12 Mart 2025 Veri seti Açık Erişim

Dansuk Emre; Tunalı Van Den Berg Nur; Ata Görkem; Kutlutürk Yıkılmaz Seval; Öktem Sedat

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    <subfield code="a">Type 1 SMA, scoliosis, bell shaped chest, rehabilitation, spinal orthosis.</subfield>
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    <subfield code="a">&lt;p&gt;&lt;strong&gt;Background: &lt;/strong&gt;Type 1 spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by severe muscle weakness, which results in progressive spinal and chest deformities. This study aims to evaluate the effect of spinal orthosis use along with pulmonary care (PC), individualized pulmonary rehabilitation (IPR), and individualized trunk exercises (ITE) in children with Type 1 SMA.&lt;/p&gt;

&lt;p&gt;&lt;strong&gt;Methods: &lt;/strong&gt;The study enrolled 24 children with Type 1 SMA aged 2&amp;ndash;6 years, with a scoliosis angle of 20&amp;deg;&amp;ndash;40&amp;deg;. Participants were randomly assigned into two groups using a stratified randomization method: Group 1 (PC, IPR, ITE) and Group 2 (PC, IPR, ITE &amp;amp; spinal orthosis). All participants underwent an 8-week treatment program. Pre- and post-treatment assessments included scoliosis progression measured by the Cobb angle, chest deformity evaluated through the basal upper-lower chest wall ratio and the Supine Angle of Trunk Rotation Test (SATR), and motor function levels assessed using the Children&amp;#39;s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND).&lt;/p&gt;

&lt;p&gt;&lt;strong&gt;Results: &lt;/strong&gt;Significant improvements were observed in Cobb angle, bell-shaped chest deformity, and motor function in both groups (p&amp;lt;0.05). Group 2 demonstrated greater improvements in effect size (ES) across all evaluation parameters. Compared to Group 1, Group 2 showed superior improvement in Cobb angle (ES=3.98), basal upper-lower chest wall ratio (ES=5.00), SATRL (lower) (ES=2.55), SATRU (upper) (ES=1.64), and CHOP INTEND (ES=1.23) (p&amp;lt;0.05).&lt;/p&gt;

&lt;p&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; The combination of PC, IPR, ITE, and spinal orthosis demonstrated superior effectiveness in Type 1 SMA, marking this as the first study to highlight its benefits. Further validation through large-scale studies with long-term follow-ups is necessary.&lt;/p&gt;</subfield>
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    <subfield code="0">(orcid)0000-0003-4415-8101</subfield>
    <subfield code="a">Dansuk Emre</subfield>
    <subfield code="u">Istanbul Medipol University</subfield>
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    <subfield code="a">The effect of spinal orthosis on scoliosis progression and chest deformity in children with type 1 spinal muscular atrophy: a randomized controlled trial</subfield>
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