A priori clinical diagnosis of CKD is defined as pre-WES clinical diagnosis per referral by primary nephrologist. CAKUT, congenital anomalies of the kidney and urinary tract; CKD, chronic kidney disease; ESKD, end-stage kidney disease; GN, glomerulonephritis; SRNS, steroid-resistant nephrotic syndrome; TIKD, tubulointerstitial kidney disease; WES, whole exome sequencing. a Age at first presentation to medical services with evidence of CKD. b Age at start of renal replacement therapy, i.e., dialysis or kidney transplantation. DM Connaughton et al.: Monogenic causation of chronic kidney disease in Ireland c l i n i c a l i n v e s t i g a t i o n Kidney International (2019) 95, 914-928 DM Connaughton et al.: Monogenic causation of chronic kidney disease in Ireland c l i n i c a l i n v e s t i g a t i o n

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The many manifestations of atheroembolism are apparent in all specialties. However, with increasing intervention in older patients with atherosclerotic disease, it has become an important renal clinical problem. Atheroembolic disease is widely recognized as a cause of acute catastrophic renal dysfunction and recent important data have suggested that effective management protocols can improve outcome. It is probable that the clinical course is insidious in the majority of patients with severe atherosclerotic aortic disease. The management of these patients is less clear and at present rests on small anecdotal reports.