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Timed Rise from Floor as a Predictor of Disease Progression in Duchenne Muscular Dystrophy: An Observational Study.
Mazzone, Elena S; Coratti, Giorgia; Sormani, Maria Pia; Messina, Sonia; Pane, Marika; D'Amico, Adele; Colia, Giulia; Fanelli, Lavinia; Berardinelli, Angela; Gardani, Alice; Lanzillotta, Valentina; D'Ambrosio, Paola; Petillo, Roberta; Cavallaro, Filippo; Frosini, Silvia; Bello, Luca; Bonfiglio, Serena; De Sanctis, Roberto; Rolle, Enrica; Forcina, Nicola; Magri, Francesca; Vita, Gianluca; Palermo, Concetta; Donati, Maria Alice; Procopio, Elena; Arnoldi, Maria Teresa; Baranello, Giovanni; Mongini, Tiziana; Pini, Antonella; Battini, Roberta; Pegoraro, Elena; Torrente, Yvan; Previtali, Stefano C; Bruno, Claudio; Politano, Luisa; Comi, Giacomo P; D'Angelo, Maria Grazia; Bertini, Enrico; Mercuri, Eugenio.
Afiliação
  • Mazzone ES; Department of Paediatric Neurology, Catholic University, Rome, Italy.
  • Coratti G; Department of Paediatric Neurology, Catholic University, Rome, Italy.
  • Sormani MP; Biostatistics Unit, Department of Health Sciences, University of Genoa, Genoa, Italy.
  • Messina S; Department of Neurosciences and Nemo and Clinical Center, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy.
  • Pane M; Department of Paediatric Neurology, Catholic University, Rome, Italy.
  • D'Amico A; Unit of Neuromuscular and Neurodegenerative Diseases, Department of Neurosciences, Bambino Gesù Children's Hospital, Rome, Italy.
  • Colia G; Unit of Neuromuscular and Neurodegenerative Diseases, Department of Neurosciences, Bambino Gesù Children's Hospital, Rome, Italy.
  • Fanelli L; Department of Paediatric Neurology, Catholic University, Rome, Italy.
  • Berardinelli A; Child Neurology and Psychiatry Unit, "C. Mondino" Foundation, Pavia, Italy.
  • Gardani A; Child Neurology and Psychiatry Unit, "C. Mondino" Foundation, Pavia, Italy.
  • Lanzillotta V; Neuromuscular Disease Unit, G. Gaslini Institute, Genoa, Italy.
  • D'Ambrosio P; Cardiomiologia e genetica medica, Dipartimento di Medicina Sperimentale, Seconda Università di Napoli, Napoli, Italy.
  • Petillo R; Cardiomiologia e genetica medica, Dipartimento di Medicina Sperimentale, Seconda Università di Napoli, Napoli, Italy.
  • Cavallaro F; Department of Neurosciences and Nemo and Clinical Center, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy.
  • Frosini S; Department of Developmental Neuroscience, Stella Maris Institute, Pisa,Italy.
  • Bello L; Department of Neurosciences, University of Padua, Padua, Italy.
  • Bonfiglio S; Child Neurology and Psychiatry Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
  • De Sanctis R; Department of Paediatric Neurology, Catholic University, Rome, Italy.
  • Rolle E; Neuromuscular Center, SG. Battista Hospital, University of Turin, Turin, Italy.
  • Forcina N; Department of Paediatric Neurology, Catholic University, Rome, Italy.
  • Magri F; Dino Ferrari Centre, Neuroscience Section, Department of Pathophysiology and Transplantation (DEPT), University of Milan, Neurology Unit, IRCSS Foudation, Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Vita G; Department of Neurosciences and Nemo and Clinical Center, Psychiatry and Anaesthesiology, University of Messina, Messina, Italy.
  • Palermo C; Department of Paediatric Neurology, Catholic University, Rome, Italy.
  • Donati MA; Metabolic and Neuromuscular Unit, Meyer Hospital, Florence, Italy.
  • Procopio E; Metabolic and Neuromuscular Unit, Meyer Hospital, Florence, Italy.
  • Arnoldi MT; Developmental Neurology Unit, Istituto Neurologico "Besta" Milan, Milan, Italy.
  • Baranello G; Developmental Neurology Unit, Istituto Neurologico "Besta" Milan, Milan, Italy.
  • Mongini T; Neuromuscular Center, SG. Battista Hospital, University of Turin, Turin, Italy.
  • Pini A; Child Neurology and Psychiatry Unit, IRCCS Istituto delle Scienze Neurologiche di Bologna, Bologna, Italy.
  • Battini R; Department of Developmental Neuroscience, Stella Maris Institute, Pisa,Italy.
  • Pegoraro E; Department of Neurosciences, University of Padua, Padua, Italy.
  • Torrente Y; Dino Ferrari Centre, Neuroscience Section, Department of Pathophysiology and Transplantation (DEPT), University of Milan, Neurology Unit, IRCSS Foudation, Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • Previtali SC; Neuromuscular repair unit, Inspe and division of neuroscience, IRCSS San Raffaele Scientific Institute, Milan, Italy.
  • Bruno C; Neuromuscular Disease Unit, G. Gaslini Institute, Genoa, Italy.
  • Politano L; Cardiomiologia e genetica medica, Dipartimento di Medicina Sperimentale, Seconda Università di Napoli, Napoli, Italy.
  • Comi GP; Dino Ferrari Centre, Neuroscience Section, Department of Pathophysiology and Transplantation (DEPT), University of Milan, Neurology Unit, IRCSS Foudation, Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
  • D'Angelo MG; Neuromuscular Disorders Unit, Scientific Institute IRCCS E. Medea, 23842, Bosisio Parini, Lecco, Italy.
  • Bertini E; Unit of Neuromuscular and Neurodegenerative Diseases, Department of Neurosciences, Bambino Gesù Children's Hospital, Rome, Italy.
  • Mercuri E; Department of Paediatric Neurology, Catholic University, Rome, Italy.
PLoS One ; 11(3): e0151445, 2016.
Article em En | MEDLINE | ID: mdl-26982196
ABSTRACT

BACKGROUND:

The role of timed items, and more specifically, of the time to rise from the floor, has been reported as an early prognostic factor for disease progression and loss of ambulation. The aim of our study was to investigate the possible effect of the time to rise from the floor test on the changes observed on the 6MWT over 12 months in a cohort of ambulant Duchenne boys. SUBJECTS AND

METHODS:

A total of 487 12-month data points were collected from 215 ambulant Duchenne boys. The age ranged between 5.0 and 20.0 years (mean 8.48 ±2.48 DS).

RESULTS:

The results of the time to rise from the floor at baseline ranged from 1.2 to 29.4 seconds in the boys who could perform the test. 49 patients were unable to perform the test at baseline and 87 at 12 month The 6MWT values ranged from 82 to 567 meters at baseline. 3 patients lost the ability to perform the 6mwt at 12 months. The correlation between time to rise from the floor and 6MWT at baseline was high (r = 0.6, p<0.01).

CONCLUSIONS:

Both time to rise from the floor and baseline 6MWT were relevant for predicting 6MWT changes in the group above the age of 7 years, with no interaction between the two measures, as the impact of time to rise from the floor on 6MWT change was similar in the patients below and above 350 m. Our results suggest that, time to rise from the floor can be considered an additional important prognostic factor of 12 month changes on the 6MWT and, more generally, of disease progression.
Assuntos

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Distrofia Muscular de Duchenne Tipo de estudo: Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Adult / Child / Child, preschool / Humans / Male Idioma: En Revista: PLoS One Ano de publicação: 2016 Tipo de documento: Article

Texto completo: 1 Coleções: 01-internacional Base de dados: MEDLINE Assunto principal: Distrofia Muscular de Duchenne Tipo de estudo: Observational_studies / Prognostic_studies / Risk_factors_studies Limite: Adolescent / Adult / Child / Child, preschool / Humans / Male Idioma: En Revista: PLoS One Ano de publicação: 2016 Tipo de documento: Article