%0 Journal Article
%T Trends in the Management and Outcomes of Kidney Transplantation for Autosomal Dominant Polycystic Kidney Disease
%A Madhukar S. Patel
%A Praveen Kandula
%A David Wojciechowski
%A James F. Markmann
%A Parsia A. Vagefi
%J Journal of Transplantation
%D 2014
%I Hindawi Publishing Corporation
%R 10.1155/2014/675697
%X Background. Autosomal dominant polycystic kidney disease (ADPKD) is the most common genetic disorder leading to end-stage renal failure. The objective of this study was to evaluate a longitudinal experience of kidney transplantation for ADPKD. Methods. A single center retrospective review of patients undergoing kidney transplantation was conducted, with comparisons across two time periods: early (02/2000¨C04/2007, ) and late (04/2007¨C08/2012, ). Results. Over the 13.5-year study period, 133 patients underwent transplantation for ADPKD. Overall, no significant difference between the early and late group with regard to intraoperative complications, need for reoperation, readmissions within 30 days, delayed graft function, and mortality was noted. There was a trend towards increase in one-year graft survival (early 93.1% versus late 100%, ). In the early group, 67% of recipients had undergone aneurysm screening, compared to 91% of recipients in the late group ( ). Conclusions. This study demonstrates consistent clinical care with a trend towards improved rates of one-year graft survival. Interestingly, we also note a significantly higher use of cerebral imaging over time, with the majority that were detected requiring surgical intervention which may justify the current practice of nonselective radiological screening until improved screening criteria are developed. 1. Introduction Autosomal dominant polycystic kidney disease (ADPKD) is a disorder characterized by the development of renal cysts that may result in end-stage renal failure. Two main genes, PKD1 and PKD2, are thought to be involved in the majority of cases [1]. Variability in the genetic phenotype of ADPKD patients, however, is thought to be due to the range of different genetic mechanisms as well as environmental factors thought to play a role in phenotypic expression [1]. In addition to affecting the kidneys, ADPKD has a number of extrarenal manifestations including cystic formation in other organs such as the liver, seminal vesicles, pancreas, and arachnoid membrane; vascular malformations such as intracranial aneurysms, thoracic aorta dissections, and coronary artery aneurysms; cardiac manifestations such as mitral valve prolapse; and a higher incidence of colonic diverticulosis and diverticulitis [1]. In those with end-stage renal disease, it is accepted that transplantation is the preferred treatment for ADPKD [1]. Of the patients on the kidney transplant waiting list as of December 31 2011, 7256 (8.4%) were listed due to cystic kidney disease and of the 16,055 renal transplants performed
%U http://www.hindawi.com/journals/jtrans/2014/675697/