Obstructive airway in Morquio A syndrome, the past, the present and the future
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- 作者：Shunji Tomatsua ; 1 ; stomatsu@nemours.org" class="auth_mail" title="E-mail the corresponding author ; Lauren W. Averilla ; 1 ; Kazuki Sawamotoa ; William G. Mackenziea ; Michael B. Bobera ; Christian Pizarroa ; Christopher J. Goffa ; Li Xiea ; Tadao Oriib ; Mary Therouxa ; 2 ; Mary.Theroux@nemours.org" class="auth_mail" title="E-mail the corresponding author
- 关键词：C6S ; chondroitin-6-sulfate ; ECM ; extracellular matrix ; ERT ; enzyme replacement therapy ; GALNS ; N-acetylgalactosamine-6-sulfate sulfatase ; KS ; keratan sulfate ; MR images ; Magnetic resonance images ; MRI ; Magnetic resonance imaging
- 刊名：Molecular Genetics and Metabolism
- 出版年：2016
- 出版时间：February 2016
- 年：2016
- 卷：117
- 期：2
- 页码：150-156
- 全文大小：959 K
文摘
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Tracheal obstruction is life-threatening in Morquio A.
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High frequency of tracheal obstruction is observed.
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The cause of tracheal obstruction is due to an imbalance of growth.
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Brachiocephalic artery compresses trachea.
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Patients over 15 years of age have over 50% of tracheal obstruction.