Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study (766 views)
Heart (ISSN: 1355-6037, 1468-201x), 2009 Jul; 95(13): 1103-1107.
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Paper type: Letter, Journal Article,
Impact factor: 5.385, 5-year impact factor: 4.838
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-67649583702&partnerID=40&md5=a963c7aeaff474a0a34efb4bfa2c9cbd
Keywords: Acetylsalicylic Acid, Agalsidase Beta, Antilipemic Agent, Calcium Antagonist, Dipeptidyl Carboxypeptidase Inhibitor, Erythropoietin, Adult, Anderson Fabry Disease, Article, Clinical Article, Controlled Study, Disorders Of Lipid And Lipoprotein Metabolism, Drug Tolerability, Enzyme Replacement, Female, Heart Left Ventricle Function, Heart Left Ventricle Mass, Heart Left Ventricle Volume, Heart Ventricle Septum, Human, Long Term Care, Physical Examination, Priority Journal, Relaxation Time, Treatment Duration, X Chromosome Linked Disorder, Alpha-Galactosidase, Drug Administration Schedule, Hypertrophy, Left Ventricular, Isoenzymes, Magnetic Resonance Imaging, Middle Aged, Prospective Studies, Stroke Volume, Ventricular Function, Young Adult, Fluorodeoxyglucose F 18, Body Posture, Breast Cancer, Cancer Diagnosis, Computer Assisted Emission Tomography, Follow Up, Intermethod Comparison, Letter, Nuclear Magnetic Resonance Imaging,
Affiliations:
*** IBB - CNR ***
Department of Biomorphological and Functional Sciences, University Federico II, Naples, Italy
Department of Nephrology, University Federico II, Naples, Italy
Department of Clinical Medicine, Cardiovascular and Immunological Sciences, University Federico II, Naples, Italy
Department of National Research Council (IBB-CNR), University Federico II, Naples, Italy
Via Posillipo 196, 80123, Naples, Italy
References:
Not available.
Heart (ISSN: 1355-6037, 1468-201x), 2009 Jul; 95(13): 1103-1107.
Export to BibTeX Export to EndNote
Paper type: Letter, Journal Article,
Impact factor: 5.385, 5-year impact factor: 4.838
Url: http://www.scopus.com/inward/record.url?eid=2-s2.0-67649583702&partnerID=40&md5=a963c7aeaff474a0a34efb4bfa2c9cbd
Keywords: Acetylsalicylic Acid, Agalsidase Beta, Antilipemic Agent, Calcium Antagonist, Dipeptidyl Carboxypeptidase Inhibitor, Erythropoietin, Adult, Anderson Fabry Disease, Article, Clinical Article, Controlled Study, Disorders Of Lipid And Lipoprotein Metabolism, Drug Tolerability, Enzyme Replacement, Female, Heart Left Ventricle Function, Heart Left Ventricle Mass, Heart Left Ventricle Volume, Heart Ventricle Septum, Human, Long Term Care, Physical Examination, Priority Journal, Relaxation Time, Treatment Duration, X Chromosome Linked Disorder, Alpha-Galactosidase, Drug Administration Schedule, Hypertrophy, Left Ventricular, Isoenzymes, Magnetic Resonance Imaging, Middle Aged, Prospective Studies, Stroke Volume, Ventricular Function, Young Adult, Fluorodeoxyglucose F 18, Body Posture, Breast Cancer, Cancer Diagnosis, Computer Assisted Emission Tomography, Follow Up, Intermethod Comparison, Letter, Nuclear Magnetic Resonance Imaging,
Affiliations:
*** IBB - CNR ***
Department of Biomorphological and Functional Sciences, University Federico II, Naples, Italy
Department of Nephrology, University Federico II, Naples, Italy
Department of Clinical Medicine, Cardiovascular and Immunological Sciences, University Federico II, Naples, Italy
Department of National Research Council (IBB-CNR), University Federico II, Naples, Italy
Via Posillipo 196, 80123, Naples, Italy
References:
Not available.
Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study
Background: Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. Objective: This study aimed to assess the long-term effects of ERT with recombinant alpha-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson Fabry disease. Patients: Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). Results: At 45 months of treatment, LV mass and LV wall thickness had significantly reduced: 188 (SD 60) g versus 153 (47) g, and 16 (4) mm versus 14 (4) mm, respectively. Furthermore, a significant reduction in myocardial T2 relaxation times was noted in all myocardial regions, that is, interventricular septum 80 (5) ms versus 66 (8) ms, apex 79 (10) ms versus 64 (10) ms, and lateral wall 80 (8) ms versus 65 (16) ms. Changes in LV ejection fraction were not significant. Amelioration of clinical symptoms was observed in all patients. Conclusions: Long-term therapy with agalsidase beta at 1 mg/kg every 2 weeks was effective in significantly reducing LV hypertrophy, improving overall cardiac performance and ameliorating clinical symptoms in patients with Anderson-Fabry disease.
Background: Anderson-Fabry disease is a multisystem X linked disorder of lipid metabolism frequently associated with cardiac symptoms, including left ventricular (LV) hypertrophy gradually impairing cardiac function. Evidence showing that enzyme-replacement therapy (ERT) can be effective in reducing LV hypertrophy and improving myocardial function in the long term is limited. Objective: This study aimed to assess the long-term effects of ERT with recombinant alpha-galactosidase A (agalsidase beta, Fabrazyme) on LV function and myocardial signal intensity in 11 patients with Anderson Fabry disease. Patients: Eleven patients (eight males, three females) with varying stages of genetically confirmed Anderson Fabry disease were examined by means of physical examination and magnetic resonance imaging before ERT with agalsidase beta at 1 mg/kg every other week (study 1) and after a mean treatment duration of 45 months (study 2). Results: At 45 months of treatment, LV mass and LV wall thickness had significantly reduced: 188 (SD 60) g versus 153 (47) g, and 16 (4) mm versus 14 (4) mm, respectively. Furthermore, a significant reduction in myocardial T2 relaxation times was noted in all myocardial regions, that is, interventricular septum 80 (5) ms versus 66 (8) ms, apex 79 (10) ms versus 64 (10) ms, and lateral wall 80 (8) ms versus 65 (16) ms. Changes in LV ejection fraction were not significant. Amelioration of clinical symptoms was observed in all patients. Conclusions: Long-term therapy with agalsidase beta at 1 mg/kg every 2 weeks was effective in significantly reducing LV hypertrophy, improving overall cardiac performance and ameliorating clinical symptoms in patients with Anderson-Fabry disease.
Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study
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Effects of enzyme-replacement therapy in patients with Anderson-Fabry disease: a prospective long-term cardiac magnetic resonance imaging study
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Petretta M, Cuocolo A
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Letter by Petretta and Cuocolo regarding article, "Four-variable risk model in men and women with heart failure" (417 views)
Circ-Heart Fail (ISSN: 1941-3289), 2014 Mar 1; 7(2): 380-380.
Impact Factor: 5.867
View Export to BibTeX Export to EndNote
Andreotti G, Citro V, Correra A, Cubellis MV
* A thermodynamic assay to test pharmacological chaperones for Fabry disease (675 views)
Bba-Gen Subjects (ISSN: 0925-4439, 0006-3002, 1570-9639), 2014 Mar; 1840(3): 1214-1224.
Impact Factor: 4.882
View Export to BibTeX Export to EndNote
Paolillo S, Rengo G, Pagano G, Pellegrino T, Savarese G, Femminella GD, Tuccillo M, Boemio A, Attena E, Formisano R, Petraglia L, Scopacasa F, Galasso G, Leosco D, Trimarco B, Cuocolo A, Perrone-Filardi P
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Impact Factor: 8.57
View Export to BibTeX Export to EndNote
Berisio R
* Editorial: cell processes in bacterial diseases (504 views)
Protein Pept Lett (ISSN: 0929-8665, 1875-5305), 2012 Oct; 19(10): 1011-1012.
Impact Factor: 1.994
View Export to BibTeX Export to EndNote
De Simone G
* Editorial: Zinc Metallo-Enzymes As Target For Drug Design (578 views)
Curr Med Chem (ISSN: 0929-8673, 1875-533x, 1875-533xelectronic), 2012 Feb; 19(6): 820-1012.
Impact Factor: 4.07
View Export to BibTeX Export to EndNote
Messalli G, Imbriaco M, Avitabile G, Russo R, Iodice D, Spinelli L, Dellegrottaglie S, Cademartiri F, Salvatore M, Pisani A
* Role of cardiac MRI in evaluating patients with Anderson-Fabry disease: Assessing cardiac effects of long-term enzyme replacement therapy (424 views)
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Impact Factor: 1.461
View Export to BibTeX Export to EndNote
Perrone-Filardi P, Paolillo S, Dellegrottaglie S, Gargiulo P, Savarese G, Marciano C, Casaretti L, Cecere M, Musella F, Pirozzi E, Parente A, Cuocolo A
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Impact Factor: 4.223
View Export to BibTeX Export to EndNote
Manganelli F, Spadafora M, Varrella P, Peluso G, Sauro R, Di Lorenzo E, Rosato G, Daniele S, Cuocolo A
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Impact Factor: 4.991
View Export to BibTeX Export to EndNote
Imbriaco M, Messalli G, Avitabile G, Cuocolo A, Maurea S, Soscia F, Pisani A
* Cardiac magnetic resonance imaging illustrating Anderson-Fabry disease progression (411 views)
Br J Radiol (ISSN: 0007-1285, 1748-880xelectronic, 0007-1285linking), 2010 Dec; 83(996): e249-e249.
Impact Factor: 2.062
View Export to BibTeX Export to EndNote
Pedone C, Di Pasquale G, Greco C, Gonzini L, Maggioni AP, Pavesi PC, Sabini A, Boccanelli A; In-Acs Outcome Investigators
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Impact Factor: 0
View Export to BibTeX Export to EndNote
Storto G, Pellegrino T, Sorrentino AR, Luongo L, Petretta M, Cuocolo A
* Estimation of coronary flow reserve by sestamibi imaging in type 2 diabetic patients with normal coronary arteries (668 views)
J Nucl Cardiol (ISSN: 1532-6551, 1071-3581, 1532-6551electronic), 2007 Apr; 14(2): 194-199.
Impact Factor: 2.359
View Export to BibTeX Export to EndNote
Maestri R, Pinna G, Accardo A, Allegrini P, Balocchi R, D'ADDIO G, Ferrario M, Menicucci D, Porta A, Sassi R
* Nonlinear indices of heart rate variability in chronic heart failure patients: Redundancy and comparative clinical value (505 views)
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Impact Factor: 3.475
View Export to BibTeX Export to EndNote
Hesse B, Tagil K, Cuocolo A, Anagnostopoulos C, Bardies M, Bax J, Bengel F, Busemann Sokole E, Davies G, Dondi M, Edenbrandt L, Franken P, Kjaer A, Knuuti J, Lassmann M, Ljungberg M, Marcassa C, Marie PY, Mckiddie F, O'connor M, Prvuolovich E, Underwood R
* 3. 0 T perfusion MR imaging (1039 views)
Rivista Di Neuroradiologia (ISSN: 1120-9976), 2004; 17(6): 807-812.
Impact Factor: 0.023
View Export to BibTeX Export to EndNote
Pauciullo P, Giannino A, De Michele M, Gentile M, Liguori R, Argiriou A, Carlotto A, Faccenda F, Mancini M, Bond MG, De Simone V, Rubba P
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Impact Factor: 2.013
View Export to BibTeX Export to EndNote
Morisco C, Argenziano L, Sarno D, Cuocolo A, Romano M, Trimarco B
The practical aspects of treatment in heart failure (487 views)
Ann Ital Med Int (ISSN: 0393-9340), 1993 Oct; 8Suppl: 14S-19S.
Impact Factor: 0
View Export to BibTeX Export to EndNote
15 Records (14 excluding Abstracts).
Total impact factor: 45.99 (43.928 excluding Abstracts).
Total 5 year impact factor: 50.819 (48.464 excluding Abstracts).
Total impact factor: 45.99 (43.928 excluding Abstracts).
Total 5 year impact factor: 50.819 (48.464 excluding Abstracts).
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