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22 results on '"Roperto, Rosa Maria"'

7. P1813CLINICAL CHARACTERIZATION OF CONGENITAL SOLITARY FUNCTIONING KIDNEY IN CHILDREN

Author

Becherucci, Francesca, primary, Guzzi, Francesca, primary, Cirillo, Luigi, primary, Bettiol, Alessandra, primary, Zuccato, Arianna, primary, Mazzierli, Tommaso, primary, Buti, Elisa, primary, Errichiello, Carmela, primary, Vaglio, Augusto, primary, Masieri, Lorenzo, primary, Olianti, Catia, primary, Materassi, Marco, primary, Roperto, Rosa Maria, primary, and Romagnani, Paola, primary

Published
2020
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8. Acute kidney injury promotes development of papillary renal cell adenoma and carcinoma from renal progenitor cells

Author

Peired, Anna Julie, primary, Antonelli, Giulia, additional, Angelotti, Maria Lucia, additional, Allinovi, Marco, additional, Guzzi, Francesco, additional, Sisti, Alessandro, additional, Semeraro, Roberto, additional, Conte, Carolina, additional, Mazzinghi, Benedetta, additional, Nardi, Sara, additional, Melica, Maria Elena, additional, De Chiara, Letizia, additional, Lazzeri, Elena, additional, Lasagni, Laura, additional, Lottini, Tiziano, additional, Landini, Samuela, additional, Giglio, Sabrina, additional, Mari, Andrea, additional, Di Maida, Fabrizio, additional, Antonelli, Alessandro, additional, Porpiglia, Francesco, additional, Schiavina, Riccardo, additional, Ficarra, Vincenzo, additional, Facchiano, Davide, additional, Gacci, Mauro, additional, Serni, Sergio, additional, Carini, Marco, additional, Netto, George J., additional, Roperto, Rosa Maria, additional, Magi, Alberto, additional, Christiansen, Christian Fynbo, additional, Rotondi, Mario, additional, Liapis, Helen, additional, Anders, Hans-Joachim, additional, Minervini, Andrea, additional, Raspollini, Maria Rosaria, additional, and Romagnani, Paola, additional

Published
2020
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9. Reverse Phenotyping after Whole-Exome Sequencing in Steroid-Resistant Nephrotic Syndrome

Author

Landini, Samuela, primary, Mazzinghi, Benedetta, additional, Becherucci, Francesca, additional, Allinovi, Marco, additional, Provenzano, Aldesia, additional, Palazzo, Viviana, additional, Ravaglia, Fiammetta, additional, Artuso, Rosangela, additional, Bosi, Emanuele, additional, Stagi, Stefano, additional, Sansavini, Giulia, additional, Guzzi, Francesco, additional, Cirillo, Luigi, additional, Vaglio, Augusto, additional, Murer, Luisa, additional, Peruzzi, Licia, additional, Pasini, Andrea, additional, Materassi, Marco, additional, Roperto, Rosa Maria, additional, Anders, Hans-Joachim, additional, Rotondi, Mario, additional, Giglio, Sabrina Rita, additional, and Romagnani, Paola, additional

Published
2019
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10. Sevoflurane as bridge therapy for plasma exchange and Anakinra in febrile infection–related epilepsy syndrome.

Author

L'Erario, Manuela, Roperto, Rosa Maria, and Rosati, Anna

Abstract

Febrile infection–related epilepsy syndrome (FIRES) is a devastating immune inflammatory–mediated epileptic encephalopathy. Herein, we discuss a previously healthy 8‐year‐old boy with FIRES in whom high dosages of conventional and nonconventional anesthetics were ineffective in treating SE, as were ketogenic diet, intravenous corticosteroids, and immunoglobulins. After 29 days of prolonged SRSE, the patient was successfully treated with sevoflurane paired with plasma exchange, for a total of five days, thus obtaining a stable EEG suppression burst pattern with no adverse events. Anakinra at the dosage of 100 mg b.i.d. was started seven days after sevoflurane and plasma exchange had been discontinued and was effective in ensuring non‐recurrence of SE. Sevoflurane as bridge therapy for immunosuppressive treatment could be considered an early, safe, and effective option in treating convulsive SE in which an autoimmune‐inflammatory etiology can reasonably be hypothesized. [ABSTRACT FROM AUTHOR]

Published
2021
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14. MO072GENETIC AND CLINICAL CHARACTERIZATION OF A LARGE COHORT OF PATIENTS WITH DISTAL RENAL TUBULAR ACIDOSIS AND CLINICAL CHARACTERIZATION OF A LARGE COHORT OF PATIENTS WITH DISTAL RENAL TUBULAR ACIDOSIS

Author

Becherucci, Francesca, primary, Palazzo, Viviana, additional, Provenzano, Aldesia, additional, Sansavini, Giulia, additional, Mazzinghi, Benedetta, additional, Roperto, Rosa Maria, additional, Materassi, Marco, additional, Romagnani, Paola, additional, and Giglio, Sabrina, additional

Published
2017
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15. The genetic and clinical spectrum of a large cohort of patients with distal renal tubular acidosis

Author

Palazzo, Viviana, primary, Provenzano, Aldesia, additional, Becherucci, Francesca, additional, Sansavini, Giulia, additional, Mazzinghi, Benedetta, additional, Orlandini, Valerio, additional, Giunti, Laura, additional, Roperto, Rosa Maria, additional, Pantaleo, Marilena, additional, Artuso, Rosangela, additional, Andreucci, Elena, additional, Bargiacchi, Sara, additional, Traficante, Giovanna, additional, Stagi, Stefano, additional, Murer, Luisa, additional, Benetti, Elisa, additional, Emma, Francesco, additional, Giordano, Mario, additional, Rivieri, Francesca, additional, Colussi, Giacomo, additional, Penco, Silvana, additional, Manfredini, Emanuela, additional, Caruso, Maria Rosa, additional, Garavelli, Livia, additional, Andrulli, Simeone, additional, Vergine, Gianluca, additional, Miglietti, Nunzia, additional, Mancini, Elena, additional, Malaventura, Cristina, additional, Percesepe, Antonio, additional, Grosso, Enrico, additional, Materassi, Marco, additional, Romagnani, Paola, additional, and Giglio, Sabrina, additional

Published
2017
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16. Prevalence of SARS-CoV-2-IgG Antibodies in Children with CKD or Immunosuppression

Author

Morello, William, Mastrangelo, Antonio, Guzzo, Isabella, Cusinato, Lisa, Annicchiarico Petruzzelli, Luigi, Benevenuta, Chiara, Martelli, Laura, Dall’Amico, Roberto, Vianello, Federica Alessandra, Puccio, Giuseppe, Massella, Laura, Benetti, Elisa, Pecoraro, Carmine, Peruzzi, Licia, Montini, Giovanni, Peruzzi, Licia, Petruzzelli, Luigi Annicchiarico, Becherucci, Francesca, Benetti, Elisa, Benevenuta, Chiara, Brugnara, Milena, Casadio, Luca, Chimenz, Roberto, Conti, Giovanni, Corrado, Ciro, D’Agostino, Viviana, Dall’Amico, Roberto, Gianoglio, Bruno, Giordano, Mario, Gualeni, Chiara, Guarino, Stefano, Guzzo, Isabella, La Manna, Angela, La Scola, Claudio, Martelli, Laura, Massella, Laura, Mastrangelo, Antonio, Materassi, Marco, Montini, Giovanni, Morello, William, Pani, Antonello, Papalia, Teresa, Pasini, Andrea, Pecoraro, Carmine, Pelliccia, Piernicola, Pennesi, Marco, Pugliese, Fabrizio, Ratsch, Ilse Maria, Romagnani, Paola, Roperto, Rosa Maria, Tamburello, Chiara, Vergine, Gianluca, Vergori, Antonio, Vianello, Federica Alessandra, and Vidal, Enrico

Published
2021
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18. A propensity-matched comparison of hard outcomes in children on chronic dialysis.

Author

Vidal, Enrico, Chesnaye, Nicholas C., Paglialonga, Fabio, Minale, Bruno, Leozappa, Giovanna, Giordano, Mario, Gianoglio, Bruno, Corrado, Ciro, Roperto, Rosa Maria, Chimenz, Roberto, Mencarelli, Francesca, Ratsch, Ilse-Maria, Murer, Luisa, Verrina, Enrico, on behalf of the Italian Registry for Paediatric Chronic Dialysis, and Italian Registry for Paediatric Chronic Dialysis

Subjects
HEMODIALYSIS, PERITONEAL dialysis, CHILD mortality, JUVENILE diseases, TREATMENT of chronic kidney failure, CHRONIC kidney failure, COMPARATIVE studies, KIDNEY transplantation, LONGITUDINAL method, RESEARCH methodology, MEDICAL cooperation, PAIRED comparisons (Mathematics), PROBABILITY theory, RESEARCH, LOGISTIC regression analysis, EVALUATION research, TREATMENT effectiveness, ACQUISITION of data, PROPORTIONAL hazards models, RETROSPECTIVE studies
Abstract

Data concerning outcomes of children on hemodialysis (HD) and peritoneal dialysis (PD) are scarce and frequently derived from single-center experiences. We sought to compare survival and transplantation rates in a large cohort of PD and HD patients. We extracted all patients initiating dialysis under 16 years of age between 2004 and 2013 from the Italian Registry of Pediatric Chronic Dialysis. Patients on PD were propensity-matched to those on HD based on gender, age, primary cause of ESRD, and the number of co-morbidities. Stratified Cox proportional hazard models were used to compare outcomes by dialysis modality. Three hundred ten patients were matched from 452 incident patients. In the unmatched cohort, PD patients were younger, more likely to be diagnosed with CAKUT, and had a higher urine output than HD patients. In the propensity-matched cohort, covariates were balanced between the two groups. At 2 years, the cumulative hazard ratio for death was similar (CHR 0.95, 95% CI 0.17-5.20) for HD relative to PD patients; and at 5 years, the CHR was lower for HD patients (0.22 95% CI 0.16-0.29). The cumulative incidence of transplantation at 3 years after dialysis initiation was 60.9% in HD patients and 59.7% in PD patients, with a CHR of 1.03 (95% CI 0.73-1.45).Conclusions: Pediatric PD and HD patients have distinct characteristics. After controlling for treatment-selection biases, children selected to start on PD or HD exhibit a similar mortality risk during the first 2 years on treatment, after which this risk increases in PD children. What is Known: • Few studies have compared hard outcomes in children on maintenance dialysis. • Children started on different dialysis modalities have distinct characteristics that impact on survival. What is New: • After controlling for treatment-selection biases, children selected to start dialysis on PD or HD exhibit a similar mortality risk during the first 2 years on treatment, after which this risk appears to be increased in PD children. • An "integrative care" approach should be used in children on PD, switching them to HD when PD-related morbidity tends to increase. [ABSTRACT FROM AUTHOR]

Published
2018
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19. Human Urine-Derived Renal Progenitors for Personalized Modeling of Genetic Kidney Disorders

Author

Lazzeri, Elena, primary, Ronconi, Elisa, additional, Angelotti, Maria Lucia, additional, Peired, Anna, additional, Mazzinghi, Benedetta, additional, Becherucci, Francesca, additional, Conti, Sara, additional, Sansavini, Giulia, additional, Sisti, Alessandro, additional, Ravaglia, Fiammetta, additional, Lombardi, Duccio, additional, Provenzano, Aldesia, additional, Manonelles, Anna, additional, Cruzado, Josep M., additional, Giglio, Sabrina, additional, Roperto, Rosa Maria, additional, Materassi, Marco, additional, Lasagni, Laura, additional, and Romagnani, Paola, additional

Published
2015
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20. SP868A PROPENSITY-MATCHED COMPARISON OF HARD OUTCOMES IN CHILDREN ON CHRONIC DIALYSIS: THE ITALIAN REGISTRY EXPERIENCE

Author

Vidal, Enrico, primary, Chimenz, Roberto, additional, Edefonti, Alberto, additional, Gianoglio, Bruno, additional, Leozappa, Giovanna, additional, Longo, Germana, additional, Maringhini, Silvio, additional, Mencarelli, Francesca, additional, Pecoraro, Carmine, additional, Puteo, Flora, additional, Ratsch, Ilse-Maria, additional, Roperto, Rosa Maria, additional, and Verrina, Enrico, additional

Published
2015
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21. Heterogeneous Genetic Alterations in Sporadic Nephrotic Syndrome Associate with Resistance to Immunosuppression

Author

Giglio, Sabrina, primary, Provenzano, Aldesia, additional, Mazzinghi, Benedetta, additional, Becherucci, Francesca, additional, Giunti, Laura, additional, Sansavini, Giulia, additional, Ravaglia, Fiammetta, additional, Roperto, Rosa Maria, additional, Farsetti, Silvia, additional, Benetti, Elisa, additional, Rotondi, Mario, additional, Murer, Luisa, additional, Lazzeri, Elena, additional, Lasagni, Laura, additional, Materassi, Marco, additional, and Romagnani, Paola, additional

Published
2015
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22. Reverse Phenotyping after Whole-Exome Sequencing in Steroid-Resistant Nephrotic Syndrome.

Author

Landini S, Mazzinghi B, Becherucci F, Allinovi M, Provenzano A, Palazzo V, Ravaglia F, Artuso R, Bosi E, Stagi S, Sansavini G, Guzzi F, Cirillo L, Vaglio A, Murer L, Peruzzi L, Pasini A, Materassi M, Roperto RM, Anders HJ, Rotondi M, Giglio SR, and Romagnani P

Subjects
Biopsy, Child, Child, Preschool, Female, Genetic Predisposition to Disease, Humans, Kidney Function Tests, Kidney Transplantation, Male, Nephrotic Syndrome diagnosis, Nephrotic Syndrome genetics, Nephrotic Syndrome surgery, Phenotype, Predictive Value of Tests, Prognosis, Reproducibility of Results, Retrospective Studies, Workflow, Genetic Variation, Nephrotic Syndrome congenital, Exome Sequencing
Abstract

Background and Objectives: Nephrotic syndrome is a typical presentation of genetic podocytopathies but occasionally other genetic nephropathies can present as clinically indistinguishable phenocopies. We hypothesized that extended genetic testing followed by reverse phenotyping would increase the diagnostic rate for these patients., Design, Setting, Participants, & Measurements: All patients diagnosed with nephrotic syndrome and referred to our center between 2000 and 2018 were assessed in this retrospective study. When indicated, whole-exome sequencing and in silico filtering of 298 genes related to CKD were combined with subsequent reverse phenotyping in patients and families. Pathogenic variants were defined according to current guidelines of the American College of Medical Genetics., Results: A total of 111 patients (64 steroid-resistant and 47 steroid-sensitive) were included in the study. Not a single pathogenic variant was detected in the steroid-sensitive group. Overall, 30% (19 out of 64) of steroid-resistant patients had pathogenic variants in podocytopathy genes, whereas a substantial number of variants were identified in other genes, not commonly associated with isolated nephrotic syndrome. Reverse phenotyping, on the basis of a personalized diagnostic workflow, permitted to identify previously unrecognized clinical signs of an unexpected underlying genetic nephropathy in a further 28% (18 out of 64) of patients. These patients showed similar multidrug resistance, but different long-term outcome, when compared with genetic podocytopathies., Conclusions: Reverse phenotyping increased the diagnostic accuracy in patients referred with the diagnosis of steroid-resistant nephrotic syndrome., (Copyright © 2020 by the American Society of Nephrology.)

Published
2020
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