Fabry's disease: an example of cardiorenal syndrome type 5
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| 024 | 7 | 0 | |a 10.1007/s10741-015-9500-0 |2 doi |
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| 245 | 0 | 0 | |a Fabry's disease: an example of cardiorenal syndrome type 5 |h [Elektronische Daten] |c [Aashish Sharma, Marco Sartori, Jose Zaragoza, Gianluca Villa, Renhua Lu, Elena Faggiana, Alessandra Brocca, Luca Di Lullo, Sandro Feriozzi, Claudio Ronco] |
| 520 | 3 | |a Cardiorenal syndrome type 5 (CRS-5) includes conditions where there is a simultaneous involvement of the heart and kidney from a systemic disorder. This is a bilateral organ cross talk. Fabry's disease (FD) is a devastating progressive inborn error of metabolism with lysosomal glycosphingolipid deposition in variety of cell types, capillary endothelial cells, renal, cardiac and nerve cells. Basic effect is absent or deficient activity of lysosomal exoglycohydrolase a-galactosidase A. Renal involvement consists of proteinuria, isosthenuria, altered tubular function, presenting in second or third decade leading to azotemia and end-stage renal disease in third to fifth decade mainly due to irreversible changes to glomerular, tubular and vascular structures, especially highlighted by podocytes foot process effacement. Cardiac involvement consists of left ventricular hypertrophy, right ventricular hypertrophy, arrhythmias (sinus node and conduction system impairment), diastolic dysfunction, myocardial ischemia, infarction, transmural replacement fibrosis, congestive heart failure and cardiac death. Management of FD is based on enzymatic replacement therapy and control of renal (with anti-proteinuric agents such as angiotensin-converting enzyme inhibitors—and/or angiotensin II receptor blockers), brain (coated aspirin, clopidogrel and statin to prevent strokes) and heart complications (calcium channel blockers for ischemic cardiomyopathy, warfarin and amiodarone or cardioverter device for arrhythmias). | |
| 540 | |a Springer Science+Business Media New York, 2015 | ||
| 690 | 7 | |a Organ cross talk |2 nationallicence | |
| 690 | 7 | |a Kidney disease |2 nationallicence | |
| 690 | 7 | |a Cardiac disease |2 nationallicence | |
| 690 | 7 | |a Fabry's disease |2 nationallicence | |
| 690 | 7 | |a Cardiorenal syndrome 5 |2 nationallicence | |
| 690 | 7 | |a ACEi : Angiotensin-converting enzyme inhibitor |2 nationallicence | |
| 690 | 7 | |a ARBs : Angiotensin receptor blockers |2 nationallicence | |
| 690 | 7 | |a BNP : Beta natriuretic peptide |2 nationallicence | |
| 690 | 7 | |a Bp : Base pairs |2 nationallicence | |
| 690 | 7 | |a Ca : Calcium |2 nationallicence | |
| 690 | 7 | |a CHF : Congestive heart failure |2 nationallicence | |
| 690 | 7 | |a CHO : Chinese hamster ovary |2 nationallicence | |
| 690 | 7 | |a CrCl : Creatinine clearance |2 nationallicence | |
| 690 | 7 | |a CRS : Cardiorenal syndrome |2 nationallicence | |
| 690 | 7 | |a CTH : Ceramide trihexoside |2 nationallicence | |
| 690 | 7 | |a DBS : Dried blood spots |2 nationallicence | |
| 690 | 7 | |a DHPLC : Denaturing high-performance liquid chromatography |2 nationallicence | |
| 690 | 7 | |a eGFR : Estimated glomerular filtration rate |2 nationallicence | |
| 690 | 7 | |a EM : Electron microscopy |2 nationallicence | |
| 690 | 7 | |a ERT : Enzyme replacement therapy |2 nationallicence | |
| 690 | 7 | |a FD : Fabry's disease |2 nationallicence | |
| 690 | 7 | |a Gb3 : Globotriaosylceramide |2 nationallicence | |
| 690 | 7 | |a GLA : Galactosidase alpha |2 nationallicence | |
| 690 | 7 | |a HR : Heart rate |2 nationallicence | |
| 690 | 7 | |a ICD : Intracardiac defibrillator |2 nationallicence | |
| 690 | 7 | |a IMT : Intimal-media thickening |2 nationallicence | |
| 690 | 7 | |a K : Potassium |2 nationallicence | |
| 690 | 7 | |a LPa : Lipoprotein a |2 nationallicence | |
| 690 | 7 | |a LVH : Left ventricular hypertrophy |2 nationallicence | |
| 690 | 7 | |a LVM : Left ventricle mass |2 nationallicence | |
| 690 | 7 | |a MLPA : Multiplex ligation-dependent probe amplification |2 nationallicence | |
| 690 | 7 | |a MRI : Magnetic resonance imaging |2 nationallicence | |
| 690 | 7 | |a NSAID : Nonsteroidal anti-inflammatory drugs |2 nationallicence | |
| 690 | 7 | |a RAAS : Renin-angiotensin-aldosterone system |2 nationallicence | |
| 690 | 7 | |a RV : Right ventricle |2 nationallicence | |
| 690 | 7 | |a RVH : Right ventricular hypertrophy |2 nationallicence | |
| 690 | 7 | |a S1P : Sphingosine-1-phosphate |2 nationallicence | |
| 690 | 7 | |a TGF-b1 : Transforming growth factor-beta 1 |2 nationallicence | |
| 700 | 1 | |a Sharma |D Aashish |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 700 | 1 | |a Sartori |D Marco |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 700 | 1 | |a Zaragoza |D Jose |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 700 | 1 | |a Villa |D Gianluca |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 700 | 1 | |a Lu |D Renhua |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 700 | 1 | |a Faggiana |D Elena |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 700 | 1 | |a Brocca |D Alessandra |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 700 | 1 | |a Di Lullo |D Luca |u Department of Nephrology and Dialysis, L. Parodi - Delfino Hospital, Piazza A. Moro, 1, 00034, Colleferro, Roma, Italy |4 aut | |
| 700 | 1 | |a Feriozzi |D Sandro |u Department of Nephrology and Dialysis, Ospedale Belcolle, Viterbo, Italy |4 aut | |
| 700 | 1 | |a Ronco |D Claudio |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | |
| 773 | 0 | |t Heart Failure Reviews |d Springer US; http://www.springer-ny.com |g 20/6(2015-11-01), 689-708 |x 1382-4147 |q 20:6<689 |1 2015 |2 20 |o 10741 | |
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| 950 | |B NATIONALLICENCE |P 856 |E 40 |u https://doi.org/10.1007/s10741-015-9500-0 |q text/html |z Onlinezugriff via DOI | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Sharma |D Aashish |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Sartori |D Marco |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Zaragoza |D Jose |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Villa |D Gianluca |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Lu |D Renhua |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Faggiana |D Elena |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Brocca |D Alessandra |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Di Lullo |D Luca |u Department of Nephrology and Dialysis, L. Parodi - Delfino Hospital, Piazza A. Moro, 1, 00034, Colleferro, Roma, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Feriozzi |D Sandro |u Department of Nephrology and Dialysis, Ospedale Belcolle, Viterbo, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 700 |E 1- |a Ronco |D Claudio |u International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy |4 aut | ||
| 950 | |B NATIONALLICENCE |P 773 |E 0- |t Heart Failure Reviews |d Springer US; http://www.springer-ny.com |g 20/6(2015-11-01), 689-708 |x 1382-4147 |q 20:6<689 |1 2015 |2 20 |o 10741 | ||