title: Fabry's disease: an example of cardiorenal syndrome type 5.
abstract: 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).
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology, St. Bortolo Hospital, 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology and Renal Transplant Medicine, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi, 110076, India.
authors.forename: Aashish
authors.initials: A
authors.lastname: Sharma
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology, St. Bortolo Hospital, 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Molecular Medicine, University of Padua, 63, via Gabelli, 35131, Padua, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: Sartori
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology, St. Bortolo Hospital, 37, Viale Rodolfi, 36100, Vicenza, Italy.
authors.forename: Jose J
authors.initials: JJ
authors.lastname: Zaragoza
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology, St. Bortolo Hospital, 37, Viale Rodolfi, 36100, Vicenza, Italy.
authors.forename: Gianluca
authors.initials: G
authors.lastname: Villa
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology, St. Bortolo Hospital, 37, Viale Rodolfi, 36100, Vicenza, Italy.
authors.forename: Renhua
authors.initials: R
authors.lastname: Lu
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology, St. Bortolo Hospital, 37, Viale Rodolfi, 36100, Vicenza, Italy.
authors.forename: Elena
authors.initials: E
authors.lastname: Faggiana
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology and Renal Transplant Medicine, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi, 110076, India.|Department of Medicine (DIMED), University of Padua, Padua, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Brocca
authors.affiliation: Department of Nephrology and Dialysis, L. Parodi - Delfino Hospital, Piazza A. Moro, 1, 00034, Colleferro, Roma, Italy. dilulloluca69@gmail.com.
authors.forename: Luca
authors.initials: L
authors.lastname: Di Lullo
authors.affiliation: Department of Nephrology and Dialysis, Ospedale Belcolle, Viterbo, Italy.
authors.forename: Sandro
authors.initials: S
authors.lastname: Feriozzi
authors.affiliation: International Renal Research Institute of Vicenza (IRRIV), 37, Viale Rodolfi, 36100, Vicenza, Italy.|Department of Nephrology, St. Bortolo Hospital, 37, Viale Rodolfi, 36100, Vicenza, Italy.
authors.forename: Claudio
authors.initials: C
authors.lastname: Ronco
mesh_terms: D000806:Angiotensin-Converting Enzyme Inhibitors / Q000627:therapeutic use; D059347:Cardio-Renal Syndrome / Q000503:physiopathology*; D056947:Enzyme Replacement Therapy; D000795:Fabry Disease / Q000188:drug therapy / Q000503:physiopathology*; D006321:Heart / Q000503:physiopathology*; D006801:Humans; D007668:Kidney / Q000503:physiopathology*
chemical_list: D000806:Angiotensin-Converting Enzyme Inhibitors
keywords: Cardiac disease; Cardiorenal syndrome 5; Fabry’s disease; Kidney disease; Organ cross talk