There is an epidemic of chronic kidney disease (CKD) clustering in rural communities, predominantly in a number of low- and middle-income countries.1 Tens of thousands of working-aged adults are estimated to have died from the disease in Central America2 with similar numbers in Sri Lanka.3 Similar diseases have been reported elsewhere, such as rural regions or communities in India and North and West Africa. Those affected do not have common risk factors or underlying conditions that lead to CKD, such as diabetes, immune-mediated glomerulonephritis, or structural renal disease. In instances where histopathology is available, the predominant feature is tubular atrophy and interstitial fibrosis. Although it is currently unclear whether there is a unified underlying cause, these conditions have been collectively termed CKD of unknown cause (CKDu). Other terms used include “CKD of nontraditional cause,” “Mesoamerican nephropathy,” “chronic intestinal nephritis in agricultural communities,” and “kidney disease of unknown cause in agricultural laborers,” but we have chosen CKDu as the most agnostic terminology.
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