jankauskiene, A., Janssens, P., Karamaria, S., Kazyra, I, Koenig, J., Krid, S., Krug, P., Kwon, V, La Manna, A., Leroy, V, Litwin, M., Lombet, J., Longo, G., Lungu, A. C., Mallawaarachchi, A., Marin, A., Marzuillo, P., Massella, L., Mastrangelo, A., McCarthy, H., Miklaszewska, M., Moczulska, A., Montini, G., Morawiec-Knysak, A., Morin, D., Murer, L., Negru, I, Nobili, F., Obrycki, L., Otoukesh, H., Ozcan, S., Pape, L., Papizh, S., Parvex, P., Pawlak-Bratkowska, M., Prikhodina, L., Prytula, A., Quinlan, C., Raes, A., Ranchin, B., Ranguelov, N., Repeckiene, R., Ronit, C., Salomon, R., Santagelo, R., Saygili, S. K., Schaefer, S., Schreuder, M., Schurmans, T., Seeman, T., Segers, N., Sinha, M., Snauwaert, E., Spasojevic, B., Stabouli, S., Stoica, C., Stroescu, R., Szczepanik, E., Szczepanska, M., Taranta-Janusz, K., Teixeira, A., Thumfart, J., Tkaczyk, M., Torra, R., Torres, D., Tram, N., Utsch, B., Vande Walle, J., Vieux, R., Vitkevic, R., Wilhelm-Bals, A., Wuehl, E., Yildirim, Z. Y., Yuksel, S., Zachwieja, K., De Rechter, Stephanie, Bockenhauer, Detlef, Guay-Woodford, Lisa M., Liu, Isaac, Mallett, Andrew J., Soliman, Neveen A., Sylvestre, Lucimary C., Schaefer, Franz, Liebau, Max C., Mekahli, Djalila, Adamczyk, P., Akinci, N., Alpay, H., Ardelean, C., Ayasreh, N., Aydin, Z., Bael, A., Baudouin, V, Bayrakci, U. S., Bensman, A., Bialkevich, H., Biebuyck, A., Boyer, O., Bjanid, O., Brylka, A., Caliskan, S., Cambier, A., Camelio, A., Carbone, V, Charbit, M., Chiodini, B., Chirita, A., Cicek, N., Cerkauskiene, R., Collard, L., Conceicao, M., Constantinescu, I, Couderc, A., Crapella, B., Cvetkovic, M., Dima, B., Diomeda, F., Docx, M., Dolan, N., Dossier, C., Drozdz, D., Drube, J., Dunand, O., Dusan, P., Eid, L. A., Emma, F., Espino Hernandez, M., Fila, M., Furlano, M., Gafencu, M., Ghuysen, Ms, Giani, M., Giordano, M., Girisgen, I, Godefroid, N., Godron-Dubrasquet, A., Gojkovic, I, Gonzalez, E., Gokce, I, Groothoff, J. W., Guarino, S., Guffens, A., Hansen, P., Harambat, J., Haumann, S., He, G., Heidet, L., Helmy, R., Hemery, F., Hooman, N., Ilanas, B., Jankauskiene, A., Janssens, P., Karamaria, S., Kazyra, I, Koenig, J., Krid, S., Krug, P., Kwon, V, La Manna, A., Leroy, V, Litwin, M., Lombet, J., Longo, G., Lungu, A. C., Mallawaarachchi, A., Marin, A., Marzuillo, P., Massella, L., Mastrangelo, A., McCarthy, H., Miklaszewska, M., Moczulska, A., Montini, G., Morawiec-Knysak, A., Morin, D., Murer, L., Negru, I, Nobili, F., Obrycki, L., Otoukesh, H., Ozcan, S., Pape, L., Papizh, S., Parvex, P., Pawlak-Bratkowska, M., Prikhodina, L., Prytula, A., Quinlan, C., Raes, A., Ranchin, B., Ranguelov, N., Repeckiene, R., Ronit, C., Salomon, R., Santagelo, R., Saygili, S. K., Schaefer, S., Schreuder, M., Schurmans, T., Seeman, T., Segers, N., Sinha, M., Snauwaert, E., Spasojevic, B., Stabouli, S., Stoica, C., Stroescu, R., Szczepanik, E., Szczepanska, M., Taranta-Janusz, K., Teixeira, A., Thumfart, J., Tkaczyk, M., Torra, R., Torres, D., Tram, N., Utsch, B., Vande Walle, J., Vieux, R., Vitkevic, R., Wilhelm-Bals, A., Wuehl, E., Yildirim, Z. Y., Yuksel, S., and Zachwieja, K.
- Abstract
Background: Autosomal dominant polycystic kidney disease (ADPKD) is the most common monogenic cause of renal failure. For several decades, ADPKD was regarded as an adult-onset disease. In the past decade, it has become more widely appreciated that the disease course begins in childhood. However, evidence-based guidelines on how to manage and approach children diagnosed with or at risk of ADPKD are lacking. Also, scoring systems to stratify patients into risk categories have been established only for adults. Overall, there are insufficient data on the clinical course during childhood. We therefore initiated the global ADPedKD project to establish a large international pediatric ADPKD cohort for deep characterization. Methods: Global ADPedKD is an international multicenter observational study focusing on childhood-diagnosed ADPKD. This collaborative project is based on interoperable Web-based databases, comprising 7 regional and independent but uniformly organized chapters, namely Africa, Asia, Australia, Europe, North America, South America, and the United Kingdom. In the database, a detailed basic data questionnaire, including genetics, is used in combination with data entry from follow-up visits, to provide both retrospective and prospective longitudinal data on clinical, radiologic, and laboratory findings, as well as therapeutic interventions. Discussion: The global ADPedKD initiative aims to characterize in detail the most extensive international pediatric ADPKD cohort reported to date, providing evidence for the development of unified diagnostic, follow-up, and treatment recommendations regarding modifiable disease factors. Moreover, this registry will serve as a platform for the development of clinical and/or biochemical markers predicting the risk of early and progressive disease.
- Published
- 2019
Catalog
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