{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,28]],"date-time":"2026-01-28T13:16:38Z","timestamp":1769606198120,"version":"3.49.0"},"reference-count":0,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[2004,7,13]],"date-time":"2004-07-13T00:00:00Z","timestamp":1089676800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[2005,6]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Fabry disease (McKusick 301500) is an X\u2010linked lysosomal storage disorder due to deficient \u03b1\u2010galactosidase A activity, which leads to accumulation of glycosphingolipids, especially in vascular smooth\u2010muscle and endothelial cells. The effect of this accumulation on peripheral and cardiac vascular function is poorly known. We studied 15 Fabry patients (mean age 35 years and mean BMI 24.8 kg\/m2) and 30 age\u2010 and BMI\u2010matched healthy controls to examine whether myocardial perfusion reserve and peripheral artery endothelial function are altered. Myocardial perfusion was measured at rest and during dipyridamole\u2010induced hyperaemia by positron emission tomography and H<jats:sub>2<\/jats:sub><jats:sup>15<\/jats:sup>O. Myocardial blood flow reserve was calculated as the ratio between the dipyridamole\u2010induced maximal blood flow and resting blood flow. Peripheral artery endothelial function was assessed by measuring the brachial artery flow\u2010mediated dilatation using ultrasound at rest and during reactive hyperaemia. The myocardial perfusion reserve was significantly lower in Fabry patients than in controls (3.3 \u00b1 1.2 vs 4.4 \u00b1 1.6, <jats:italic>p<\/jats:italic> = 0.02), while the brachial artery flow\u2010mediated dilatation was similar (5.9% \u00b1 3.9%vs 4.5% \u00b1 3.6%, <jats:italic>p<\/jats:italic> = 0.27). Thus, inFabry disease, myocardial perfusion reserve is reduced while the peripheral artery endothelial function is preserved.<\/jats:p>","DOI":"10.1007\/s10545-005-0563-2","type":"journal-article","created":{"date-parts":[[2005,5,17]],"date-time":"2005-05-17T12:50:32Z","timestamp":1116334232000},"page":"563-573","source":"Crossref","is-referenced-by-count":50,"title":["Impaired myocardial perfusion reserve but preserved peripheral endothelial function in patients with Fabry disease"],"prefix":"10.1002","volume":"28","author":[{"given":"R. J.","family":"Kalliokoski","sequence":"first","affiliation":[{"name":"Turku PET Centre University of Turku  Turku"},{"name":"Turku PET Centre  PO Box 52 Turku FIN\u201020521 Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"K. K.","family":"Kalliokoski","sequence":"additional","affiliation":[{"name":"Turku PET Centre University of Turku  Turku"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Sundell","sequence":"additional","affiliation":[{"name":"Turku PET Centre University of Turku  Turku"},{"name":"Department of Medicine University of Turku  Turku"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"E.","family":"Engblom","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Turku  Turku"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M.","family":"Penttinen","sequence":"additional","affiliation":[{"name":"Clinical Genetics Unit, Department of Pediatrics Turku University Central Hospital  Turku Finland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"I.","family":"Kantola","sequence":"additional","affiliation":[{"name":"Department of Medicine University of Turku  Turku"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"O. T.","family":"Raitakari","sequence":"additional","affiliation":[{"name":"Turku PET Centre University of Turku  Turku"},{"name":"Department of Clinical Physiology University of Turku  Turku"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"J.","family":"Knuuti","sequence":"additional","affiliation":[{"name":"Turku PET Centre University of Turku  Turku"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P.","family":"Nuutila","sequence":"additional","affiliation":[{"name":"Turku PET Centre University of Turku  Turku"},{"name":"Department of Medicine University of Turku  Turku"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2004,7,13]]},"container-title":["Journal of Inherited Metabolic Disease"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-005-0563-2","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1007\/s10545-005-0563-2","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,9,15]],"date-time":"2023-09-15T00:22:25Z","timestamp":1694737345000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1007\/s10545-005-0563-2"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2004,7,13]]},"references-count":0,"journal-issue":{"issue":"4","published-print":{"date-parts":[[2005,6]]}},"alternative-id":["10.1007\/s10545-005-0563-2"],"URL":"https:\/\/doi.org\/10.1007\/s10545-005-0563-2","archive":["Portico"],"relation":{},"ISSN":["0141-8955","1573-2665"],"issn-type":[{"value":"0141-8955","type":"print"},{"value":"1573-2665","type":"electronic"}],"subject":[],"published":{"date-parts":[[2004,7,13]]}}}