https://scholars.lib.ntu.edu.tw/handle/123456789/525845
DC 欄位 | 值 | 語言 |
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dc.contributor.author | Lin, Hsiang-Yu | en_US |
dc.contributor.author | Chuang, Chih-Kuang | en_US |
dc.contributor.author | Chen, Ming-Ren | en_US |
dc.contributor.author | Lin, Shan-Miao | en_US |
dc.contributor.author | Hung, Chung-Lieh | en_US |
dc.contributor.author | Chang, Chia-Ying | en_US |
dc.contributor.author | Chiu, Pao Chin | en_US |
dc.contributor.author | Tsai, Wen-Hui | en_US |
dc.contributor.author | Niu, Dau-Ming | en_US |
dc.contributor.author | Tsai, Fuu-Jen | en_US |
dc.contributor.author | Lin, Shio Jean | en_US |
dc.contributor.author | WUH-LIANG HWU | en_US |
dc.contributor.author | Lin, Ju-Li | en_US |
dc.contributor.author | SHAU-PING LIN | en_US |
dc.creator | Lin 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.accessioned | 2020-12-16T02:25:20Z | - |
dc.date.available | 2020-12-16T02:25:20Z | - |
dc.date.issued | 2016 | - |
dc.identifier.issn | 1096-7192 | - |
dc.identifier.uri | https://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.uri | https://scholars.lib.ntu.edu.tw/handle/123456789/525845 | - |
dc.description.abstract | Background 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.publisher | Academic Press Inc. | - |
dc.relation.ispartof | Molecular Genetics and Metabolism | - |
dc.subject.classification | [SDGs]SDG3 | - |
dc.subject.other | elosulfase 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.title | Cardiac structure and function and effects of enzyme replacement therapy in patients with mucopolysaccharidoses I, II, IVA and VI | en_US |
dc.type | journal article | en |
dc.identifier.doi | 10.1016/j.ymgme.2016.02.003 | - |
dc.identifier.pmid | 26899310 | - |
dc.identifier.scopus | 2-s2.0-84958580864 | - |
dc.relation.pages | 431-437 | - |
dc.relation.journalvolume | 117 | - |
dc.relation.journalissue | 4 | - |
item.openairecristype | http://purl.org/coar/resource_type/c_6501 | - |
item.openairetype | journal article | - |
item.grantfulltext | none | - |
item.cerifentitytype | Publications | - |
item.fulltext | no fulltext | - |
crisitem.author.dept | Pediatrics | - |
crisitem.author.dept | Medical Genetics-NTUH | - |
crisitem.author.dept | Pediatrics-NTUH | - |
crisitem.author.dept | Biotechnology | - |
crisitem.author.dept | Genome and Systems Biology Degree Program | - |
crisitem.author.orcid | 0000-0001-6690-4879 | - |
crisitem.author.orcid | 0000-0003-3423-991X | - |
crisitem.author.parentorg | College of Medicine | - |
crisitem.author.parentorg | National Taiwan University Hospital | - |
crisitem.author.parentorg | National Taiwan University Hospital | - |
crisitem.author.parentorg | College of Bioresources and Agriculture | - |
crisitem.author.parentorg | College of Life Science | - |
顯示於: | 醫學系 |
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