Cross-sectional retrospective study of muscle function in patients with glycogen storage disease type III
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- 作者：Valé ; rie Decostrea ; v.decostre@institut-myologie.org" class="auth_mail" title="E-mail the corresponding author ; Pascal Laforê ; tb ; c ; Aleksandra Nadaj-Paklezad ; Marie De Antonioe ; Sylvain Leveuglee ; Gwenn Olliviera ; Auré ; lie Canala ; Kahina Kachetelb ; Franç ; ois Petitf ; Bruno Eymardb ; Anthony Behinb ; Karim Wahbia ; g ; Philippe Labruneh ; i ; Jean-Yves Hogrela
- 关键词：Glycogen storage disease type III ; Debranching enzyme deficiency ; Metabolic myopathy ; Outcome measures
- 刊名：Neuromuscular Disorders
- 出版年：2016
- 出版时间：September 2016
- 年：2016
- 卷：26
- 期：9
- 页码：584-592
- 全文大小：709 K
文摘
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Age-related muscle outcomes in glycogen storage disease type III were analysed.
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Purdue test, key pinch and knee extension strength are the most age-dependent tests.
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Handgrip strength and the MFM become age sensitive outcomes after the third decade.
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MMT and elbow extension strength are not sensitive outcomes in GSDIII patients.
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The minimal number of subjects was calculated for future longitudinal trials.