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1. Reducing agalsidase beta infusion time in Fabry patients: low incidence of antibody formation and infusion-associated reactions in an Italian multicenter study

Author

Mignani, R, Americo, C, Aucella, F, Battaglia, Y, Cianci, V, Sapuppo, A, Lanzillo, C, Pennacchiotti, F, Tartaglia, L, Marchi, G, Pieruzzi, F, Mignani, Renzo, Americo, Claudio, Aucella, Filippo, Battaglia, Yuri, Cianci, Vittoria, Sapuppo, Annamaria, Lanzillo, Chiara, Pennacchiotti, Fabio, Tartaglia, Luciano, Marchi, Giacomo, Pieruzzi, Federico, Mignani, R, Americo, C, Aucella, F, Battaglia, Y, Cianci, V, Sapuppo, A, Lanzillo, C, Pennacchiotti, F, Tartaglia, L, Marchi, G, Pieruzzi, F, Mignani, Renzo, Americo, Claudio, Aucella, Filippo, Battaglia, Yuri, Cianci, Vittoria, Sapuppo, Annamaria, Lanzillo, Chiara, Pennacchiotti, Fabio, Tartaglia, Luciano, Marchi, Giacomo, and Pieruzzi, Federico

Abstract

Background Fabry disease is a rare progressive X-linked lysosomal storage disease caused by mutations in the GLA gene that encodes alpha-galactosidase A. Agalsidase beta is a recombinant enzyme replacement therapy authorized in Europe at a standard dose of 1.0 mg/kg intravenously every other week at an initial infusion rate of <= 0.25 mg/min until patient tolerance is established, after which the infusion rate may be increased gradually. However, specific practical guidance regarding the progressive reduction in infusion time is lacking. This study investigated a new and specific protocol for reducing agalsidase beta infusion time in which a stable dosage of 15 mg/h is infused for the first four months, and the infusion rate is increased progressively from 15 to 35 mg/h for the subsequent four infusions. The shortest infusion time is reached after six months and maintained thereafter. The incidence of infusion-associated reactions (IARs) and the development of anti-drug antibodies were analyzed, and the disease burden and the clinical evolution of the disease at 12 months were evaluated. Results Twenty-five of the 31 patients were naive to enzyme or chaperone treatment at baseline and six patients had been switched from agalsidase alfa. The reduced infusion time protocol was well tolerated. Only one patient exhibited an IAR, with mild symptoms that resolved with low-dose steroids. Six patients globally seroconverted during treatment (4 with a classic phenotype and 2 with late-onset disease). All but three patients were seronegative at month 12. All patients were stable at the study's end (FAbry STabilization indEX value < 20%); reducing infusion time did not negatively impact clinical outcomes in any patient. The perceived medical assessment showed that the quality of life of all patients improved. Conclusions The study demonstrates that reducing agalsidase beta infusion time is possible and safe from both an immunogenic and clinical point of view. The use of

Published
2024

2. Omics in chronic kidney disease: Focus on prognosis and prediction

Author

Provenzano, M, Serra, R, Garofalo, C, Michael, A, Crugliano, G, Battaglia, Y, Ielapi, N, Bracale, U, Faga, T, Capitoli, G, Galimberti, S, Andreucci, M, Provenzano M., Serra R., Garofalo C., Michael A., Crugliano G., Battaglia Y., Ielapi N., Bracale U. M., Faga T., Capitoli G., Galimberti S., Andreucci M., Provenzano, M, Serra, R, Garofalo, C, Michael, A, Crugliano, G, Battaglia, Y, Ielapi, N, Bracale, U, Faga, T, Capitoli, G, Galimberti, S, Andreucci, M, Provenzano M., Serra R., Garofalo C., Michael A., Crugliano G., Battaglia Y., Ielapi N., Bracale U. M., Faga T., Capitoli G., Galimberti S., and Andreucci M.

Abstract

Chronic kidney disease (CKD) patients are characterized by a high residual risk for cardiovascular (CV) events and CKD progression. This has prompted the implementation of new prognostic and predictive biomarkers with the aim of mitigating this risk. The ‘omics’ techniques, namely genomics, proteomics, metabolomics, and transcriptomics, are excellent candidates to provide a better understanding of pathophysiologic mechanisms of disease in CKD, to improve risk stratification of patients with respect to future cardiovascular events, and to identify CKD patients who are likely to respond to a treatment. Following such a strategy, a reliable risk of future events for a particular patient may be calculated and consequently the patient would also benefit from the best available treatment based on their risk profile. Moreover, a further step forward can be represented by the aggregation of multiple omics information by combining different techniques and/or different biological samples. This has already been shown to yield additional information by revealing with more accuracy the exact individual pathway of disease.

Published
2022

3. Recovery of dialysis patients with COVID-19: health outcomes 3 months after diagnosis in ERACODA

Author

Hemmelder, M. H., Noordzij, M., Vart, P., Hilbrands, L. B., Jager, K. J., Abrahams, A. C., Arroyo, D., Battaglia, Y., Ekart, R., Mallamaci, F., Malloney, S. -R., Oliveira, J., Rydzewski, A., Sridharan, S., Vogt, L., Duivenvoorden, R., Gansevoort, R. T., Franssen, C. F. M., van der Net, J. B., Essig, M., du Buf-Vereijken, P. W. G., van Ginneken, B., Maas, N., van Jaarsveld, B. C., Bemelman, F. J., Klingenberg-Salahova, F., Heenan-Vos, F., Vervloet, M. G., Nurmohamed, A., Abramowicz, D., Verhofstede, S., Maoujoud, O., Malfait, T., Fialova, J., Melilli, E., Fava, A., Cruzado, J. M., Perez, N. M., Lips, J., Krepel, H., Adilovic, H., Hengst, M., Konings, C. J. A. M., Braconnier, P., Weis, D., Gellert, R., Alferes, D. G., Radulescu, D., Zakharova, E. V., Ambuehl, P. M., Guidotti, R., Walker, A., Lepeytre, F., Rabate, C., Rostoker, G., Marques, S., Azasevac, T., Majstorovic, G. S., Katicic, D., Dam, M. T., Kruger, T., Brzosko, S., Liakopoulos, V., Zanen, A. L., Logtenberg, S. J. J., Fricke, L., Kuryata, O., Slebe, J. J. P., Abd ElHafeez, S., Kemlin, D., van de Wetering, J., Reinders, M. E. J., Hesselink, D. A., van Gestel, J. K., Eiselt, J., Kielberger, L., El-Wakil, H. S., Verhoeven, M. A. M., Logan, I., Canal, C., Facundo, C., Ramos, A. M., Debska-Slizien, A., Veldhuizen, N. M. H., Tigka, E., Polyzou Konsta, M. A., Panagoutsos, S., Postorino, A., Cambareri, F., Matceac, I., Nistor, I., Covic, A., Groeneveld, J. H. M., Jousma, J., Diekmann, F., Oppenheimer, F., Blasco, M., Pereira, T. A., dos Santos Junior, A. C. S., Arias-Cabrales, C., Crespo, M., Llinas-Mallol, L., Buxeda, A., Tarrega, C. B., Redondo-Pachon, D., Arenas Jimenez, M. D., Mendoza-Valderrey, A., Martins, A. C., Mateus, C., Alvila, G., Laranjinha, I., Hofstra, J. M., Siezenga, M. A., Franco, A., Castellano, S., Rodriguez-Ferrero, M. L., Manzanos, S. B., Haridian Sosa Barrios, R., Lemahieu, W., Bartelet, K., Dirim, A. B., Demir, E., Sever, M. S., Turkmen, A., Safak, S., Hollander, D. A. M. J., Kerckhoffs, A., Buttner, S., de Vries, A. P. J., Meziyerh, S., van der Helm, D., Mallat, M., Bouwsma, H., Petruliene, K., Verberk, I., van der Sande, F. M., Christiaans, M. H. L., Mohankumar, N., Luca, M. D., Tuglular, S. Z., Kramer, A., Beerenhout, C., Luik, P. T., Kerschbaum, J., Tiefenthaler, M., Watschinger, B., Adema, A. Y., Stepanov, V. A., Zulkarnaev, A. B., Turkmen, K., Gandolfini, I., Maggiore, U., Fliedner, A., Asberg, A., Mjoen, G., Miyasato, H., de Fijter, C. W. H., Mongera, N., Pini, S., de Biase, C., van de Logt, A. E., Maas, R., Lebedeva, O., Lopez, V., Reichert, L. J. M., Verhave, J., Titov, D., Parshina, E. V., Zanoli, L., Marcantoni, C., van Kempen, G., van Gils-Verrij, L. E. A., Harty, J. C., Meurs, M., Myslak, M., Lentini, P., den Deurwaarder, E., Stendahl, M., Rahimzadeh, H., Schouten, M., Rychlik, I., Cabezas-Reina, C. J., Roca, A. M., Nauta, F., Sahin, I., Goffin, E., Kanaan, N., Labriola, L., Devresse, A., Diaz-Mareque, A., Coca, A., de Arriba, G., Meijers, B. K. I., Naesens, M., Kuypers, D., Desschans, B., Tonnerlier, A., Wissing, K. M., Dedinska, I., Pessolano, G., Malik, S., Dounousi, E., Papachristou, E., Berger, S. P., Meijer, E., Sanders, J. S. F., Ozyilmaz, A., Ponikvar, J. B., Pernat, A. M., Kovac, D., Arnol, M., Molenaar, F. M., van Zuilen, A. D., Meijvis, S. C. A., Dolmans, H., Tantisattamo, E., Esposito, P., Krzesinski, J. -M., Barahira, J. D., Gallieni, M., Martin-Moreno, P. L., Guglielmetti, G., Guzzo, G., Toapanta, N., Soler, M. J., Luik, A. J., van Kuijk, W. H. M., Stikkelbroeck, L. W. H., Hermans, M. M. H., Rimsevicius, L., Righetti, M., Islam, M., Heitink-Ter Braak, N., Nephrology, ACS - Microcirculation, ACS - Diabetes & metabolism, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service de néphrologie, Clinical sciences, Faculteit Medische Wetenschappen/UMCG, Groningen Kidney Center (GKC), Cardiovascular Centre (CVC), Groningen Institute for Organ Transplantation (GIOT), Interne Geneeskunde, RS: Carim - V02 Hypertension and target organ damage, Medical Informatics, APH - Aging & Later Life, APH - Quality of Care, APH - Global Health, ACS - Pulmonary hypertension & thrombosis, APH - Health Behaviors & Chronic Diseases, and Internal Medicine

Subjects
Male, Outcome Assessment, survival, mental health status, COVID-19 Testing, SDG 3 - Good Health and Well-being, Renal Dialysis, functional health status, Outcome Assessment, Health Care, 80 and over, Humans, KIDNEY-TRANSPLANT, AcademicSubjects/MED00340, Aged, Aged, 80 and over, Transplantation, SARS-CoV-2, MORTALITY, COVID-19, Middle Aged, Health Care, Intensive Care Units, Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], Nephrology, dialysis, Original Article, Female, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11]
Abstract

Background Coronavirus disease 2019 (COVID-19)-related short-term mortality is high in dialysis patients, but longer-term outcomes are largely unknown. We therefore assessed patient recovery in a large cohort of dialysis patients 3 months after their COVID-19 diagnosis. Methods We analyzed data on dialysis patients diagnosed with COVID-19 from 1 February 2020 to 31 March 2021 from the European Renal Association COVID-19 Database (ERACODA). The outcomes studied were patient survival, residence and functional and mental health status (estimated by their treating physician) 3 months after COVID-19 diagnosis. Complete follow-up data were available for 854 surviving patients. Patient characteristics associated with recovery were analyzed using logistic regression. Results In 2449 hemodialysis patients (mean ± SD age 67.5 ± 14.4 years, 62% male), survival probabilities at 3 months after COVID-19 diagnosis were 90% for nonhospitalized patients (n = 1087), 73% for patients admitted to the hospital but not to an intensive care unit (ICU) (n = 1165) and 40% for those admitted to an ICU (n = 197). Patient survival hardly decreased between 28 days and 3 months after COVID-19 diagnosis. At 3 months, 87% functioned at their pre-existent functional and 94% at their pre-existent mental level. Only few of the surviving patients were still admitted to the hospital (0.8–6.3%) or a nursing home (∼5%). A higher age and frailty score at presentation and ICU admission were associated with worse functional outcome. Conclusions Mortality between 28 days and 3 months after COVID-19 diagnosis was low and the majority of patients who survived COVID-19 recovered to their pre-existent functional and mental health level at 3 months after diagnosis.

Published
2022
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4. Recovery of dialysis patients with COVID-19: health outcomes 3 months after diagnosis in ERACODA

Author

Hemmelder, M.H., Noordzij, M., Vart, P., Hilbrands, L.B., Jager, K.J., Abrahams, A.C., Arroyo, D., Battaglia, Y., Ekart, R., Mallamaci, F., Malloney, S.R., Oliveira, J., Rydzewski, A., Sridharan, S., Vogt, L., Duivenvoorden, R., Gansevoort, R.T., Franssen, C.F., Hemmelder, M.H., Noordzij, M., Vart, P., Hilbrands, L.B., Jager, K.J., Abrahams, A.C., Arroyo, D., Battaglia, Y., Ekart, R., Mallamaci, F., Malloney, S.R., Oliveira, J., Rydzewski, A., Sridharan, S., Vogt, L., Duivenvoorden, R., Gansevoort, R.T., and Franssen, C.F.

Abstract

Item does not contain fulltext, BACKGROUND: Coronavirus disease 2019 (COVID-19)-related short-term mortality is high in dialysis patients, but longer-term outcomes are largely unknown. We therefore assessed patient recovery in a large cohort of dialysis patients 3 months after their COVID-19 diagnosis. METHODS: We analyzed data on dialysis patients diagnosed with COVID-19 from 1 February 2020 to 31 March 2021 from the European Renal Association COVID-19 Database (ERACODA). The outcomes studied were patient survival, residence and functional and mental health status (estimated by their treating physician) 3 months after COVID-19 diagnosis. Complete follow-up data were available for 854 surviving patients. Patient characteristics associated with recovery were analyzed using logistic regression. RESULTS: In 2449 hemodialysis patients (mean ± SD age 67.5 ± 14.4 years, 62% male), survival probabilities at 3 months after COVID-19 diagnosis were 90% for nonhospitalized patients (n = 1087), 73% for patients admitted to the hospital but not to an intensive care unit (ICU) (n = 1165) and 40% for those admitted to an ICU (n = 197). Patient survival hardly decreased between 28 days and 3 months after COVID-19 diagnosis. At 3 months, 87% functioned at their pre-existent functional and 94% at their pre-existent mental level. Only few of the surviving patients were still admitted to the hospital (0.8-6.3%) or a nursing home (∼5%). A higher age and frailty score at presentation and ICU admission were associated with worse functional outcome. CONCLUSIONS: Mortality between 28 days and 3 months after COVID-19 diagnosis was low and the majority of patients who survived COVID-19 recovered to their pre-existent functional and mental health level at 3 months after diagnosis.

Published
2022

5. Trabecular complexity as an early marker of cardiac involvement in Fabry disease

Author

Camporeale, A, Moroni, F, Lazzeroni, D, Garibaldi, S, Pieroni, M, Pieruzzi, F, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Econimo, L, Battaglia, Y, Graziani, F, Pica, S, Chow, K, Camici, P, Lombardi, M, Camporeale, Antonia, Moroni, Francesco, Lazzeroni, Davide, Garibaldi, Silvia, Pieroni, Maurizio, Pieruzzi, Federico, Lusardi, Paola, Spada, Marco, Mignani, Renzo, Burlina, Alessandro, Carubbi, Francesca, Econimo, Laura, Battaglia, Yuri, Graziani, Francesca, Pica, Silvia, Chow, Kelvin, Camici, Paolo G, Lombardi, Massimo, Camporeale, A, Moroni, F, Lazzeroni, D, Garibaldi, S, Pieroni, M, Pieruzzi, F, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Econimo, L, Battaglia, Y, Graziani, F, Pica, S, Chow, K, Camici, P, Lombardi, M, Camporeale, Antonia, Moroni, Francesco, Lazzeroni, Davide, Garibaldi, Silvia, Pieroni, Maurizio, Pieruzzi, Federico, Lusardi, Paola, Spada, Marco, Mignani, Renzo, Burlina, Alessandro, Carubbi, Francesca, Econimo, Laura, Battaglia, Yuri, Graziani, Francesca, Pica, Silvia, Chow, Kelvin, Camici, Paolo G, and Lombardi, Massimo

Abstract

AIMS: Fabry cardiomyopathy is characterized by glycosphingolipid storage and increased myocardial trabeculation has also been demonstrated. This study aimed to explore by cardiac magnetic resonance whether myocardial trabecular complexity, quantified by endocardial border fractal analysis, tracks phenotype evolution in Fabry cardiomyopathy. METHODS AND RESULTS: Study population included 20 healthy controls (12 males, age 32±9) and 45 Fabry patients divided into three groups: 15 left ventricular hypertrophy (LVH)-negative patients with normal T1 (5 males, age 28±13; Group 1); 15 LVH-negative patients with low T1 (9 males, age 33±9.6; Group 2); 15 LVH-positive patients (11 males, age 53.5±9.6; Group 3). Trabecular fractal dimensions (Dfs) (total, basal, mid-ventricular, and apical) were evaluated on cine images. Total Df was higher in all Fabry groups compared to controls, gradually increasing from controls to Group 3 (1.27±0.02 controls vs. 1.29±0.02 Group 1 vs. 1.30±0.02 Group 2 vs. 1.34±0.02 Group 3; P<0.001). Group 3 showed significantly higher values of all Dfs compared to the other Groups. Both basal and total Dfs were significantly higher in Group 1 compared with controls (basal: 1.30±0.03 vs. 1.26±0.04, P =0.010; total: 1.29±0.02 vs. 1.27±0.02, P=0.044). Total Df showed significant correlations with: (i) T1 value (r=-0.569; P<0.001); (ii) LV mass (r=0.664, P<0.001); (iii) trabecular mass (r=0.676; P <0.001); (iv) Mainz Severity Score Index (r=0.638; P<0.001). CONCLUSION: Fabry cardiomyopathy is characterized by a progressive increase in Df of endocardial trabeculae together with shortening of T1 values. Myocardial trabeculation is increased before the presence of detectable sphingolipid storage, thus representing an early sign of cardiac involvement. Published on behalf of the European Society of Cardiology.

Published
2022

7. A randomized multicenter trial on a lung ultrasound-guided treatment strategy in patients on chronic hemodialysis with high cardiovascular risk see commentary

Author

Zoccali, C., Torino, C., Mallamaci, F., Sarafidis, P., Papagianni, A., Ekart, R., Hojs, R., Klinger, M., Letachowicz, K., Fliser, D., Seiler-Mussler, S., Lizzi, F., Wiecek, A., Miskiewicz, A., Siamopoulos, K., Balafa, O., Slotki, I., Shavit, L., Stavroulopoulos, A., Covic, A., Siriopol, D., Massy, Z.A., Seidowsky, A., Battaglia, Y., Martinez-Castelao, A., Polo-Torcal, C., Coudert-Krier, M.J., Rossignol, P., Fiaccadori, E., Regolisti, G., Hannedouche, T., Bachelet, T., Jager, K.J., Dekker, F.W., Tripepi, R., Tripepi, G., Gargani, L., Sicari, R., Picano, E., and London, G.M.

Subjects
cardiovascular risk, lung congestion, heart failure hemodialysis, chronic kidney failure, ESRD, lung ultrasound
Abstract

Lung congestion is a risk factor for all-cause and cardiovascular mortality in patients on chronic hemodialysis, and its estimation by ultrasound may be useful to guide ultrafiltration and drug therapy in this population. In an international, multi-center randomized controlled trial (NCT02310061) we investigated whether a lung ultrasound-guided treatment strategy improved a composite end point (all-cause death, non-fatal myocardial infarction, decompensated heart failure) vs usual care in patients receiving chronic hemodialysis with high cardiovascular risk. Patient-Reported Outcomes (Depression and the Standard Form 36 Quality of Life Questionnaire, SF36) were assessed as secondary outcomes. A total of 367 patients were enrolled: 183 in the active arm and 180 in the control arm. In the active arm, the pre-dialysis lung scan was used to titrate ultrafiltration during dialysis and drug treatment. Three hundred and seven patients completed the study: 152 in the active arm and 155 in the control arm. During a mean follow-up of 1.49 years, lung congestion was significantly more frequently relieved in the active (78%) than in the control (56%) arm and the intervention was safe. The primary composite end point did not significantly differ between the two study arms (Hazard Ratio 0.88; 95% Confidence Interval: 0.63-1.24). The risk for all-cause and cardiovascular hospitalization and the changes of left ventricular mass and function did not differ among the two groups. A post hoc analysis for recurrent episodes of decompensated heart failure (0.37; 0.15-0.93) and cardiovascular events (0.63; 0.41-0.97) showed a risk reduction for these outcomes in the active arm. There were no differences in patientreported outcomes between groups. Thus, in patients on chronic hemodialysis with high cardiovascular risk, a treatment strategy guided by lung ultrasound effectively relieved lung congestion but was not more effective than usual care in improving the primary or secondary end points of the trial.

Published
2021
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8. Clinical triage of patients on kidney replacement therapy presenting with COVID-19: An ERACODA registry analysis

Author

Mitra, S., Jayanti, A., Vart, P., Coca, A., Gallieni, M., Ovrehus, M. A., Midtvedt, K., Abd Elhafeez, S., Gandolfini, I., Buttner, S., Franssen, C. F. M., Hemmelder, M. H., Van Der Net, J. B., Essig, M., Du Buf-Vereijken, P. W. G., Van Ginneken, B., Maas, N., Vogt, L., Van Jaarsveld, B. C., Jager, K. J., Bemelman, F. J., Klingenberg-Salahova, F., Heenan-Vos, F., Vervloet, M. G., Nurmohamed, A., Abramowicz, D., Verhofstede, S., Maoujoud, O., Malfait, T., Fialova, J., Melilli, E., Fava, A., Cruzado, J. M., Perez, N. M., Lips, J., Krepel, H., Adilovic, H., Hengst, M., Rydzewski, A., Gellert, R., Oliveira, J., Alferes, D. G., Zakharova, E. V., Ambuehl, P. M., Walker, A., Winzeler, R., Lepeytre, F., Rabate, C., Rostoker, G., Marques, S., Azasevac, T., Katicic, D., Dam, M. T., Kruger, T., Brzosko, S., Zanen, A. L., Logtenberg, S. J. J., Fricke, L., Slebe, J. J. P., Kemlin, D., Van De Wetering, J., Reinders, M. E. J., Eiselt, J., Kielberger, L., El-Wakil, H. S., Verhoeven, M. A. M., Canal, C., Facundo, C., Ramos, A. M., Debska-Slizien, A., Veldhuizen, N. M. H., Tigka, E., Konsta, M. A. P., Panagoutsos, S., Mallamaci, F., Postorino, A., Cambareri, F., Covic, A., Matceac, I., Nistor, I., Cordos, M., Groeneveld, J. H. M., Jousma, J., Marjolijn Van Buren, Diekmann, F., Tiago Assis Pereira, Santos, A. C. S., Arias-Cabrales, C., Crespo, M., Llinas-Mallol, L., Buxeda, A., Tarrega, C. B., Redondo-Pachon, D., Jimenez, M. D. A., Hofstra, J. M., Franco, A., Arroyo, D., Rodriguez-Ferrero, M. L., Manzanos, S. B., Barrios, R. H. S., Avila, G., Laranjinha, I., Mateus, C., Lemahieu, W., Bartelet, K., Dirim, A. B., Sever, M. S., Demir, E., Safak, S., Turkmen, A., Hollander, D. A. M. J., De Vries, A. P. J., Meziyerh, S., Van Der Helm, D., Mallat, M., Bouwsma, H., Sridharan, S., Petruliene, K., Maloney, S. -R., Verberk, I., Van Der Sande, F. M., Christiaans, M. H. L., Mohankumar, N., Di Luca, M., Tuglular, S. Z., Kramer, A., Beerenhout, C., Luik, P. T., Kerschbaum, J., Tiefenthaler, M., Watschinger, B., Adema, A. Y., Stepanov, V. A., Zulkarnaev, A. B., Turkmen, K., Fliedner, A., Asberg, A., Mjoen, G., Miyasato, H., De Fijter, C. W. H., Mongera, N., Pini, S., De Biase, C., Duivenvoorden, R., Hilbrands, L., Kerckhoffs, A., Van De Logt, A. -E., Maas, R., Lebedeva, O., Lopez, V., Verhave, J., Reichert, L. J. M., Titov, D., Parshina, E. V., Zanoli, L., Marcantoni, C., Van Gils-Verrij, L. E. A., Harty, J. C., Meurs, M., Myslak, M., Battaglia, Y., Lentini, P., Den Deurwaarder, E., Stendahl, M., Rahimzadeh, H., Schouten, M., Rychlik, I., Cabezas-Reina, C. J., Roca, A. M., Nauta, F., Goffin, E., Kanaan, N., Labriola, L., Devresse, A., Diaz-Mareque, A., Meijers, B. K. I., Naesens, M., Kuypers, D., Desschans, B., Tonnelier, A., Wissing, K. M., De Arriba, G., Dedinska, I., Pessolano, G., Maggiore, U., Malik, S., Papachristou, E., Gansevoort, R. T., Noordzij, M., Berger, S. P., Meijer, E., Ozyilmaz, A., Sanders, J. S. F., Ponikvar, J. B., Arnol, M., Pernat, A. M., Kovac, D., Ekart, R., Abrahams, A. C., Molenaar, F. M., Van Zuilen, A. D., Meijvis, S. C. A., Dolmans, H., Tantisattamo, E., Esposito, P., Krzesinski, J. -M., Barahira, J. D., Sabiu, G., Martin-Moreno, P. L., Guglielmetti, G., Guzzo, G., Toapanta, N., Soler, M. J., Luik, A. J., Van Kuijk, W. H. M., Stikkelbroeck, L. W. H., Hermans, M. M. H., Rimsevicius, L., Righetti, M., Islam, M., Braak, N. H. -T., Nephrology, UCL - SSS/IREC/NEFR - Pôle de Néphrologie, UCL - (SLuc) Service de néphrologie, Interne Geneeskunde, RS: Carim - V02 Hypertension and target organ damage, Groningen Kidney Center (GKC), ACS - Diabetes & metabolism, AII - Inflammatory diseases, AII - Infectious diseases, Internal Medicine, and Clinical sciences

Subjects
kidney, medicine.medical_specialty, Coronavirus disease 2019 (COVID-19), medicine.medical_treatment, Pulmonary insufficiency, infectious diseases, Kidney, Second presentation, Interquartile range, Internal medicine, medicine, Humans, Registries, Mortality, AcademicSubjects/MED00340, Dialysis, Aged, Transplantation, second presentation, business.industry, SARS-CoV-2, COVID-19, medicine.disease, mortality, Triage, Hospitalization, Renal Replacement Therapy, Renal disorders Radboud Institute for Molecular Life Sciences [Radboudumc 11], Nephrology, Oxygen Saturation, dialysis, Original Article, Hemodialysis, Renal disorders Radboud Institute for Health Sciences [Radboudumc 11], Presentation (obstetrics), business, transplantation
Abstract

Background Patients on kidney replacement therapy (KRT) are at very high risk of coronavirus disease 2019 (COVID-19). The triage pathway for KRT patients presenting to hospitals with varying severity of COVID-19 illness remains ill-defined. We studied the clinical characteristics of patients at initial and subsequent hospital presentations and the impact on patient outcomes. Methods The European Renal Association COVID-19 Database (ERACODA) was analysed for clinical and laboratory features of 1423 KRT patients with COVID-19 either hospitalized or non-hospitalized at initial triage and those re-presenting a second time. Predictors of outcomes (hospitalization, 28-day mortality) were then determined for all those not hospitalized at initial triage. Results Among 1423 KRT patients with COVID-19 [haemodialysis (HD), n = 1017; transplant, n = 406), 25% (n = 355) were not hospitalized at first presentation due to mild illness (30% HD, 13% transplant). Of the non-hospitalized patients, only 10% (n = 36) re-presented a second time, with a 5-day median interval between the two presentations (interquartile range 2–7 days). Patients who re-presented had worsening respiratory symptoms, a decrease in oxygen saturation (97% versus 90%) and an increase in C-reactive protein (26 versus 73 mg/L) and were older (72 vs 63 years) compared with those who did not return a second time. The 28-day mortality between early admission (at first presentation) and deferred admission (at second presentation) was not significantly different (29% versus 25%; P = 0.6). Older age, prior smoking history, higher clinical frailty score and self-reported shortness of breath at first presentation were identified as risk predictors of mortality when re-presenting after discharge at initial triage. Conclusions This study provides evidence that KRT patients with COVID-19 and mild illness can be managed effectively with supported outpatient care and with vigilance of respiratory symptoms, especially in those with risk factors for poor outcomes. Our findings support a risk-stratified clinical approach to admissions and discharges of KRT patients presenting with COVID-19 to aid clinical triage and optimize resource utilization during the ongoing pandemic.

Published
2021
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9. The GALA project: Practical recommendations for the use of migalastat in clinical practice on the basis of a structured survey among Italian experts

Author

Chimenti, C, Nencini, P, Pieruzzi, F, Feriozzi, S, Mignani, R, Pieroni, M, Pisani, A, Battaglia, Y, Bersano, A, Fiumara, A, Lanzillo, C, Piga, S, Re, F, Rubino, M, Zedde, M, Chimenti C., Nencini P., Pieruzzi F., Feriozzi S., Mignani R., Pieroni M., Pisani A., Battaglia Y., Bersano A., Fiumara A., Lanzillo C., Piga S., Re F., Rubino M., Zedde M. L., Chimenti, C, Nencini, P, Pieruzzi, F, Feriozzi, S, Mignani, R, Pieroni, M, Pisani, A, Battaglia, Y, Bersano, A, Fiumara, A, Lanzillo, C, Piga, S, Re, F, Rubino, M, Zedde, M, Chimenti C., Nencini P., Pieruzzi F., Feriozzi S., Mignani R., Pieroni M., Pisani A., Battaglia Y., Bersano A., Fiumara A., Lanzillo C., Piga S., Re F., Rubino M., and Zedde M. L.

Abstract

Background: Oral migalastat has recently been approved for the treatment of Anderson-Fabry disease (FD) in patients aged ≥16 years with amenable mutations on the basis of two phase III trials, FACETS and ATTRACT. However, with the introduction of migalastat into clinical practice, it is important to correctly identify the patients who may gain the most benefits from this therapy. Due to the relatively recent availability of migalastat, its role in clinical practice still has to be included in guidelines or recommendations. On these bases, a multidisciplinary group of Italian Experts in the treatment of FD has run the GALA project, with the aim to collect the opinions of expert physicians and to propose some starting points for an experience-based use of migalastat. Results: Overall, although studies and data from longer-term follow-up with migalastat are still emerging, available evidence is consistent in showing that this molecule does represent a suitable therapy for the treatment of FD, in patients aged ≥16 years and with amenable mutations. The use of migalastat as an oral option appears to be overall safe, and experience thus far indicates potential for improving quality of life, controlling GI symptoms, stabilizing renal function and reducing cardiac hypertrophy. Conclusion: Migalastat can be considered either as a first-line therapy - given its efficacy, extensive tissue penetration, convenient oral regimen, and the current limited therapeutic options available - or in patients on enzyme-replacement therapy (ERT) who experience side effects, with poor compliance to chronic i.v. therapy, or with clinical evidence of progression of the disease.

Published
2020

10. Atrial Dysfunction Assessed by Cardiac Magnetic Resonance as an Early Marker of Fabry Cardiomyopathy

Author

Bernardini, A, Camporeale, A, Pieroni, M, Pieruzzi, F, Figliozzi, S, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Battaglia, Y, Graziani, F, Pica, S, Tondi, L, Chow, K, Boveri, S, Olivotto, I, Lombardi, M, Bernardini A., Camporeale A., Pieroni M., Pieruzzi F., Figliozzi S., Lusardi P., Spada M., Mignani R., Burlina A., Carubbi F., Battaglia Y., Graziani F., Pica S., Tondi L., Chow K., Boveri S., Olivotto I., Lombardi M., Bernardini, A, Camporeale, A, Pieroni, M, Pieruzzi, F, Figliozzi, S, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Battaglia, Y, Graziani, F, Pica, S, Tondi, L, Chow, K, Boveri, S, Olivotto, I, Lombardi, M, Bernardini A., Camporeale A., Pieroni M., Pieruzzi F., Figliozzi S., Lusardi P., Spada M., Mignani R., Burlina A., Carubbi F., Battaglia Y., Graziani F., Pica S., Tondi L., Chow K., Boveri S., Olivotto I., and Lombardi M.

Published
2020

11. [Home hemodialysis: multicenter observational study]

Author

PAOLO LENTINI, Gemelli A, Battaglia Y, Ambrogio A, Esposito R, Zanoli L, Previti A, Dell'Aquila R, and Fiorini F

Subjects
home hemodialysis, Hemodialysis, Home, Phosphorus, Kidney Failure, home dialysis, Parathyroid Hormone, Renal Dialysis, Hemodialysis, Humans, Kidney Failure, Chronic, Calcium, Chronic, Home, Peritoneal Dialysis, chronic kidney disease
Abstract

Home dialysis is a primary objective of Italian Ministry of Health. As stated in the National Chronicity Plan and the Address Document for Chronic Renal Disease, it is mostly home hemodialysis and peritoneal dialysis to be carried out in the patient's home. Home hemodialysis has already been used in the past and today has found new technologies and new applications. The patient's autonomy and the need for a caregiver during the sessions are still the main limiting factors. In this multicenter observational study, 7 patients were enrolled for 24 months. They underwent six weekly hemodialysis sessions of 180' each; periodic medical examinations and blood tests were performed (3, 6, 12, 18 and 24 months). After 3-6 months of home hemodialysis there was already an improvement in the control of calcium-phosphorus metabolism (improvement in phosphorus values, (p0.01), a reduction in parathyroid hormone (p0.01)); in the number of phosphorus binders used (p0.02); in blood pressure control (with a reduction in the number of hypotensive drugs p0.02). Home hemodialysis, although applicable to a small percentage of patients (10-15%), has improved blood pressure control, calcium-phosphorus metabolism and anemia, reducing the need for rhEPO.

Published
2021

12. The GALA project: Practical recommendations for the use of migalastat in clinical practice on the basis of a structured survey among Italian experts

Author

Chimenti C., Nencini P., Pieruzzi F., Feriozzi S., Mignani R., Pieroni M., Pisani A., Battaglia Y., Bersano A., Fiumara A., Lanzillo C., Piga S., Re F., Rubino M., Zedde M. L., Chimenti, C, Nencini, P, Pieruzzi, F, Feriozzi, S, Mignani, R, Pieroni, M, Pisani, A, Battaglia, Y, Bersano, A, Fiumara, A, Lanzillo, C, Piga, S, Re, F, Rubino, M, and Zedde, M

Subjects
medicine.medical_specialty, 1-Deoxynojirimycin, lcsh:Medicine, Disease, Tissue penetration, Quality of life, Migalastat, medicine, Humans, Pharmacology (medical), In patient, Position Statement, Intensive care medicine, Genetics (clinical), Fabry disease, business.industry, lcsh:R, General Medicine, Clinical Practice, Treatment, Regimen, Italy, enzyme-replacement therapy, expert opinion, fabry disease, migalastat, treatment, Expert opinion, alpha-Galactosidase, Cardiac hypertrophy, Quality of Life, business, Enzyme-replacement therapy
Abstract

Background Oral migalastat has recently been approved for the treatment of Anderson-Fabry disease (FD) in patients aged ≥16 years with amenable mutations on the basis of two phase III trials, FACETS and ATTRACT. However, with the introduction of migalastat into clinical practice, it is important to correctly identify the patients who may gain the most benefits from this therapy. Due to the relatively recent availability of migalastat, its role in clinical practice still has to be included in guidelines or recommendations. On these bases, a multidisciplinary group of Italian Experts in the treatment of FD has run the GALA project, with the aim to collect the opinions of expert physicians and to propose some starting points for an experience-based use of migalastat. Results Overall, although studies and data from longer-term follow-up with migalastat are still emerging, available evidence is consistent in showing that this molecule does represent a suitable therapy for the treatment of FD, in patients aged ≥16 years and with amenable mutations. The use of migalastat as an oral option appears to be overall safe, and experience thus far indicates potential for improving quality of life, controlling GI symptoms, stabilizing renal function and reducing cardiac hypertrophy. Conclusion Migalastat can be considered either as a first-line therapy – given its efficacy, extensive tissue penetration, convenient oral regimen, and the current limited therapeutic options available – or in patients on enzyme-replacement therapy (ERT) who experience side effects, with poor compliance to chronic i.v. therapy, or with clinical evidence of progression of the disease.

Published
2020

13. The Role of Prognostic and Predictive Biomarkers for Assessing Cardiovascular Risk in Chronic Kidney Disease Patients

Author

Provenzano M, Andreucci M, De Nicola L, Garofalo C, Battaglia Y, Borrelli S, Gagliardi I, Faga T, Michael A, Mastroroberto P, Serraino GF, Licastro N, Ielapi N, Serra R., Provenzano, M, Andreucci, M, De Nicola, L, Garofalo, C, Battaglia, Y, Borrelli, S, Gagliardi, I, Faga, T, Michael, A, Mastroroberto, P, Serraino, Gf, Licastro, N, Ielapi N, and Serra, R.

Published
2020

19. Progressive electrocardiographic changes in parallel with cardiac magnetic resonance findings in fabry disease

Author

Figliozzi, S, primary, Camporeale, A, additional, Pieroni, M, additional, Pieruzzi, F, additional, Namdar, M, additional, Lusardi, P, additional, Spada, M, additional, Mignani, R, additional, Burlina, A, additional, Scolari, F, additional, Carrubbi, F, additional, Battaglia, Y, additional, Graziani, F, additional, Boveri, S, additional, and Lombardi, M, additional

Published
2020
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20. Redefining the pulvinar sign in fabry disease

Author

Cocozza, S, Russo, C, Pisani, A, Olivo, G, Riccio, E, Cervo, A, Pontillo, G, Feriozzi, S, Veroux, M, Battaglia, Y, Concolino, D, Pieruzzi, F, Mignani, R, Borrelli, P, Imbriaco, M, Brunetti, A, Tedeschi, E, Palma, G, Cocozza, S., Russo, C., Pisani, A., Olivo, G., Riccio, E., Cervo, A., Pontillo, G., Feriozzi, S., Veroux, M., Battaglia, Y., Concolino, D., Pieruzzi, F., Mignani, R., Borrelli, P., Imbriaco, M., Brunetti, A., Tedeschi, E., Palma, G., Cocozza, S, Russo, C, Pisani, A, Olivo, G, Riccio, E, Cervo, A, Pontillo, G, Feriozzi, S, Veroux, M, Battaglia, Y, Concolino, D, Pieruzzi, F, Mignani, R, Borrelli, P, Imbriaco, M, Brunetti, A, Tedeschi, E, Palma, G, Cocozza, S., Russo, C., Pisani, A., Olivo, G., Riccio, E., Cervo, A., Pontillo, G., Feriozzi, S., Veroux, M., Battaglia, Y., Concolino, D., Pieruzzi, F., Mignani, R., Borrelli, P., Imbriaco, M., Brunetti, A., Tedeschi, E., and Palma, G.

Abstract

BACKGROUND AND PURPOSE: The pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity. MATERIALS AND METHODS: We retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls. RESULTS: The pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables. CONCLUSIONS: The pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease

Published
2017

21. New insights from the application of the FAbry STabilization indEX in a large population of Fabry cases

Author

Mignani, R, Pieroni, M, Pisani, A, Spada, M, Battaglia, Y, Verrecchia, E, Mangeri, M, Feriozzi, S, Tanini, I, De Danieli, G, Pieruzzi, F, Mignani, R, Pieroni, M, Pisani, A, Spada, M, Battaglia, Y, Verrecchia, E, Mangeri, M, Feriozzi, S, Tanini, I, De Danieli, G, and Pieruzzi, F

Abstract

Background: The FAbry STabilization indEX (FASTEX) is an innovative index allowing the assessment of clinical stability over time in Fabry disease patients. This index was developed in a population of 28 male patients with the classical form of Fabry disease. Objectives: The aim of the study was to test the accuracy of the FASTEX in evaluating Fabry disease stability in 132 male and female patients with classical and non-classical Fabry disease from nine Italian centres and it also aimed to define the sensitivity and specificity of this new tool. In particular, we aimed to investigate the correlation between the FASTEX and clinical judgement in a large-scale cohort of the study population. Methods: Statistical methods applied to this investigation included the calculation of accuracy, specificity and sensitivity, receiver operating characteristic (ROC) curves and Cohen’s j index related to the FASTEX and clinical judgement. Results: The patient population included 58 males (43.9%). The mean age of the overall population was 46.3 6 15. 1 years (range 31.2–61.4). The median interval between the two multidisciplinary evaluations used for FASTEX calculation was 398 days. Since no gold standard method is available to define the overall clinical condition of Fabry patients over time, the results of the FASTEX were compared with clinical judgements given by the physicians involved in this study. In this way, the FASTEX classified 121 of 132 (92%) patients correctly. In particular, the FASTEX correctly identified 93% (41/44) of clinically ‘unstable’ and 91% (80/88) of clinically ‘stable’ patients. The area under the curve of the ROC related to the FASTEX index cut-off (20) was equal to 0.967, very close to its theoretical maximum (1), which means that it is an excellent test for classifying patients as ‘stable’ or ‘unstable’ compared with clinical judgement. In addition, the FASTEX cut-off >20 provides themost acceptable balance between sensitivity and specificity. The C

Published
2019

22. Awareness of kidney diseases in general population and in high school students. Italian report for World Kidney Days 2010-2011

Author

Apperti, V, Auricchio, Mr, Barbato, A, Bedani, Pl, Bellinghieri, G, Costantino, G, Boggi, R, Bonomini, M, Calabria, L, Calabria, M, Grimaldi, M, Capuano, M, Terribile, M, Caputo, C, Castellino, S, Centrone, E, Ciccarelli, M, Cicchetti, T, Costantino, E, D'Amaro, E, Dagostino, F, Napolitano, F, Bonifati, C, Dal Canton, A, Esposito, P, Delgado, G, D'Apice, L, Di Luca, M, Farfaglia, P, Cantù, P, Farina, M, Fasianos, E, Feriozzi, S, Galeotti, P, Fiorini, F, Frattolillo, P, Garibotto, Giacomo, Giannattasio, M, Detomaso, F, Latino, A, Li Vecchi, M, Maffucci, G, Anelli, Am, Mangano, S, Meneghel, G, Morrone, L, Mura, C, Novizio, D, Paglia, S, Cardone, F, Parsi, R, Pizzini, M, Polito, P, Prati, E, Brognoli, M, Rubino, F, Turchetta, L, Parravano, M, Sambati, M, Sicignano, M, Tarchini, R, Teatini, U, Gallieni, M, Colussi, G, Limido, A, Pozzi, C, Spotti, D, Brancaccio, D, Traversari, L, Venditti, G, Aucella, F, Garganico, R, Giovanni Rotondo, S, Cenerelli, S, Bozzi, M, Petrarulo, F, Casu, Md, Cavatorta, F, Montesano, C, Ferrara, Dd, Mazzola, Ma, De Simone, W, Del Rosso, G, Sozzo, Ee, Mangione, D, Emiliani, G, Fasianos, R, Ganadu, M, Garibotto, G, Lusenti, T, Manno, C, Orbello, G, Rondanini, V, Russo, D, Battaglia, Y, Sarli, P, Sozzo, E, Napoli, M, Valentini, Wd, Viganò, L., Y., Battaglia 1, L., Russo 1, R., Spadola 1, Russo, Domenico, Battaglia, Y, Russo, L., and Spadola, R.

Subjects
Male, Nephrology, Health Knowledge, Attitudes, Practice, Pediatrics, Kidney Disease, Global Health, Surveys and Questionnaires, Prevalence, Global health, Surveys and Questionnaire, Young adult, Reagent Strips, Practice, Kidney, education.field_of_study, Health Knowledge, Urinalysi, Awareness, Middle Aged, Proteinuria, Treatment Outcome, medicine.anatomical_structure, Italy, Hypertension, Kidney Diseases, Female, Reagent Strip, Student, Comprehension, Human, Adult, medicine.medical_specialty, Adolescent, Population, Health knowledge, Health literacy, Health Promotion, Urinalysis, Young Adult, Terminology as Topic, Internal medicine, medicine, Humans, Students, education, Aged, business.industry, Blood Pressure Determination, Health Literacy, Adolescent Behavior, Awarene, Health promotion, Attitudes, Family medicine, business
Published
2012
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23. Parapelvic cysts, a distinguishing feature of renal Fabry disease

Author

Pisani, A, Petruzzelli Annicchiarico, L, Pellegrino, A, Bruzzese, D, Feriozzi, S, Imbriaco, M, Tedeschi, E, Cocozza, S, De Rosa, D, Mignani, R, Veroux, M, Battaglia, Y, Concolino, D, Sestito, S, Pieruzzi, F, Caroti, L, Manna, R, Zizzo, C, Santangelo, M, Sabbatini, M, Riccio, E, PIERUZZI, FEDERICO UMBERTO EMILIO GUGLIE, Riccio, E., Pisani, A, Petruzzelli Annicchiarico, L, Pellegrino, A, Bruzzese, D, Feriozzi, S, Imbriaco, M, Tedeschi, E, Cocozza, S, De Rosa, D, Mignani, R, Veroux, M, Battaglia, Y, Concolino, D, Sestito, S, Pieruzzi, F, Caroti, L, Manna, R, Zizzo, C, Santangelo, M, Sabbatini, M, Riccio, E, PIERUZZI, FEDERICO UMBERTO EMILIO GUGLIE, and Riccio, E.

Published
2018

24. Supplementary Material for: What Is the Key to Improving Renal Transplant Recipients' Awareness of Skin Cancer Risk?

Author

Borghi, A., Corazza, M., Battaglia, Y., Maietti, E., Minghetti, S., and Virgili, A.

Abstract

Background: Previous studies have shown poor compliance rates regarding sun protection among organ transplant recipients. Objective: The main objective of the present study was to assess the awareness among renal transplant recipients (RTRs) of their risk of non-melanoma skin cancer (NMSC) development and their sunscreen use. The influence of several potentially relevant variables was also assessed in order to identify possible weak points on which to concentrate efforts in this respect. Methods: A total of 132 RTRs (92 males and 40 females) were included. The following information was collected and elaborated: (a) demographics; (b) skin phototype; (c) educational level; (d) time elapsed since transplantation; (e) immunosuppressive treatments; (f) previous dermatological visits; (g) patients' awareness of their NMSC risk; (h) use of sunscreen; and (i) previous documented NMSCs or NMSCs found during the study visit. Results: Overall, 65 patients (49.2%) expressed awareness of their susceptibility to skin cancers. A high educational level was the main factor associated with patients' awareness. Thirty-six RTRs (27.3%) reported using sunscreen regularly. High educational level and awareness of personal susceptibility to NMSC development were the most relevant factors associated with sun protection habits. Conclusion: The present study showed the low level of sunscreen use among RTRs and their scanty awareness of personal skin cancer risk. Since educational level has been found to be highly related to both awareness of cancer risk and adequate use of sunscreen among RTRs, it is necessary to improve the way education is delivered by dermatologists and nephrologists, especially to subjects with a low educational level.

Published
2017
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25. Redefining the Pulvinar Sign in Fabry Disease

Author

Cocozza, S., primary, Russo, C., additional, Pisani, A., additional, Olivo, G., additional, Riccio, E., additional, Cervo, A., additional, Pontillo, G., additional, Feriozzi, S., additional, Veroux, M., additional, Battaglia, Y., additional, Concolino, D., additional, Pieruzzi, F., additional, Mignani, R., additional, Borrelli, P., additional, Imbriaco, M., additional, Brunetti, A., additional, Tedeschi, E., additional, and Palma, G., additional

Published
2017
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26. Redefining the Pulvinar Sign in Fabry Disease

Author

Cocozza, S., Russo, C., Pisani, A., Olivo, Gaia, Riccio, E., Cervo, A., Pontillo, G., Feriozzi, S., Veroux, M., Battaglia, Y., Concolino, D., Pieruzzi, F., Mignani, R., Borrelli, P., Imbriaco, M., Brunetti, A., Tedeschi, E., Palma, G., Cocozza, S., Russo, C., Pisani, A., Olivo, Gaia, Riccio, E., Cervo, A., Pontillo, G., Feriozzi, S., Veroux, M., Battaglia, Y., Concolino, D., Pieruzzi, F., Mignani, R., Borrelli, P., Imbriaco, M., Brunetti, A., Tedeschi, E., and Palma, G.

Abstract

BACKGROUND AND PURPOSE: The pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity. MATERIALS AND METHODS: We retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls. RESULTS: The pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables. CONCLUSIONS: The pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease.

Published
2017
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27. Exercise in patients on dialysis: A multicenter, randomized clinical trial

Author

Manfredini, F, Mallamaci, F, D'Arrigo, G, Baggetta, R, Bolignano, D, Torino, C, Lamberti, N, Bertoli, S, Ciurlino, D, Rocca-rey, L, Barillà , A, Battaglia, Y, Rapanà, R, Zuccalà, A, Bonanno, G, Fatuzzo, P, Rapisarda, F, Rastelli, S, Fabrizi, F, Messa, P, De Paola, L, Lombardi, L, Cupisti, A, Fuiano, G, Lucisano, G, Summaria, C, Felisatti, M, Pozzato, E, Malagoni, A, Castellino, P, Aucella, F, Elhafeez, S, Provenzano, P, Tripepi, G, Catizone, L, Zoccali, C, D'arrigo, G, Rapanà, Rm, Malagoni, Am, Elhafeez, Sa, Provenzano, Pf, Zoccali, C., Manfredini, F, Mallamaci, F, D'Arrigo, G, Baggetta, R, Bolignano, D, Torino, C, Lamberti, N, Bertoli, S, Ciurlino, D, Rocca-rey, L, Barillà , A, Battaglia, Y, Rapanà, R, Zuccalà, A, Bonanno, G, Fatuzzo, P, Rapisarda, F, Rastelli, S, Fabrizi, F, Messa, P, De Paola, L, Lombardi, L, Cupisti, A, Fuiano, G, Lucisano, G, Summaria, C, Felisatti, M, Pozzato, E, Malagoni, A, Castellino, P, Aucella, F, Elhafeez, S, Provenzano, P, Tripepi, G, Catizone, L, Zoccali, C, D'arrigo, G, Rapanà, Rm, Malagoni, Am, Elhafeez, Sa, Provenzano, Pf, and Zoccali, C.

Abstract

Previous studies have suggested the benefits of physical exercise for patients on dialysis. We conducted the Exercise Introduction to Enhance Performance in Dialysis trial, a 6-month randomized, multicenter trial to test whether a simple, personalized walking exercise program at home, managed by dialysis staff, improves functional status in adult patients on dialysis. The main study outcomes included change in physical performance at 6 months, assessed by the 6-minute walking test and the five times sit-to-stand test, and in quality of life, assessed by the Kidney Disease Quality of Life Short Form (KDQOL-SF) questionnaire. We randomized 296 patients to normal physical activity (control; n=145) or walking exercise (n=151); 227 patients (exercise n=104; control n=123) repeated the 6-month evaluations. The distance covered during the 6-minute walking test improved in the exercise group (mean distance +/- SD: baseline, 328 +/- 96 m; 6 months, 367 +/- 113 m) but not in the control group (baseline, 321 +/- 107 m; 6 months, 324 +/- 116 m; P<0.001 between groups). Similarly, the five times sit-to-stand test time improved in the exercise group (mean time SD: baseline, 20.5 +/- 6.0 seconds; 6 months, 18.2 +/- 5.7 seconds) but not in the control group (baseline, 20.9 +/- 5.8 seconds; 6 months, 20.2 +/- 6.4 seconds; P=0.001 between groups). The cognitive function score (P=0.04) and quality of social interaction score (P=0.01) in the kidney disease component of the KDQOL-SF improved significantly in the exercise arm compared with the control arm. Hence, a simple, personalized, home-based, low-intensity exercise program managed by dialysis staff may improve physical performance and quality of life in patients on dialysis.

Published
2017

28. Corpus callosum involvement: a useful clue for differentiating Fabry Disease from Multiple Sclerosis

Author

Cocozza, S, Olivo, G, Riccio, E, Russo, C, Pontillo, G, Ugga, L, Migliaccio, S, de Rosa, D, Feriozzi, S, Veroux, M, Battaglia, Y, Concolino, D, Pieruzzi, F, Tuttolomondo, A, Caronia, A, Lanzillo, R, Brescia Morra, V, Imbriaco, M, Brunetti, A, Tedeschi, E, Pisani, A, PIERUZZI, FEDERICO UMBERTO EMILIO GUGLIE, Pisani, A., Cocozza, S, Olivo, G, Riccio, E, Russo, C, Pontillo, G, Ugga, L, Migliaccio, S, de Rosa, D, Feriozzi, S, Veroux, M, Battaglia, Y, Concolino, D, Pieruzzi, F, Tuttolomondo, A, Caronia, A, Lanzillo, R, Brescia Morra, V, Imbriaco, M, Brunetti, A, Tedeschi, E, Pisani, A, PIERUZZI, FEDERICO UMBERTO EMILIO GUGLIE, and Pisani, A.

Abstract

Purpose: Multiple sclerosis (MS) has been proposed as a possible differential diagnosis for Fabry disease (FD). The aim of this work was to evaluate the involvement of corpus callosum (CC) on MR images and its possible role as a radiological sign to differentiate between FD and MS. Methods: In this multicentric study, we retrospectively evaluated the presence of white matter lesions (WMLs) on the FLAIR images of 104 patients with FD and 117 patients with MS. The incidence of CC-WML was assessed in the two groups and also in a subgroup of 37 FD patients showing neurological symptoms. Results: WMLs were detected in 50 of 104 FD patients (48.1%) and in all MS patients. However, a lesion in the CC was detected in only 3 FD patients (2.9%) and in 106 MS patients (90.6%). In the FD subgroup with neurological symptoms, WMLs were present in 26 of 37 patients (70.3%), with two subjects (5.4%) showing a definite callosal lesion. Conclusion: FD patients have a very low incidence of CC involvement on conventional MR images compared to MS, independently from the clinical presentation and the overall degree of WM involvement. Evaluating the presence of CC lesions on brain MR scans can be used as a radiological sign for a differential diagnosis between MS and FD, rapidly addressing the physician toward a correct diagnosis and subsequent treatment options.

Published
2017

29. URGENZE IN NEFROLOGIA

Author

Granata, A., Battaglia, Y., DI NICOLÒ, P., Romano, M., Fatuzzo, Pasquale Mario, and Fiorini, F.

Published
2015

31. IDONEITÀ ALL’INSERIMENTO IN UN PROGRAMMA DI ESERCIZIO SEMPLICE E MORTALITÀ: UN’ANALISI COROLLARIA DELLO STUDIO EXCITE (EXERCISE INTRODUCTION TO ENHANCE PERFORMANCE IN DIALYSIS)

Author

Baggetta, R, Bolignano, D, Torino, C, Manfredini, F, Aucella, F, Barillà, A, Battaglia, Y, Bertoli, S, Bonanno, G, Castellino, Pietro, Rastelli, S, Ciurlino, D, Cupisti, A, D'Arrigo, G, De Paola, L, Fabrizi, F, Fatuzzo, Pasquale Mario, Fuiano, G, Lombardi, L, Gaetano, Lucisano, Messa, P, Rapanà, R, Rapisarda, Francesco, Rocca Rey, L, Summaria, C, Alessandro, Zuccalà, Tripepi, G, Catizone, L, Mallamaci, F, Zoccali, C, Ipertensione di Reggio Calabria, Gruppo di lavoro EXCITE CNR IFC/IBIM Epidemiologia Clinica e. Fisiopatologia delle Malattie Renali e., Italia, Biologia Molecolare, Dipartimento di Biochimica e., Divisione di Biochimica di allenamento fisico Centro Studi Biomedici applicati allo Sport Centro di Malattie Vascolari Dipartimento di Medicina Riabilitativa, Università degli Studi di Ferrara, Nefrologia, U. O., Ospedale Casa Sollievo della Sofferenza, San Giovanni Rotondo, Anna, AOU di Ferrara Arcispedale S., Ferrara, Dialisi, U. O. Nefrologia e., IRCCS Multimedica Policlinico Multispecialistico, Giovanni, Sesto S., Divisione Clinica di Nefrologia Chirurgica, Tecniche Dialitiche, Università degli Studi di Catania Azzurra Ambulatorio Medico Nefrologico e., Catania, Dipartimento di Medicina Interna, and Università di Catania

Published
2014

32. Risk factors for kidney diseases and awareness of blood pressure and proteinuria in general population and in high school students: Italian report for World Kidney Days 2012-2013

Author

Agate, *Square Project: V. M., Anelli, A. M., Apicella, L., Avino, D., Battaglia, Y., Bedani, P. L., Bellinghieri, Guido, Bernardi, A. M., Bonifati, C., Borzumati, M., Botti, P. L., Brigante, M., Brighina, F., Calzavara, P., Caputo, C., Cardone, F., Cavatorta, F., Confessore, N., Cossu, M., Costantino, E., Costantino, Giuseppe, D’Apice, L., D’Arcangelo, R., Dagostino, F., Dal Canton, A., Delgado, G., Di Pietro, R., Esposito, C., Esposito, P., Fasianos, E., Fiorini, F., Galeotti, P., Garibotto, G., Gemelli, A., Gregorini, M. C., Iuliano, P., La Peccerella, L., Liuzzi, M., Merola, M., Montesano, C., Morrone, L. F., Napoli, M., Napolitano, F., Paglia, S., Parravano, M., Pasquali, S., Rosa, A., Sambati, M. L., Schiavone, P., Sozzo, E., Storari, A., Tarchini, R., Tirino, G., Bellinghieri, L. T. u. r. c. h. e. t. t. a. School Project: G., Beltrame, G., Bozzi, M., Casolino, E., Centrone, E., Ciccarelli, M., Cicchetti, T., Colturi, C., Costantino, G., Garibotto, Indraccolo, F., Lombardi, M., Minoretti, C., Parsi, R., Petrarulo, F., Prati, E., Quarello, F., Rondanini, V., Russo, D., Tira, P., Turina, S., and Valentini, W. D.

Subjects
Adult, Male, Pathology, medicine.medical_specialty, Health Knowledge, Attitudes, Practice, Adolescent, Population, Health Promotion, Risk Factors, Medicine, Humans, Renal Insufficiency, Chronic, Renal Insufficiency, Chronic, education, Students, education.field_of_study, Practice, business.industry, Health Knowledge, Middle Aged, Proteinuria, Italy, Nephrology, Cardiovascular Diseases, Attitudes, Female, Kidney Diseases, business, Humanities
Abstract

*Square Project: V.M. Agate (Palmanova, UD), A.M. Anelli (Pistoia), L. Apicella (Salerno), D. Avino (Vairano Scalo, CE), Y. Battaglia (Ferrara), P.L. Bedani (Ferrara), G. Bellinghieri (Messina), A.M. Bernardi (Rovigo), C. Bonifati (Corato, BA), M. Borzumati (Verbania), P.L. Botti (Mantova), M. Brigante (Campobasso), F. Brighina (San Nicola La Strada, CE), P. Calzavara (Conegliano, TV), C. Caputo (Albenga, SV), F. Cardone (Lavello, PZ), F. Cavatorta (Imperia), N. Confessore (Scafati, SA), M. Cossu (Sassari), E. Costantino (Gavardo, BS), G. Costantino (Messina), L. D’Apice (Caserta), R. D’Arcangelo (Arzano, NA), F. Dagostino (Corato, BA), A. Dal Canton (Pavia), G. Delgado (Teano, CE), R. Di Pietro (Napoli), C. Esposito (Pavia), P. Esposito (Pavia), E. Fasianos (Altamura, BA), F. Fiorini (Rovigo), P. Galeotti (Viterbo), G. Garibotto (Genova), A. Gemelli (Rovigo), M.C. Gregorini (Reggio Emilia), P. Iuliano (Benevento), L. La Peccerella (Benevento), M. Liuzzi (Benevento), M. Merola (Sorrento, NA), C. Montesano (Imperia), L.F. Morrone (Benevento), M. Napoli (Galatina, LE), F. Napolitano (Corato, BA), S. Paglia (Lavello, PZ), M. Parravano (Sora, FR), S. Pasquali (Reggio Emilia), A. Rosa (Benevento), M.L. Sambati (Taranto), P. Schiavone (Brindisi), E. Sozzo (Galatina, LE), A. Storari (Ferrara), R. Tarchini (Mantova), G. Tirino (Montesarchio, BN), L. Turchetta (Sora, FR). School Project: G. Bellinghieri (Messina), G. Beltrame (Torino), M. Bozzi (Bari), E. Casolino (Rionero in Vulture, PZ), E. Centrone (Ruvo di Puglia, BA), M. Ciccarelli (Reggio Calabria), T. Cicchetti (Rossano, CS), C. Colturi (Sondrio), E. Costantino (Gavardo, BS), G. Costantino (Messina), F. Dagostino (Corato, BA), E. Fasianos (Altamura, BA), P. Galeotti (Viterbo), Garibotto (Genova), F. Indraccolo (S. Fermo della Battaglia, CO), M. Lombardi (Borgo S. Lorenzo, FI), C. Minoretti (S. Fermo della Battaglia, CO), F. Napolitano (Corato, BA), R. Parsi (Alcamo, TP), F. Petrarulo (Bari), E. Prati (Desenzano del Garda, BS), F. Quarello (Torino), V. Rondanini (Palmi, RC), D. Russo (Barletta), P. Tira (Manerbio, BS), S. Turina (Manerbio, BS), W.D. Valentini (Rieti) WORLD KIDNEY DAY

Published
2013

33. IL SIX-MINUTE WALKING TEST PREDICE IL RISCHIO DI MORTE, EVENTI CARDIOVASCOLARI ED OSPEDALIZZAZIONI NEI PAZIENTI IN DIALISI: un’analisi secondaria del trial EXCITE

Author

Baggetta, R., Manfredini, F., Bolignano, D., Rastelli, S., Bertoli, S., Messa, P., Zuccalà, A., Fatuzzo, P., Lombardi, L., Cupisti, A., Fuiano, G., Torino, C., Barillà, A., Ciurlino, D., Fabrizi, F., Rapanà, R., Bonanno, G., De Luciano, P., Lucisano, G., Catizone, L., Aucella, F., Battaglia, Y., Summaria, C., Rocca Rey, L., D'Arrigo, G., Tripepi, G., Zoccali, C., Mallamaci, F., EXCITE Working Group, and Rapisarda, Francesco

Published
2013

36. [Management of color-Doppler imaging in dialysis patients]

Author

Battaglia Y, Granata Antonio, Zamboli P, Lusenti T, Di Lullo L, Floccari F, Logias F, D'Amelio A, Fiorini F, Battaglia, Y, Granata, A, Zamboli, Pasquale, Lusenti, T, Di Lullo, L, Floccari, F, Logias, F, D'Amelio, A, and Fiorini, F.

37. [Chest ultrasound in nephrology]

Author

Fiorini F, Alessandro Zanforlin, D'Amelio A, Battaglia Y, Zamboli P, Granata A, Fiorini, F, Zanforlin, A, D'Amelio, A, Battaglia, Y, Zamboli, Pasquale, and Granata, A.

38. Exercise in patients on dialysis: A multicenter, randomized clinical trial (vol 28, pg 1259, 2017)

Author

Manfredini, F., Mallamaci, F., D Arrigo, G., Baggetta, R., Bolignano, D., Torino, C., Lamberti, N., Bertoli, S., Ciurlino, D., Rocca-Rey, L., Barilla, A., Battaglia, Y., Rapana, R. M., Zuccala, A., Bonanno, G., Fatuzzo, P., Rapisarda, F., Rastelli, S., Fabrizi, F., Messa, P., Paola, L., Lombardi, L., ADAMASCO CUPISTI, Fuiano, G., Lucisano, G., Summaria, C., Felisatti, M., Pozzato, E., Malagoni, A. M., Castellino, P., Aucella, F., Abd Elhafeez, S., Provenzano, P. F., Tripepi, G., Catizone, L., and Zoccali, C.

41. OMICS in Chronic Kidney Disease: Focus on Prognosis and Prediction

Author

Michele Provenzano, Raffaele Serra, Carlo Garofalo, Ashour Michael, Giuseppina Crugliano, Yuri Battaglia, Nicola Ielapi, Umberto Marcello Bracale, Teresa Faga, Giulia Capitoli, Stefania Galimberti, Michele Andreucci, Provenzano, M, Serra, R, Garofalo, C, Michael, A, Crugliano, G, Battaglia, Y, Ielapi, N, Bracale, U, Faga, T, Capitoli, G, Galimberti, S, Andreucci, M, Provenzano, M., Serra, R., Garofalo, C., Michael, A., Crugliano, G., Battaglia, Y., Ielapi, N., Bracale, U. M., Faga, T., Capitoli, G., Galimberti, S., Andreucci, M., Provenzano, Michele, Serra, Raffaele, Garofalo, Carlo, Michael, Ashour, Crugliano, Giuseppina, Battaglia, Yuri, Ielapi, Nicola, Bracale, Umberto Marcello, Faga, Teresa, Capitoli, Giulia, Galimberti, Stefania, and Andreucci, Michele

Subjects
QH301-705.5, precision medicine, SNP, Metabolomic, Review, Models, Biological, Catalysis, albuminuria, Inorganic Chemistry, proteomics, Models, chronic renal failure, Animals, Humans, Renal Insufficiency, Physical and Theoretical Chemistry, Chronic, Biology (General), Renal Insufficiency, Chronic, Molecular Biology, QD1-999, Spectroscopy, genomics, metabolomics, Organic Chemistry, Proteomic, General Medicine, Genomics, Biological, Prognosis, Computer Science Applications, Chemistry, Genomic
Abstract

Chronic kidney disease (CKD) patients are characterized by a high residual risk for cardiovascular (CV) events and CKD progression. This has prompted the implementation of new prognostic and predictive biomarkers with the aim of mitigating this risk. The ‘omics’ techniques, namely genomics, proteomics, metabolomics, and transcriptomics, are excellent candidates to provide a better understanding of pathophysiologic mechanisms of disease in CKD, to improve risk stratification of patients with respect to future cardiovascular events, and to identify CKD patients who are likely to respond to a treatment. Following such a strategy, a reliable risk of future events for a particular patient may be calculated and consequently the patient would also benefit from the best available treatment based on their risk profile. Moreover, a further step forward can be represented by the aggregation of multiple omics information by combining different techniques and/or different biological samples. This has already been shown to yield additional information by revealing with more accuracy the exact individual pathway of disease.

Published
2021

42. Mutations in the GLA Gene and LysoGb3: Is It Really Anderson-Fabry Disease?

Author

Maria Angela Losi, Riccardo Alessandro, Maurizio Postorino, Federico Pieruzzi, Paolo Colomba, Sandro Feriozzi, Yuri Battaglia, Andrea Frustaci, Alessandra Testa, Ines Monte, Daniele Masarone, Serafina Sciarrino, Antonello Giordano, Antonio Pisani, Cinzia Castana, Carmine Zoccali, Elisabetta Zachara, Giuseppe Limongelli, Eleonora Riccio, Luisa Amico, Claudio Ferri, Alessandro P. Burlina, Renzo Mignani, Margherita Stefania Rodolico, Rosa Napoletano, Marina Caserta, Carmela Zizzo, Simone Scalia, Marco Spada, Roberta Oliveri, Giuseppe Cammarata, Marco Lombardi, Cristina Chimenti, Daniele Francofonte, Giovanni Duro, Maurizio Tenuta, Giuseppe Palladino, Alberto Burlina, Camillo Autore, Giulia Polo, Maurizio Pieroni, Duro, G., Zizzo, C., Cammarata, G., Burlina, A., Polo, G., Scalia, S., Oliveri, R., Sciarrino, S., Francofonte, D., Alessandro, R., Pisani, A., Palladino, G., Napoletano, R., Tenuta, M., Masarone, D., Limongelli, G., Riccio, E., Frustaci, A., Chimenti, C., Ferri, C., Pieruzzi, F., Pieroni, M., Spada, M., Castana, C., Caserta, M., Monte, I., Rodolico, M. S., Feriozzi, S., Battaglia, Y., Amico, L., Losi, M. A., Autore, C., Lombardi, M., Zoccali, C., Testa, A., Postorino, M., Mignani, R., Zachara, E., Giordano, A., Colomba, P., Duro G., Zizzo C., Cammarata G., Burlina A., Polo G., Scalia S., Oliveri R., Sciarrino S., Francofonte D., Alessandro R., Pisani A., Palladino G., Napoletano R., Tenuta M., Masarone D., Limongelli G., Riccio E., Frustaci A., Chimenti C., Ferri C., Pieruzzi F., Pieroni M., Spada M., Castana C., Caserta M., Monte I., Rodolico M.S., Feriozzi S., Battaglia Y., Amico L., Losi M.A., Autore C., Lombardi M., Zoccali C., Testa A., Postorino M., Mignani R., Zachara E., Giordano A., Colomba P., Duro, G, Zizzo, C, Cammarata, G, Burlina, A, Polo, G, Scalia, S, Oliveri, R, Sciarrino, S, Francofonte, D, Alessandro, R, Pisani, A, Palladino, G, Napoletano, R, Tenuta, M, Masarone, D, Limongelli, G, Riccio, E, Frustaci, A, Chimenti, C, Ferri, C, Pieruzzi, F, Pieroni, M, Spada, M, Castana, C, Caserta, M, Monte, I, Rodolico, M, Feriozzi, S, Battaglia, Y, Amico, L, Losi, M, Autore, C, Lombardi, M, Zoccali, C, Testa, A, Postorino, M, Mignani, R, Zachara, E, Giordano, A, and Colomba, P

Subjects
0301 basic medicine, Proband, Male, Disease, medicine.disease_cause, Sphingolipid, Catalysi, lcsh:Chemistry, 0302 clinical medicine, Gla gene, Fabry disease, GLA gene, LysoGb3, Medicine, Child, lcsh:QH301-705.5, Spectroscopy, chemistry.chemical_classification, Genetics, Allele, Aged, 80 and over, Mutation, Computer Science Applications1707 Computer Vision and Pattern Recognition, General Medicine, Middle Aged, Phenotype, 3. Good health, Computer Science Applications, Child, Preschool, Female, Human, Adult, Adolescent, Genotype, Glycolipid, Catalysis, Article, Inorganic Chemistry, 03 medical and health sciences, Young Adult, otorhinolaryngologic diseases, Humans, Physical and Theoretical Chemistry, Molecular Biology, Gene, Alleles, Aged, Sphingolipids, business.industry, Organic Chemistry, Infant, Newborn, Infant, Biomarker, gla gene, lysogb3, adolescent, adult, aged, aged, 80 and over, alleles, amino acid substitution, biomarkers, child, child, preschool, fabry disease, female, genotype, glycolipids, humans, infant, infant, newborn, male, middle aged, phenotype, sphingolipids, young adult, alpha-galactosidase, mutation, medicine.disease, 030104 developmental biology, Enzyme, chemistry, lcsh:Biology (General), lcsh:QD1-999, Amino Acid Substitution, alpha-Galactosidase, Glycolipids, business, 030217 neurology & neurosurgery, Biomarkers
Abstract

Anderson-Fabry disease (FD) is a rare, progressive, multisystem storage disorder caused by the partial or total deficit of the lysosomal enzyme &alpha, galactosidase A (&alpha, Gal A). It is an X-linked, lysosomal enzymopathy due to mutations in the galactosidase alpha gene (GLA), encoding the &alpha, Gal A. To date, more than 900 mutations in this gene have been described. In our laboratories, the study of genetic and enzymatic alterations related to FD was performed in about 17,000 subjects with a symptomatology referable to this disorder. The accumulation of globotriaosylsphingosine (LysoGb3) was determined in blood of positives. Exonic mutations in the GLA gene were detected in 471 patients (207 Probands and 264 relatives): 71.6% of mutations were associated with the classic phenotype, 19.8% were associated with the late-onset phenotype, and 8.6% of genetic variants were of unknown significance (GVUS). The accumulation of LysoGb3 was found in all male patients with a mutation responsible for classic or late-onset FD. LysoGb3 levels were consistent with the type of mutations and the symptomatology of patients. &alpha, Gal A activity in these patients is absent or dramatically reduced. In recent years, confusion about the pathogenicity of some mutations led to an association between non-causative mutations and FD. Our study shows that the identification of FD patients is possible by associating clinical history, GLA gene analysis, &alpha, Gal A assay, and blood accumulation of LysoGB3. In our experience, LysoGB3 can be considered a reliable marker, which is very useful to confirm the diagnosis of Fabry disease.

Published
2018

43. Corpus callosum involvement: a useful clue for differentiating Fabry Disease from Multiple Sclerosis

Author

Enrico Tedeschi, Lorenzo Ugga, Silvia Migliaccio, Antonino Tuttolomondo, Massimiliano Veroux, Dario De Rosa, Sirio Cocozza, Massimo Imbriaco, Aurelio Caronia, Yuri Battaglia, Cinzia Valeria Russo, Roberta Lanzillo, Arturo Brunetti, Giuseppe Pontillo, Gaia Olivo, Antonio Pisani, Daniela Concolino, Vincenzo Brescia Morra, Federico Pieruzzi, Camilla Russo, Eleonora Riccio, Sandro Feriozzi, Cocozza S, Olivo G, Riccio E, Russo C, Pontillo G, Ugga L, Migliaccio S, de Rosa D, Feriozzi S, Veroux M, Battaglia Y, Concolino D, Pieruzzi F, Tuttolomondo A, Caronia A, Russo CV, Lanzillo R, Brescia Morra V, Imbriaco M, Brunetti A, Tedeschi E, Pisani A, Cocozza, S, Olivo, G, Riccio, E, Russo, C, Pontillo, G, Ugga, L, Migliaccio, S, de Rosa, D, Feriozzi, S, Veroux, M, Battaglia, Y, Concolino, D, Pieruzzi, F, Tuttolomondo, A, Caronia, A, Lanzillo, R, Brescia Morra, V, Imbriaco, M, Brunetti, A, Tedeschi, E, Pisani, A, Cocozza, Sirio, Olivo, Gaia, Riccio, Eleonora, Russo, Camilla, Pontillo, Giuseppe, Ugga, Lorenzo, Migliaccio, Silvia, DE ROSA, Dario, Feriozzi, Sandro, Veroux, Massimiliano, Battaglia, Yuri, Concolino, Daniela, Pieruzzi, Federico, Tuttolomondo, Antonino, Caronia, Aurelio, Russo, CINZIA VALERIA, Lanzillo, Roberta, BRESCIA MORRA, Vincenzo, Imbriaco, Massimo, Brunetti, Arturo, Tedeschi, Enrico, and Pisani, Antonio

Subjects
Adult, Male, medicine.medical_specialty, Pathology, Neurology, Settore MED/09 - Medicina Interna, Adolescent, Corpus callosum, Fluid-attenuated inversion recovery, Fabry disease, MRI, Multiple sclerosis, 030218 nuclear medicine & medical imaging, Diagnosis, Differential, 03 medical and health sciences, 0302 clinical medicine, medicine, Humans, Radiology, Nuclear Medicine and imaging, Aged, Retrospective Studies, Neuroradiology, business.industry, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Hyperintensity, Female, Neurology (clinical), Radiology, Differential diagnosis, Cardiology and Cardiovascular Medicine, business, 030217 neurology & neurosurgery
Abstract

PURPOSE: Multiple sclerosis (MS) has been proposed as a possible differential diagnosis for Fabry disease (FD). The aim of this work was to evaluate the involvement of corpus callosum (CC) on MR images and its possible role as a radiological sign to differentiate between FD and MS. METHODS: In this multicentric study, we retrospectively evaluated the presence of white matter lesions (WMLs) on the FLAIR images of 104 patients with FD and 117 patients with MS. The incidence of CC-WML was assessed in the two groups and also in a subgroup of 37 FD patients showing neurological symptoms. RESULTS: WMLs were detected in 50 of 104 FD patients (48.1%) and in all MS patients. However, a lesion in the CC was detected in only 3 FD patients (2.9%) and in 106 MS patients (90.6%). In the FD subgroup with neurological symptoms, WMLs were present in 26 of 37 patients (70.3%), with two subjects (5.4%) showing a definite callosal lesion. CONCLUSION: FD patients have a very low incidence of CC involvement on conventional MR images compared to MS, independently from the clinical presentation and the overall degree of WM involvement. Evaluating the presence of CC lesions on brain MR scans can be used as a radiological sign for a differential diagnosis between MS and FD, rapidly addressing the physician toward a correct diagnosis and subsequent treatment options.

Published
2017

44. Redefining the Pulvinar Sign in Fabry Disease

Author

Amedeo Cervo, Massimiliano Veroux, Pasquale Borrelli, Gaia Olivo, Federico Pieruzzi, Yuri Battaglia, Arturo Brunetti, Gaetano Di Palma, Renzo Mignani, Eleonora Riccio, Massimo Imbriaco, Sirio Cocozza, Enrico Tedeschi, Giuseppe Pontillo, Antonio Pisani, Camilla Russo, Daniela Concolino, Sandro Feriozzi, Cocozza, S., Russo, C., Pisani, A., Olivo, G., Riccio, E., Cervo, A., Pontillo, G., Feriozzi, S., Veroux, M., Battaglia, Y., Concolino, D., Pieruzzi, F., Mignani, R., Borrelli, P., Imbriaco, M., Brunetti, A., Tedeschi, E., Palma, G., Cocozza, S, Russo, C, Pisani, A, Olivo, G, Riccio, E, Cervo, A, Pontillo, G, Feriozzi, S, Veroux, M, Battaglia, Y, Concolino, D, Pieruzzi, F, Mignani, R, Borrelli, P, Imbriaco, M, Brunetti, A, Tedeschi, E, and Palma, G

Subjects
Adult, Male, medicine.medical_specialty, Relaxometry, Radiology, Nuclear Medicine and Imaging, Neurology, Adolescent, Population, Pulvinar, 030218 nuclear medicine & medical imaging, Young Adult, 03 medical and health sciences, 0302 clinical medicine, Nuclear Medicine and Imaging, medicine, Humans, education, Aged, Retrospective Studies, education.field_of_study, business.industry, Adult Brain, Quantitative susceptibility mapping, Enzyme replacement therapy, Middle Aged, medicine.disease, Magnetic Resonance Imaging, Fabry disease, Hyperintensity, Neurology (clinical), Cohort, Fabry Disease, Female, Radiology, business, 030217 neurology & neurosurgery
Abstract

BACKGROUND AND PURPOSE: The pulvinar sign refers to exclusive T1WI hyperintensity of the lateral pulvinar. Long considered a common sign of Fabry disease, the pulvinar sign has been reported in many pathologic conditions. The exact incidence of the pulvinar sign has never been tested in representative cohorts of patients with Fabry disease. The aim of this study was to assess the prevalence of the pulvinar sign in Fabry disease by analyzing T1WI in a large Fabry disease cohort, determining whether relaxometry changes could be detected in this region independent of the pulvinar sign positivity. MATERIALS AND METHODS: We retrospectively analyzed brain MR imaging of 133 patients with Fabry disease recruited through specialized care clinics. A subgroup of 26 patients underwent a scan including 2 FLASH sequences for relaxometry that were compared with MRI scans of 34 healthy controls. RESULTS: The pulvinar sign was detected in 4 of 133 patients with Fabry disease (3.0%). These 4 subjects were all adult men (4 of 53, 7.5% of the entire male population) with renal failure and under enzyme replacement therapy. When we tested for discrepancies between Fabry disease and healthy controls in quantitative susceptibility mapping and relaxometry maps, no significant difference emerged for any of the tested variables. CONCLUSIONS: The pulvinar sign has a significantly lower incidence in Fabry disease than previously described. This finding, coupled with a lack of significant differences in quantitative MR imaging, allows hypothesizing that selective involvement of the pulvinar is a rare neuroradiologic sign of Fabry disease.

Published
2017

45. New insights from the application of the FAbry STabilization indEX in a large population of Fabry cases

Author

Mario Mangeri, Sandro Feriozzi, Federico Pieruzzi, Yuri Battaglia, Elena Verrecchia, Ilaria Tanini, Gianluca De Danieli, Marco Spada, Renzo Mignani, Antonio Pisani, Maurizio Pieroni, Mignani, R, Pieroni, M, Pisani, A, Spada, M, Battaglia, Y, Verrecchia, E, Mangeri, M, Feriozzi, S, Tanini, I, De Danieli, G, Pieruzzi, F, Mignani, R., Pieroni, M., Pisani, A., Spada, M., Battaglia, Y., Verrecchia, E., Mangeri, M., Feriozzi, S., Tanini, I., De Danieli, G., and Pieruzzi, F.

Subjects
Pediatrics, medicine.medical_specialty, Population, 030232 urology & nephrology, Large population, Correlation, 03 medical and health sciences, 0302 clinical medicine, disease progression, Medicine, AcademicSubjects/MED00340, education, a-galactosidase A/a-galactosidase A deficiency, disease stability, education.field_of_study, Fabry disease, Transplantation, Errata, Receiver operating characteristic, business.industry, Gold standard (test), medicine.disease, α-galactosidase A/α-galactosidase A deficiency, organ dysfunction scores, a-galactosidase A/a-galactosidase A deficiency, disease progression, disease stability, Fabry disease, organ dysfunction scores, Nephrology, Cohort, Population study, business, organ dysfunction score
Abstract

Background: The FAbry STabilization indEX (FASTEX) is an innovative index allowing the assessment of clinical stability over time in Fabry disease patients. This index was developed in a population of 28 male patients with the classical form of Fabry disease. Objectives: The aim of the study was to test the accuracy of the FASTEX in evaluating Fabry disease stability in 132 male and female patients with classical and non-classical Fabry disease from nine Italian centres and it also aimed to define the sensitivity and specificity of this new tool. In particular, we aimed to investigate the correlation between the FASTEX and clinical judgement in a large-scale cohort of the study population. Methods: Statistical methods applied to this investigation included the calculation of accuracy, specificity and sensitivity, receiver operating characteristic (ROC) curves and Cohen’s j index related to the FASTEX and clinical judgement. Results: The patient population included 58 males (43.9%). The mean age of the overall population was 46.3 6 15. 1 years (range 31.2–61.4). The median interval between the two multidisciplinary evaluations used for FASTEX calculation was 398 days. Since no gold standard method is available to define the overall clinical condition of Fabry patients over time, the results of the FASTEX were compared with clinical judgements given by the physicians involved in this study. In this way, the FASTEX classified 121 of 132 (92%) patients correctly. In particular, the FASTEX correctly identified 93% (41/44) of clinically ‘unstable’ and 91% (80/88) of clinically ‘stable’ patients. The area under the curve of the ROC related to the FASTEX index cut-off (20) was equal to 0.967, very close to its theoretical maximum (1), which means that it is an excellent test for classifying patients as ‘stable’ or ‘unstable’ compared with clinical judgement. In addition, the FASTEX cut-off >20 provides themost acceptable balance between sensitivity and specificity. The Cohen’s j index value obtained in our study was 0.82, showing a highly statistically significant P-value

Published
2019
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46. Evaluation of Hypertension, Proteinuria, and Abnormalities of Body Weight in Italian Adolescents Participating in the World Kidney Days

Author

Domenico Russo, Pasquale Esposito, Alessandro Balducci, Luigi Russo, Alda Storari, Yuri Battaglia, Salvatore Corrao, Battaglia Y., Esposito P., Corrao S., Russo L., Balducci A., Storari A., Russo D., Battaglia, Yuri, Esposito, Pasquale, Corrao, Salvatore, Russo, Domenico, Balducci, Alessandro, and Storari, Alda

Subjects
Adult, Male, Adolescents, Anthropometric indicators, BMI, Hypertension, Obesity, Proteinuria, lcsh:Diseases of the circulatory (Cardiovascular) system, medicine.medical_specialty, Waist, Adolescent, Anthropometric indicators · BMI · Proteinuria · Hypertension · Obesity · Adolescents, 030232 urology & nephrology, Overweight, Kidney, lcsh:RC870-923, Young Adult, 03 medical and health sciences, anthropometric indicators, 0302 clinical medicine, Risk Factors, Internal medicine, lcsh:Dermatology, Anthropometric indicator, medicine, Humans, adolescents, cardiovascular diseases, business.industry, Body Weight, General Medicine, lcsh:RL1-803, Anthropometry, lcsh:Diseases of the genitourinary system. Urology, medicine.disease, Blood pressure, Italy, lcsh:RC666-701, Nephrology, Female, proteinuria, Underweight, medicine.symptom, Cardiology and Cardiovascular Medicine, business, Body mass index
Abstract

Introduction: World Kidney Day (WKD) was promoted by the Italian Kidney Foundation and the Italian Society of Nephrology for raising awareness, detection, prevention, and treatment of kidney diseases. The Italian WKD focused on the “School Project” by screening students attending the fifth year of high school. The main goal of the “School Project” was to assess in healthy adolescents the presence of hypertension (HTN) and proteinuria; as well as to evaluate potential interrelations between overweight, obesity (both measured with different anthropometric methods), blood pressure (BP) levels, and proteinuria. The ancillary goal was to have an estimate of awareness on some nephrology topics. Methods: The study population consisted of 17- to 19-year-old students. HTN was defined as systolic BP (SBP) ≥140 mm Hg and/or diastolic BP (DBP) ≥90 mm Hg. Isolated systolic hypertension (ISH) was defined as SBP ≥140 mm Hg and DBP 120 mm Hg but 80 mm Hg but

Published
2020
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47. Trabecular complexity as an early marker of cardiac involvement in Fabry disease

Author

Silvia Pica, Davide Lazzeroni, Kelvin Chow, Alessandro P. Burlina, Francesco Moroni, Yuri Battaglia, Antonia Camporeale, Federico Pieruzzi, Maurizio Pieroni, Massimo Lombardi, Francesca Carubbi, Marco Spada, Paolo G. Camici, Paola Lusardi, Francesca Graziani, Renzo Mignani, Silvia Garibaldi, Laura Econimo, Camporeale, A, Moroni, F, Lazzeroni, D, Garibaldi, S, Pieroni, M, Pieruzzi, F, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Econimo, L, Battaglia, Y, Graziani, F, Pica, S, Chow, K, Camici, P, and Lombardi, M

Subjects
Male, medicine.medical_specialty, Left, Cardiomyopathy, 030204 cardiovascular system & hematology, fractal analysis, Left ventricular hypertrophy, Endocardial border, cardiac magnetic resonance, Ventricular Function, Left, 030218 nuclear medicine & medical imaging, 03 medical and health sciences, Basal (phylogenetics), 0302 clinical medicine, Fabry disease, T1 mapping, Internal medicine, Medicine, Ventricular Function, Humans, Radiology, Nuclear Medicine and imaging, Prospective Studies, business.industry, General Medicine, Hypertrophy, medicine.disease, Left Ventricular, fractal analysi, Settore MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, Cardiology, Population study, Fabry Disease, Hypertrophy, Left Ventricular, Cardiology and Cardiovascular Medicine, business, Cardiac magnetic resonance, Cardiomyopathies
Abstract

Aims Fabry cardiomyopathy is characterized by glycosphingolipid storage and increased myocardial trabeculation has also been demonstrated. This study aimed to explore by cardiac magnetic resonance whether myocardial trabecular complexity, quantified by endocardial border fractal analysis, tracks phenotype evolution in Fabry cardiomyopathy. Methods and results Study population included 20 healthy controls (12 males, age 32±9) and 45 Fabry patients divided into three groups: 15 left ventricular hypertrophy (LVH)-negative patients with normal T1 (5 males, age 28±13; Group 1); 15 LVH-negative patients with low T1 (9 males, age 33±9.6; Group 2); 15 LVH-positive patients (11 males, age 53.5±9.6; Group 3). Trabecular fractal dimensions (Dfs) (total, basal, mid-ventricular, and apical) were evaluated on cine images. Total Df was higher in all Fabry groups compared to controls, gradually increasing from controls to Group 3 (1.27±0.02 controls vs. 1.29±0.02 Group 1 vs. 1.30±0.02 Group 2 vs. 1.34±0.02 Group 3; P Conclusion Fabry cardiomyopathy is characterized by a progressive increase in Df of endocardial trabeculae together with shortening of T1 values. Myocardial trabeculation is increased before the presence of detectable sphingolipid storage, thus representing an early sign of cardiac involvement.

Published
2020

48. Atrial Dysfunction Assessed by Cardiac Magnetic Resonance as an Early Marker of Fabry Cardiomyopathy

Author

Andrea Bernardini, Francesca Carubbi, Maurizio Pieroni, Yuri Battaglia, Kelvin Chow, Alessandro P. Burlina, Marco Spada, Paola Lusardi, Silvia Pica, Renzo Mignani, Stefano Figliozzi, Massimo Lombardi, Francesca Graziani, Lara Tondi, Sara Boveri, Iacopo Olivotto, Antonia Camporeale, Federico Pieruzzi, Bernardini, A, Camporeale, A, Pieroni, M, Pieruzzi, F, Figliozzi, S, Lusardi, P, Spada, M, Mignani, R, Burlina, A, Carubbi, F, Battaglia, Y, Graziani, F, Pica, S, Tondi, L, Chow, K, Boveri, S, Olivotto, I, and Lombardi, M

Subjects
medicine.medical_specialty, Magnetic Resonance Spectroscopy, Cardiomyopathy, 030204 cardiovascular system & hematology, Anderson-Fabry disease, 030218 nuclear medicine & medical imaging, Muscle hypertrophy, 03 medical and health sciences, chemistry.chemical_compound, 0302 clinical medicine, Left atrial, Predictive Value of Tests, Internal medicine, Medicine, Humans, Radiology, Nuclear Medicine and imaging, Fabry disease, cardiomyopathy, atrial dysfunction, cardiac magnetic resonance, T1 mapping, cardiovascular diseases, Atrial myocytes, Heart Atria, LA strain, business.industry, Anderson-Fabry disease, T1 mapping, LA strain, Glycosphingolipid, T1 mapping, medicine.disease, Anderson-Fabry Disease, chemistry, Settore MED/11 - MALATTIE DELL'APPARATO CARDIOVASCOLARE, cardiovascular system, Cardiology, Cardiology and Cardiovascular Medicine, business, Cardiac magnetic resonance, Cardiomyopathies
Abstract

Anderson-Fabry disease (AFD) cardiomyopathy is characterized by glycosphingolipid (Gb3) storage in all cellular components, with consequent left ventricular hypertrophy (LVH). Gb3 accumulation also involves atrial myocytes ([1][1]), ultimately leading to left atrial (LA) enlargement and reduced

Published
2020

49. Parapelvic cysts, a distinguishing feature of renal Fabry Disease

Author

Massimo Sabbatini, Dario Bruzzese, Daniela Concolino, Sirio Cocozza, Carmela Zizzo, Leonardo Caroti, Massimiliano Veroux, Raffaele Manna, Michele Santangelo, Massimo Imbriaco, Luigi Petruzzelli Annicchiarico, Angela Maria Pellegrino, Yuri Battaglia, Antonio Pisani, Sandro Feriozzi, Enrico Tedeschi, Dario De Rosa, Simona Sestito, Federico Pieruzzi, Eleonora Riccio, Renzo Mignani, Pisani, Antonio, Annicchiarico, Luigi Petruzzelli, Pellegrino, ANGELA MARIA, Bruzzese, Dario, Feriozzi, Sandro, Imbriaco, Massimo, Tedeschi, Enrico, Cocozza, Sirio, Rosa, Dario De, Mignani, Renzo, Veroux, Massimiliano, Battaglia, Yuri, Concolino, Daniela, Sestito, Simona, Pieruzzi, Federico, Caroti, Leonardo, Manna, Raffaele, Zizzo, Carmela, Santangelo, Michele, Sabbatini, Massimo, Riccio, Eleonora, Pisani, A, Petruzzelli Annicchiarico, L, Pellegrino, A, Bruzzese, D, Feriozzi, S, Imbriaco, M, Tedeschi, E, Cocozza, S, De Rosa, D, Mignani, R, Veroux, M, Battaglia, Y, Concolino, D, Sestito, S, Pieruzzi, F, Caroti, L, Manna, R, Zizzo, C, Santangelo, M, Sabbatini, M, and Riccio, E

Subjects
0301 basic medicine, Adult, Male, medicine.medical_specialty, Pathology, biomarkers, chronic renal failure, Fabry disease, parapelvic cyst, ultrasonography, Nephrology, Transplantation, 030232 urology & nephrology, Disease, Gastroenterology, 03 medical and health sciences, 0302 clinical medicine, Internal medicine, Prevalence, Medicine, Humans, Family history, Retrospective Studies, Ultrasonography, business.industry, Settore MED/09 - MEDICINA INTERNA, Ultrasound, Renal ultrasound, biomarkers, chronic renal failure, Fabry disease, parapelvic cyst, ultrasonography, Enzyme replacement therapy, Kidney Diseases, Cystic, Middle Aged, medicine.disease, Control subjects, Prognosis, 030104 developmental biology, Cross-Sectional Studies, Italy, alpha-Galactosidase, Cohort, biomarker, Fabry Disease, Female, business
Abstract

Background Fabry's disease (FD) is a rare, multi-organ lysosomal disease, caused by the deficiency of the enzyme α-galactosidase A, and is difficult to diagnose. Although parapelvic cysts (PC) were previously associated with FD, their prevalence and significance are unclear. Methods The present study aimed to: (i) evaluate, by renal ultrasound, the real prevalence of PC and of their determinants in a multicentre, nationwide cohort of FD patients (n = 173, Study 1) and (ii) ascertain whether a greater accuracy of PC detection improved their identification, in FD patients from a single centre (n = 67, Study 2). In both studies, for each FD patient, an age- and renal function-matched subject was selected for comparison (1:1). Results In Study 1, PC were detected in 28.9% of FD subjects and in only 1.1% of control subjects (P

Published
2018

50. Exercise in Patients on Dialysis: A Multicenter, Randomized Clinical Trial

Author

Rossella Baggetta, Daniele Ciurlino, Nicola Lamberti, Fabrizio Fabrizi, Luciano De Paola, Chiara Summaria, Enrico Pozzato, Giorgio Fuiano, Fabio Manfredini, Graziella D'Arrigo, Silvio Bertoli, Piergiorgio Messa, Samar Abd ElHafeez, Yuri Battaglia, Anna Maria Malagoni, Carmine Zoccali, Francesca Mallamaci, Claudia Torino, Pasquale Fabio Provenzano, Renato Rapanà, Alessandro Zuccalà, Stefania Rastelli, Lisa Rocca-Rey, Gaetano Lucisano, Davide Bolignano, Michele Felisatti, Pasquale Fatuzzo, Luigi Lombardi, Graziella Bonanno, Luigi Catizone, Pietro Castellino, Francesco Rapisarda, Giovanni Tripepi, Antonio Barillà, Adamasco Cupisti, Filippo Aucella, Manfredini, F, Mallamaci, F, D'Arrigo, G, Baggetta, R, Bolignano, D, Torino, C, Lamberti, N, Bertoli, S, Ciurlino, D, Rocca-rey, L, Barillà , A, Battaglia, Y, Rapanà, R, Zuccalà, A, Bonanno, G, Fatuzzo, P, Rapisarda, F, Rastelli, S, Fabrizi, F, Messa, P, De Paola, L, Lombardi, L, Cupisti, A, Fuiano, G, Lucisano, G, Summaria, C, Felisatti, M, Pozzato, E, Malagoni, A, Castellino, P, Aucella, F, Elhafeez, S, Provenzano, P, Tripepi, G, Catizone, L, and Zoccali, C

Subjects
Male, medicine.medical_specialty, dialysi, medicine.medical_treatment, Physical fitness, 030232 urology & nephrology, Physical exercise, Walking, 030204 cardiovascular system & hematology, law.invention, NO, rehabilitation, 03 medical and health sciences, CKD, dialysis, exercise, physical functioning, rehabilitation, six minute walking test, 0302 clinical medicine, CKD, dialysis, exercise, physical functioning, six minute walking test, Combined Modality Therapy, Female, Humans, Middle Aged, Renal Insufficiency, Chronic, Exercise Therapy, Physical Fitness, Quality of Life, Renal Dialysis, Nephrology, Randomized controlled trial, law, Renal Dialysi, Multicenter trial, medicine, Renal Insufficiency, Chronic, Dialysis, business.industry, Continuous ambulatory peritoneal dialysis, General Medicine, medicine.disease, Physical therapy, Physical Fitne, Hemodialysis, business, Kidney disease, Human
Abstract

Previous studies have suggested the benefits of physical exercise for patients on dialysis. We conducted the Exercise Introduction to Enhance Performance in Dialysis trial, a 6-month randomized, multicenter trial to test whether a simple, personalized walking exercise program at home, managed by dialysis staff, improves functional status in adult patients on dialysis. The main study outcomes included change in physical performance at 6 months, assessed by the 6-minute walking test and the five times sit-to-stand test, and in quality of life, assessed by the Kidney Disease Quality of Life Short Form (KDQOL-SF) questionnaire. We randomized 296 patients to normal physical activity (control; n=145) or walking exercise (n=151); 227 patients (exercise n=104; control n=123) repeated the 6-month evaluations. The distance covered during the 6-minute walking test improved in the exercise group (mean distance +/- SD: baseline, 328 +/- 96 m; 6 months, 367 +/- 113 m) but not in the control group (baseline, 321 +/- 107 m; 6 months, 324 +/- 116 m; P

Published
2017

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