North Star Ambulatory Assessment, 6-minute walk test and timed items in ambulant boys with Duchenne muscular dystrophy

被引：167

作者：

Mazzone, Elena ^{[1
]}

Martinelli, Diego ^{[1
]}

Berardinelli, Angela ^{[2
]}

Messina, Sonia ^{[1
,3
]}

D'Amico, Adele ^{[4
]}

Vasco, Gessica ^{[1
]}

Main, Marion ^{[5
]}

Doglio, Luca ^{[6
]}

Politano, Luisa ^{[7
]}

Cavallaro, Filippo ^{[3
]}

Frosini, Silvia ^{[8
]}

Bello, Luca ^{[9
]}

Carlesi, Adelina ^{[4
]}

Bonetti, Anna Maria ^{[4
]}

Zucchini, Elisabetta ^{[10
]}

De Sanctis, Roberto ^{[1
]}

Scutifero, Marianna ^{[7
]}

Bianco, Flaviana ^{[1
]}

Rossi, Francesca ^{[11
]}

Motta, Maria Chiara ^{[2
]}

Sacco, Annalisa ^{[1
]}

Donati, Maria Alice ^{[13
]}

Mongini, Tiziana ^{[11
]}

Pini, Antonella ^{[10
]}

Battini, Roberta ^{[8
]}

Pegoraro, Elena ^{[9
]}

Pane, Marika ^{[1
]}

Pasquini, Elisabetta ^{[13
]}

Bruno, Claudio ^{[6
]}

Vita, Giuseppe ^{[3
]}

de Waure, Chiara ^{[12
]}

Bertini, Enrico ^{[4
]}

Mercuri, Eugenio ^{[1
,5
]}

机构：

[1] Catholic Univ, Dept Paediat Neurol, Rome, Italy

[2] Univ Pavia, IRCCS C Mondino Fdn, Child Neurol & Psychiat Unit, I-27100 Pavia, Italy

[3] Univ Messina, Dept Neurosci Psychiat & Anaesthesiol, Messina, Italy

[4] Bambino Gesu Pediat Hosp, Dept Lab Med, Mol Med Unit, Rome, Italy

[5] Inst Child Hlth, Dubowitz Neuromuscular Unit, London, England

[6] G Gaslini Inst Children, Neuromuscular Dis Unit, Genoa, Italy

[7] Seconda Univ Napoli, Dipartimento Med Chirurg Internist Clin & Sperime, Naples, Italy

[8] Univ Pisa, Stella Maris Inst, Dept Dev Neurosci, I-56100 Pisa, Italy

[9] Univ Padua, Dept Neurosci, Padua, Italy

[10] Maggiore Hosp, Child Neurol & Psychiat Unit, Bologna, Italy

[11] Univ Turin, SG Battista Hosp, Neuromuscular Ctr, I-10124 Turin, Italy

[12] Catholic Univ, Dept Stat, Rome, Italy

[13] Meyer Hosp, Metab & Neuromuscular Unit, Florence, Italy

来源：

NEUROMUSCULAR DISORDERS | 2010年 / 20卷 / 11期

关键词：

North Star Ambulatory Assessment; Duchenne muscular dystrophy; 6-Minute walk test; MULTICENTER CLINICAL-TRIALS; FUNCTIONAL MOTOR SCALE; NEUROMUSCULAR DISEASES; CHILDREN; RELIABILITY; ABILITY; PAIN;

D O I：

10.1016/j.nmd.2010.06.014

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

100204 [神经病学];

摘要：

The North Star Ambulatory Assessment is a functional scale specifically designed for ambulant boys affected by Duchenne muscular dystrophy (DMD). Recently the 6-minute walk test has also been used as an outcome measure in trials in DMD. The aim of our study was to assess a large cohort of ambulant boys affected by DMD using both North Star Assessment and 6-minute walk test. More specifically, we wished to establish the spectrum of findings for each measure and their correlation. This is a prospective multicentric study involving 10 centers. The cohort included 112 ambulant DMD boys of age ranging between 4.10 and 17 years (mean 8.18 +/- 2.3 DS). Ninety-one of the 112 were on steroids: 37/91 on intermittent and 54/91 on daily regimen. The scores on the North Star assessment ranged from 6/34 to 34/34. The distance on the 6-minute walk test ranged from 127 to 560.6 m. The time to walk 10 m was between 3 and 15s. The time to rise from the floor ranged from 1 to 27.5 s. Some patients were unable to rise from the floor. As expected the results changed with age and were overall better in children treated with daily steroids. The North Star assessment had a moderate to good correlation with 6-minute walk test and with timed rising from floor but less with 10 m timed walk/run test. The 6-minute walk test in contrast had better correlation with 10 m timed walk/run test than with timed rising from floor. These findings suggest that a combination of these outcome measures can be effectively used in ambulant DMD boys and will provide information on different aspects of motor function, that may not be captured using a single measure. (C) 2010 Published by Elsevier B.V.

引用

页码：712 / 716

页数：5

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