Longitudinal changes in respiratory and upper limb function in a pediatric type III spinal muscular atrophy cohort after loss of ambulation,Muscle & Nerve

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Longitudinal changes in respiratory and upper limb function in a pediatric type III spinal muscular atrophy cohort after loss of ambulation
Muscle & Nerve ( IF 2.8 ) Pub Date : 2021-08-25 , DOI: 10.1002/mus.27404
Amy Wolfe _{1,

2} , Mariacristina Scoto _{1,

2} , Evelin Milev _{1,

2} , Robert Muni Lofra ₃ , Lianne Abbott _{1,

2} , Ruth Wake ₃ , Annemarie Rohwer _{1,

2} , Marion Main ₁ , Giovanni Baranello _{1,

2} , Anna Mayhew ₃ , Chiara Marini-Bettolo ₃ , Francesco Muntoni _{1,

2}

Affiliation

Spinal muscular atrophy (SMA) type III is a relatively mild form of SMA. Few studies have investigated the changes in both respiratory and upper limb function within this population after loss of ambulation. The aim of this study was to assess change in percentage of predicted forced vital capacity (FVC% predicted) and change in the Revised Upper Limb Module (RULM) score in these patients throughout a 24-month period after loss of ambulation. Effect of scoliosis and its surgical correction, disease duration since loss of ambulation, weight, and height were also investigated.

中文翻译：

小儿 III 型脊髓性肌萎缩症患者丧失行走能力后呼吸和上肢功能的纵向变化

III 型脊髓性肌萎缩 (SMA) 是一种相对温和的 SMA。很少有研究调查该人群在失去行走后呼吸和上肢功能的变化。本研究的目的是评估这些患者在失去行走后的 24 个月期间内预测用力肺活量百分比的变化（FVC% 预测）和修订上肢模块 (RULM) 评分的变化。还研究了脊柱侧弯及其手术矫正的影响、自失去行走后的疾病持续时间、体重和身高。

更新日期：2021-10-15

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