{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,13]],"date-time":"2026-07-13T15:24:22Z","timestamp":1783956262033,"version":"3.55.0"},"reference-count":37,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2017,12,1]],"date-time":"2017-12-01T00:00:00Z","timestamp":1512086400000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"}],"content-domain":{"domain":["clinicalkey.jp","clinicalkey.com","internationaljournalofcardiology.com","clinicalkey.es","clinicalkey.fr","clinicalkey.com.au","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["International Journal of Cardiology"],"published-print":{"date-parts":[[2017,12]]},"DOI":"10.1016\/j.ijcard.2017.06.079","type":"journal-article","created":{"date-parts":[[2017,6,23]],"date-time":"2017-06-23T21:31:30Z","timestamp":1498253490000},"page":"257-262","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":23,"special_numbering":"C","title":["Evolution of cardiac pathology in classic Fabry disease: Progressive cardiomyocyte enlargement leads to increased cell death and fibrosis, and correlates with severity of ventricular hypertrophy\u202c\u202c\u202c\u202c\u202c\u202c\u202c\u202c"],"prefix":"10.1016","volume":"248","author":[{"given":"Andrea","family":"Frustaci","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cristina","family":"Chimenti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0301-0098","authenticated-orcid":false,"given":"Dana","family":"Doheny","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert J.","family":"Desnick","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ijcard.2017.06.079_bb0005","series-title":"The Online Metabolic and Molecular Bases of Inherited Diseases","first-page":"3733","article-title":"Alpha-galactosidase A deficiency: Fabry disease","author":"Desnick","year":"2001"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0010","series-title":"Genetic Diseases of the Kidney","first-page":"597","article-title":"Fabry's disease (\u03b1Galactosidase A deficiency): an X-linked nephropathy","author":"Desnick","year":"2009"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0015","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/1750-1172-5-30","article-title":"Fabry disease","volume":"5","author":"Germain","year":"2010","journal-title":"Orphanet J. Rare Dis."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0020","doi-asserted-by":"crossref","first-page":"2561","DOI":"10.1161\/CIRCULATIONAHA.108.841494","article-title":"Cardiac microvascular pathology in Fabry disease: evaluation of endomyocardial biopsies before and after enzyme replacement therapy","volume":"119","author":"Thurberg","year":"2009","journal-title":"Circulation"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0025","doi-asserted-by":"crossref","first-page":"663","DOI":"10.1038\/ki.2010.484","article-title":"Progressive podocyte injury and globotriasylceramide (GL-3) accumulation in young patients with Fabry disease","volume":"79","author":"Najafian","year":"2011","journal-title":"Kidney Int."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0030","doi-asserted-by":"crossref","first-page":"161","DOI":"10.1161\/CIRCHEARTFAILURE.108.769729","article-title":"Angina in Fabry disease reflects coronary small vessel disease","volume":"1","author":"Chimenti","year":"2008","journal-title":"Circ. Heart Fail."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0035","doi-asserted-by":"crossref","first-page":"395","DOI":"10.1056\/NEJM199102073240607","article-title":"An atypical variant of Fabry's disease with manifestations confined to the myocardium","volume":"324","author":"von Scheidt","year":"1991","journal-title":"N. Engl. J. Med."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0040","first-page":"449","article-title":"Fabry's disease with isolated disease of the cardiac muscle manifesting as hypertrophic cardiomyopathy","author":"Elleder","year":"1990","journal-title":"Cas. Lek. Cesk."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0045","doi-asserted-by":"crossref","first-page":"164","DOI":"10.1053\/j.ajkd.2003.09.022","article-title":"Fabry disease: renal involvement limited to podocyte pathology and proteinuria in a septuagenarian cardiac variant. Pathologic and therapeutic implications","volume":"43","author":"Meehan","year":"2004","journal-title":"Am. J. Kidney Dis."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0050","doi-asserted-by":"crossref","first-page":"217","DOI":"10.1136\/jmg.2009.072116","article-title":"Screening for Fabry disease in high-risk populations: a systematic review","volume":"47","author":"Linthorst","year":"2010","journal-title":"J. Med. Genet."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0055","doi-asserted-by":"crossref","first-page":"31","DOI":"10.1086\/504601","article-title":"High incidence of later-onset Fabry disease revealed by newborn screening","volume":"79","author":"Spada","year":"2006","journal-title":"Am. J. Hum. Genet."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0060","doi-asserted-by":"crossref","first-page":"818","DOI":"10.1161\/01.CIR.54.5.818","article-title":"Cardiac valvular anomalies in Fabry disease: clinical, morphologic and biochemical studies","volume":"54","author":"Desnick","year":"1976","journal-title":"Circulation"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0065","doi-asserted-by":"crossref","first-page":"699","DOI":"10.1016\/S0002-8703(77)80064-1","article-title":"Electrocardiographic and vector-cardiographic abnormalities in Fabry's disease","volume":"93","author":"Mehta","year":"1977","journal-title":"Am. Heart J."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0070","doi-asserted-by":"crossref","first-page":"1093","DOI":"10.1016\/j.jacc.2010.11.018","article-title":"Cardiovascular events in patients with Fabry disease natural history data from the Fabry registry","volume":"57","author":"Patel","year":"2011","journal-title":"J. Am. Coll. Cardiol."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0075","doi-asserted-by":"crossref","first-page":"2151","DOI":"10.1016\/j.ehj.2003.09.017","article-title":"Gadolinium enhanced cardiovascular magnetic resonance in Anderson-Fabry disease. Evidence for a disease specific abnormality of the myocardial interstitium","volume":"24","author":"Moon","year":"2003","journal-title":"Eur. Heart J."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0080","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1111\/cge.12613","article-title":"X chromosome inactivation in female patients with Fabry disease","volume":"89","author":"Echevarria","year":"2016","journal-title":"Clin. Genet."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0085","doi-asserted-by":"crossref","first-page":"249","DOI":"10.1046\/j.1523-1755.2002.00097.x","article-title":"Patients with Fabry disease on dialysis in the United States","volume":"61","author":"Thadhani","year":"2002","journal-title":"Kidney Int."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0090","doi-asserted-by":"crossref","first-page":"592","DOI":"10.1016\/j.jcmg.2011.01.020","article-title":"Differences in Fabry cardiomyopathy between female and male patients: consequences for diagnostic assessment","volume":"4","author":"Niemann","year":"2011","journal-title":"JACC Cardiovasc. Imaging"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0095","doi-asserted-by":"crossref","first-page":"2102","DOI":"10.1093\/ndt\/gfp031","article-title":"Fabry disease: progression of nephropathy, and prevalence of cardiac and cerebrovascular events before enzyme replacement therapy","volume":"24","author":"Schiffmann","year":"2009","journal-title":"Nephrol. Dial. Transplant."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0100","doi-asserted-by":"crossref","first-page":"112","DOI":"10.1016\/j.ymgme.2007.09.013","article-title":"Females with Fabry disease frequently have major organ involvement: lessons from the Fabry registry","volume":"93","author":"Wilcox","year":"2008","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0105","doi-asserted-by":"crossref","first-page":"788","DOI":"10.1161\/STROKEAHA.108.526293","article-title":"Stroke in Fabry disease frequently occurs before diagnosis and in the absence of other clinical events: natural history data from the Fabry registry","volume":"40","author":"Sims","year":"2009","journal-title":"Stroke"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0110","doi-asserted-by":"crossref","first-page":"873","DOI":"10.1007\/s10545-012-9540-8","article-title":"Crossectional baseline analysis of electrocardiography in a large cohort of patients with untreated Fabry disease","volume":"36","author":"Niemann","year":"2013","journal-title":"J. Inherit. Metab. Dis."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0115","first-page":"98","article-title":"End-stage cardiac manifestations and autopsy findings in patients with cardiac Fabry disease","volume":"43","author":"Teraguchi","year":"2004","journal-title":"J. Cardiol."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0120","doi-asserted-by":"crossref","first-page":"1397","DOI":"10.1002\/humu.21074","article-title":"Newborn screening for Fabry disease in Taiwan reveals a high incidence of the later-onset GLA mutation c.936_919G>A (IVS4+919G>A)","volume":"30","author":"Hwu","year":"2009","journal-title":"Hum. Mutat."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0125","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1186\/1479-7364-2-5-297","article-title":"Fabry disease: identification of 50 novel alpha-galactosidase A mutations causing the classic phenotype and three-dimensional structural analysis of 29 missense mutations","volume":"2","author":"Shabbeer","year":"2006","journal-title":"Hum. Genomics"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0130","doi-asserted-by":"crossref","first-page":"1978","DOI":"10.1161\/01.CIR.0000061952.27445.A0","article-title":"Early detection of Fabry cardiomyopathy by Tissue Doppler Imaging","volume":"107","author":"Pieroni","year":"2003","journal-title":"Circulation"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0135","doi-asserted-by":"crossref","first-page":"2414","DOI":"10.1016\/j.jacc.2008.03.018","article-title":"Late gadolinium enhancement by cardiovascular magnetic resonance heralds an adverse prognosis in nonischemic cardiomyopathy","volume":"51","author":"Wu","year":"2008","journal-title":"J. Am. Coll. Cardiol."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0140","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1161\/01.RES.0000093985.76901.AF","article-title":"Senescence and death of primitive cells and myocytes lead to premature cardiac aging and heart failure","volume":"93","author":"Chimenti","year":"2003","journal-title":"Circ. Res."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0145","doi-asserted-by":"crossref","first-page":"1482","DOI":"10.2353\/ajpath.2008.070576","article-title":"Myofilament degradation and dysfunction of human cardiomyocytes in Fabry disease","volume":"172","author":"Chimenti","year":"2008","journal-title":"Am. J. Pathol."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0150","doi-asserted-by":"crossref","first-page":"1444","DOI":"10.1016\/j.humpath.2011.09.020","article-title":"Cardiac and skeletal myopathy in Fabry disease: a clinicopathologic correlative study","volume":"43","author":"Chimenti","year":"2012","journal-title":"Hum. Pathol."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0155","doi-asserted-by":"crossref","first-page":"1530","DOI":"10.1161\/CIRCULATIONAHA.114.012178","article-title":"Microvascular angina as prehypertrophic presentation of Fabry disease cardiomyopathy","volume":"130","author":"Frustaci","year":"2014","journal-title":"Circulation"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0160","doi-asserted-by":"crossref","first-page":"350","DOI":"10.1161\/CIRCULATIONAHA.107.723387","article-title":"Cryptogenic ventricular arrhythmias and sudden death by Fabry disease: prominent infiltration of cardiac conduction tissue","volume":"116","author":"Frustaci","year":"2007","journal-title":"Circulation"},{"key":"10.1016\/j.ijcard.2017.06.079_bb0165","doi-asserted-by":"crossref","first-page":"823","DOI":"10.1007\/s00428-007-0468-6","article-title":"Cellular and tissue localization of globotriaosylceramide in Fabry disease","volume":"451","author":"Askari","year":"2007","journal-title":"Virchows Arch."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0170","doi-asserted-by":"crossref","first-page":"8-4","DOI":"10.1016\/j.carpath.2009.10.003","article-title":"The heart in Fabry's disease","volume":"20","author":"Sheppard","year":"2011","journal-title":"Cardiovasc. Pathol."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0175","doi-asserted-by":"crossref","first-page":"1760","DOI":"10.1016\/j.humpath.2015.07.017","article-title":"Increased oxidative stress contributes to cardiomyocyte dysfunction and death in patients with Fabry disease cardiomyopathy","volume":"46","author":"Chimenti","year":"2015","journal-title":"Hum. Pathol."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0180","doi-asserted-by":"crossref","first-page":"163","DOI":"10.1016\/j.ymgme.2008.06.016","article-title":"Globotriaosylceramide induces oxidative stress and up-regulates cell adhesion molecule expression in Fabry disease endothelial cells","volume":"95","author":"Shen","year":"2008","journal-title":"Mol. Genet. Metab."},{"key":"10.1016\/j.ijcard.2017.06.079_bb0185","doi-asserted-by":"crossref","first-page":"34","DOI":"10.1159\/000327075","article-title":"Quantitating glomerular endothelial fenestration: an unbiased stereological approach","volume":"1","author":"Najafian","year":"2011","journal-title":"Am. J. Nephrol."}],"container-title":["International Journal of Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0167527317329261?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S0167527317329261?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2018,8,31]],"date-time":"2018-08-31T08:50:51Z","timestamp":1535705451000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S0167527317329261"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2017,12]]},"references-count":37,"alternative-id":["S0167527317329261"],"URL":"https:\/\/doi.org\/10.1016\/j.ijcard.2017.06.079","relation":{},"ISSN":["0167-5273"],"issn-type":[{"value":"0167-5273","type":"print"}],"subject":[],"published":{"date-parts":[[2017,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Evolution of cardiac pathology in classic Fabry disease: Progressive cardiomyocyte enlargement leads to increased cell death and fibrosis, and correlates with severity of ventricular hypertrophy\u202c\u202c\u202c\u202c\u202c\u202c\u202c\u202c","name":"articletitle","label":"Article Title"},{"value":"International Journal of Cardiology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ijcard.2017.06.079","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2017 Elsevier B.V. All rights reserved.","name":"copyright","label":"Copyright"}]}}