Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease(206 views visite) Imbriaco M, Pellegrino T, Piscopo V, Petretta M, Ponsiglione A, Nappi C, Puglia M, Dell'Aversana S, Riccio E, Spinelli L, Pisani A, Cuocolo A
Keywords Parole chiave: Cardiac Magnetic Resonance Imaging, Fabry Disease, Fibrosis, Radionuclide Imaging, Sympathetic Nervous System,
Affiliations Affiliazioni: *** IBB - CNR ***
Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy., Institute of Biostructure and Bioimaging, National Council of Research, Naples, Italy., Department of Translational Medical Sciences, University Federico II, Naples, Italy., Department of Public Health, University of Naples Federico II, Naples, Italy., Department of Advanced Biomedical Sciences, University Federico II, Naples, Italy. cuocolo@unina.it.,
References Riferimenti: Not available. Non disponibili.
Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
PURPOSE: Cardiac sympathetic denervation may be detectable in patients with Anderson-Fabry disease (AFD), suggesting its usefulness for early detection of the disease. However, the relationship between sympathetic neuronal damage measured by 123I-metaiodobenzylguanidine (MIBG) imaging with myocardial fibrosis on cardiac magnetic resonance (CMR) is still unclear. METHODS: Cardiac sympathetic innervation was assessed by 123I-MIBG single-photon emission computed tomography (SPECT) in 25 patients with genetically proved AFD. Within one month from MIBG imaging, all patients underwent contrast-enhanced CMR. MIBG defect size and fibrosis size on CMR were measured for the left ventricle (LV) and expressed as %LV. RESULTS: Patients were divided into three groups according to MIBG and CMR findings: (1) matched normal, without MIBG defects and without fibrosis on CMR (n = 10); (2) unmatched, with MIBG defect but without fibrosis (n = 5); and (3) matched abnormal, with MIBG defect and fibrosis (n = 10). The three groups did not differ with respect to age, gender, alpha-galactosidase, proteinuria, glomerular filtration rate, and troponin I, while New York Heart Association class (p = 0.008), LV hypertrophy (p = 0.05), and enzyme replacement therapy (p = 0.02) were different among groups. Although in patients with matched abnormal findings, there was a significant correlation between MIBG defect size and area of fibrosis at CMR (r2 = 0.98, p < 0.001), MIBG defect size was larger than fibrosis size (26 +/- 23 vs. 18 +/- 13%LV, p = 0.02). CONCLUSION: Sympathetic neuronal damage is frequent in AFD patients, and it may precede myocardial damage, such as fibrosis. Thus, 123I-MIBG imaging can be considered a challenging technique for early detection of cardiac involvement in AFD.
Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
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Cardiac sympathetic neuronal damage precedes myocardial fibrosis in patients with Anderson-Fabry disease
Ciccarelli M, Sorriento D, Coscioni E, Iaccarino G, Santulli G * Adrenergic Receptors(194 visite) Endocrinol Of The Heart In Health And Dis (ISSN: 9780-1280311249780128031117), 2016; N/D: 285-315. Impact Factor:0 DettagliEsporta in BibTeXEsporta in EndNote
Testino G, Leone S, Fagoonee S, Del Bas JM, Rodriguez B, Puiggros F, Marine S, Rodriguez MA, Morina D, Armengol L, Caimari A, Arola L, Cimini FA, Barchetta I, Carotti S, Bertoccini L, Baroni MG, Vespasiani-gentilucci U, Cavallo MG, Morini S, Nelson JE, Roth CL, Wilson LA, Yates KP, Aouizerat B, Morgan-stevenson V, Whalen E, Hoofnagle A, Mason M, Gersuk V, Yeh MM, Kowdley KV, Lee SM, Jun DW, Cho YK, Jang KS, Kucukazman M, Ata N, Dal K, Yeniova AO, Kefeli A, Basyigit S, Aktas B, Akin KO, Agladioglu K, Ure OS, Topal F, Nazligul Y, Beyan E, Ertugrul DT, Catena C, Cosma C, Camozzi V, Plebani M, Ermani M, Sechi LA, Fallo F, Goto Y, Ray MB, Mendenhall CL, French SW, Gartside PS Serum vitamin A deficiency and increased intrahepatic expression of cytokeratin antigen in alcoholic liver disease(433 visite) Hepatology (ISSN: 1827-1669electronic, 0026-4806linking), 1988 Sep; 83120693611123109(5): 1019-1026. Impact Factor:0.913 DettagliEsporta in BibTeXEsporta in EndNote
129 Records (113 escludendo Abstract e Conferenze). Impact factor totale: 412.896 (364.53 escludendo Abstract e Conferenze). Impact factor a 5 anni totale: 432.484 (376.262 escludendo Abstract e Conferenze).
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