Skip navigation
  • 中文
  • English

DSpace CRIS

  • DSpace logo
  • Home
  • Organizations
  • Researchers
  • Research Outputs
  • Explore by
    • Organizations
    • Researchers
    • Research Outputs
  • Academic & Publications
  • Sign in
  • 中文
  • English
  1. NTU Scholars
  2. 醫學院
  3. 醫學系
Please use this identifier to cite or link to this item: https://scholars.lib.ntu.edu.tw/handle/123456789/525845
DC FieldValueLanguage
dc.contributor.authorLin, Hsiang-Yuen_US
dc.contributor.authorChuang, Chih-Kuangen_US
dc.contributor.authorChen, Ming-Renen_US
dc.contributor.authorLin, Shan-Miaoen_US
dc.contributor.authorHung, Chung-Liehen_US
dc.contributor.authorChang, Chia-Yingen_US
dc.contributor.authorChiu, Pao Chinen_US
dc.contributor.authorTsai, Wen-Huien_US
dc.contributor.authorNiu, Dau-Mingen_US
dc.contributor.authorTsai, Fuu-Jenen_US
dc.contributor.authorLin, Shio Jeanen_US
dc.contributor.authorWUH-LIANG HWUen_US
dc.contributor.authorLin, Ju-Lien_US
dc.contributor.authorSHAU-PING LINen_US
dc.creatorLin H.-Y.;Chuang C.-K.;Chen M.-R.;Lin S.-M.;Hung C.-L.;Chang C.-Y.;Chiu P.C.;Tsai W.-H.;Niu D.-M.;Tsai F.-J.;Lin S.J.;Wuh-Liang Hwu;Lin J.-L.;Lin S.-P.-
dc.date.accessioned2020-12-16T02:25:20Z-
dc.date.available2020-12-16T02:25:20Z-
dc.date.issued2016-
dc.identifier.issn1096-7192-
dc.identifier.urihttps://www.scopus.com/inward/record.uri?eid=2-s2.0-84958580864&doi=10.1016%2fj.ymgme.2016.02.003&partnerID=40&md5=45f46b94475abd159dca066d5b6a9a26-
dc.identifier.urihttps://scholars.lib.ntu.edu.tw/handle/123456789/525845-
dc.description.abstractBackground While enzyme replacement therapy (ERT) has been shown to improve endurance and joint mobility for patients with mucopolysaccharidoses (MPS) I, II, IVA and VI, the impact of ERT on cardiac abnormalities remains uncertain. Methods Medical records and echocardiograms of 28 Taiwanese MPS patients (9 with MPS I, 7 with MPS II, 7 with MPS IVA, and 5 with MPS VI) treated with ERT for 1–10.8?years were retrospectively reviewed. Results At start of ERT, z scores?>?2 were identified in 46% and 75% for left ventricular mass index (LVMI) and interventricular septum thickness in diastole (IVSd) in these patients, respectively. Twenty-four patients (86%) had valvular heart disease. After ERT, the mean IVSd z score of all patients decreased significantly from 3.87 to 2.57 (p?=?0.016). For 11 patients starting ERT before 12?years of age, z scores for both LVMI and IVSd decreased significantly (p?<?0.01) after ERT. However, the condition of valve regurgitation or stenosis did not show improvement despite ERT. Conclusions ERT was shown to be an effective therapy for reducing cardiac hypertrophy, with best results seen when ERT was started at an early age. ERT, however, had little impact on valvular heart disease. ? 2016 Elsevier Inc.-
dc.publisherAcademic Press Inc.-
dc.relation.ispartofMolecular Genetics and Metabolism-
dc.subject.otherelosulfase alfa; galsulfase; iduronate 2 sulfatase; laronidase; adolescent; adult; aorta stenosis; aorta valve regurgitation; Article; bi atrial enlargement; cardiac structure; cardiomegaly; cardiovascular parameters; child; clinical article; diastolic dysfunction; ECG abnormality; enzyme replacement; female; first degree atrioventricular block; heart; heart atrium enlargement; heart murmur; heart right ventricle hypertrophy; heart ventricle extrasystole; heart ventricle hypertrophy; human; infant; interventricular septum thickness in diastole; left ventricular mass index; male; Maroteaux Lamy syndrome; mitral valve prolapse; mitral valve regurgitation; mitral valve stenosis; mucopolysaccharide i; mucopolysaccharide i; mucopolysaccharide ii; mucopolysaccharide ii; mucopolysaccharide iva; mucopolysaccharide iva; mucopolysaccharidosis; priority journal; retrospective study; Taiwanese; valvular heart disease; cardiac muscle; complication; echocardiography; electrocardiography; follow up; heart; Heart Diseases; Mucopolysaccharidoses; pathology; pathophysiology; preschool child; treatment outcome; young adult; Adolescent; Adult; Child; Child, Preschool; Echocardiography; Electrocardiography; Enzyme Replacement Therapy; Female; Follow-Up Studies; Heart; Heart Diseases; Humans; Infant; Male; Mucopolysaccharidoses; Myocardium; Retrospective Studies; Treatment Outcome; Young Adult-
dc.subject.other[SDGs]SDG3-
dc.titleCardiac structure and function and effects of enzyme replacement therapy in patients with mucopolysaccharidoses I, II, IVA and VIen_US
dc.typejournal article-
dc.identifier.doi10.1016/j.ymgme.2016.02.003-
dc.identifier.pmid26899310-
dc.identifier.scopus2-s2.0-84958580864-
dc.relation.pages431-437-
dc.relation.journalvolume117-
dc.relation.journalissue4-
item.cerifentitytypePublications-
item.openairetypejournal article-
item.openairecristypehttp://purl.org/coar/resource_type/c_6501-
item.fulltextno fulltext-
item.grantfulltextnone-
crisitem.author.deptPediatrics-
crisitem.author.deptMedical Genetics-NTUH-
crisitem.author.deptPediatrics-NTUH-
crisitem.author.deptBiotechnology-
crisitem.author.deptGenome and Systems Biology Degree Program-
crisitem.author.orcid0000-0001-6690-4879-
crisitem.author.orcid0000-0003-3423-991X-
crisitem.author.parentorgCollege of Medicine-
crisitem.author.parentorgNational Taiwan University Hospital-
crisitem.author.parentorgNational Taiwan University Hospital-
crisitem.author.parentorgCollege of Bioresources and Agriculture-
crisitem.author.parentorgCollege of Life Science-
Appears in Collections:醫學系
Show simple item record

SCOPUSTM   
Citations

28
checked on May 24, 2023

WEB OF SCIENCETM
Citations

28
checked on May 19, 2023

Page view(s)

16
checked on May 10, 2023

Google ScholarTM

Check

Altmetric

Altmetric

Related Items in TAIR


Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

臺大位居世界頂尖大學之列,為永久珍藏及向國際展現本校豐碩的研究成果及學術能量,圖書館整合機構典藏(NTUR)與學術庫(AH)不同功能平台,成為臺大學術典藏NTU scholars。期能整合研究能量、促進交流合作、保存學術產出、推廣研究成果。

To permanently archive and promote researcher profiles and scholarly works, Library integrates the services of “NTU Repository” with “Academic Hub” to form NTU Scholars.

總館學科館員 (Main Library)
醫學圖書館學科館員 (Medical Library)
社會科學院辜振甫紀念圖書館學科館員 (Social Sciences Library)

開放取用是從使用者角度提升資訊取用性的社會運動,應用在學術研究上是透過將研究著作公開供使用者自由取閱,以促進學術傳播及因應期刊訂購費用逐年攀升。同時可加速研究發展、提升研究影響力,NTU Scholars即為本校的開放取用典藏(OA Archive)平台。(點選深入了解OA)

  • 請確認所上傳的全文是原創的內容,若該文件包含部分內容的版權非匯入者所有,或由第三方贊助與合作完成,請確認該版權所有者及第三方同意提供此授權。
    Please represent that the submission is your original work, and that you have the right to grant the rights to upload.
  • 若欲上傳已出版的全文電子檔,可使用Sherpa Romeo網站查詢,以確認出版單位之版權政策。
    Please use Sherpa Romeo to find a summary of permissions that are normally given as part of each publisher's copyright transfer agreement.
  • 網站簡介 (Quickstart Guide)
  • 使用手冊 (Instruction Manual)
  • 線上預約服務 (Booking Service)
  • 方案一:臺灣大學計算機中心帳號登入
    (With C&INC Email Account)
  • 方案二:ORCID帳號登入 (With ORCID)
  • 方案一:定期更新ORCID者,以ID匯入 (Search for identifier (ORCID))
  • 方案二:自行建檔 (Default mode Submission)
  • 方案三:學科館員協助匯入 (Email worklist to subject librarians)
Build with DSpace-CRIS - Extension maintained and optimized by Logo 4SCIENCE Feedback