{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,13]],"date-time":"2026-02-13T17:03:05Z","timestamp":1771002185090,"version":"3.50.1"},"reference-count":39,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"6","content-domain":{"domain":["www.ahajournals.org"],"crossmark-restriction":true},"short-container-title":["JAHA"],"published-print":{"date-parts":[[2018,3,20]]},"abstract":"<jats:sec xml:lang=\"en\">\n            <jats:title>Background<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Cardiomyopathy is a major determinant of overall Fabry disease (\n              <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n              ) prognosis, with the worst outcomes in patients with myocardial fibrosis. Late gadolinium enhancement is currently the gold standard for evaluation of replacement myocardial fibrosis; however, this event is irreversible, thus identification of biomarkers of earlier diffuse fibrosis is paramount.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Methods and Results<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Type I collagen synthesis and degradation biomarkers (PICP [carboxyterminal propeptide of procollagen type I], ICTP [carboxyterminal telopeptide of type I collagen], and MMP1 [matrix metalloproteinase 1] and MMP2) and markers of bone synthesis and degradation were evaluated (to adjust type I collagen metabolism to bone turnover) in\n              <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n              patients and controls.\n              <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n              patients were grouped by cardiomyopathy severity, according to echocardiogram: (1) normal, (2) tissue Doppler abnormalities, (3) left ventricular hypertrophy. A significant increase in\n              <jats:styled-content style=\"fixed-case\">PICP<\/jats:styled-content>\n              and a significant decrease in matrix metalloproteinases were observed in\n              <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n              patients; even the group with normal echocardiogram had a significant increase in\n              <jats:styled-content style=\"fixed-case\">PICP<\/jats:styled-content>\n              . We also found a significant correlation between left ventricular mass and\n              <jats:styled-content style=\"fixed-case\">PICP<\/jats:styled-content>\n              (\u03c1=0.378,\n              <jats:italic>P<\/jats:italic>\n              =0.003) and\n              <jats:styled-content style=\"fixed-case\">MMP<\/jats:styled-content>\n              1 (\u03c1=\u22120.484,\n              <jats:italic>P<\/jats:italic>\n              &lt;0.001).\n              <jats:styled-content style=\"fixed-case\">PICP<\/jats:styled-content>\n              (adjusted for bone turnover) was the better predictor of left ventricular mass in multivariable regression, and its diagnostic accuracy to predict late gadolinium enhancement was also significant.\n            <\/jats:p>\n          <\/jats:sec>\n          <jats:sec xml:lang=\"en\">\n            <jats:title>Conclusions<\/jats:title>\n            <jats:p xml:lang=\"en\">\n              Collagen type I synthesis is increased in\n              <jats:styled-content style=\"fixed-case\">FD<\/jats:styled-content>\n              cardiomyopathy, even in the earlier stages of the disease, and this profibrotic state has good predictive value for and is likely to be critical to the development of overt left ventricular hypertrophy. Moreover, inhibition of enzymes involved in collagen type I cleavage also seems crucial to myocardial collagen deposition.\n            <\/jats:p>\n          <\/jats:sec>","DOI":"10.1161\/jaha.117.007124","type":"journal-article","created":{"date-parts":[[2018,3,14]],"date-time":"2018-03-14T00:10:30Z","timestamp":1520986230000},"update-policy":"https:\/\/doi.org\/10.1161\/crossmarkpolicy","source":"Crossref","is-referenced-by-count":20,"title":["Biomarkers of Myocardial Fibrosis: Revealing the Natural History of Fibrogenesis in Fabry Disease Cardiomyopathy"],"prefix":"10.1161","volume":"7","author":[{"given":"Patr\u00edcio","family":"Aguiar","sequence":"first","affiliation":[{"name":"Medicine 1 Department, Centro Hospitalar Lisboa Norte, Lisbon, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Olga","family":"Azevedo","sequence":"additional","affiliation":[{"name":"Cardiology Department, Reference Center on Lysosomal Storage Disorders, Hospital Senhora da Oliveira, Guimar\u00e3es, Portugal"},{"name":"Life and Health Sciences Research Institute (ICVS), School of Medicine, University of Minho, Braga, Portugal"},{"name":"ICVS\/3Bs PT Government Associate Laboratory, Braga\/Guimar\u00e3es, Portugal"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rui","family":"Pinto","sequence":"additional","affiliation":[{"name":"JCS. 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