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End-stage renal disease (ESRD) is a major public health problem, affecting 1 in 1,000 individuals and with an annual death rate of 20% despite dialysis treatment 1, 2. IgA nephropathy (IgAN) is the most common form of glomerulonephritis, a principal cause of ESRD worldwide 1, 2; it affects up to 1.3% of the population 3, 4, 5, 6 and its pathogenesis is unknown. Kidneys of people with IgAN show deposits of IgA-containing immune complexes with proliferation of the glomerular mesangium (Fig. 1). Typical clinical features include onset before age 40 with haematuria and proteinuria (blood and protein in the urine), and episodes of gross haematuria following mucosal infections are common; 30% of patients develop progressive renal failure 6, 7, 8, 9. Although not generally considered a hereditary disease, striking ethnic variation in prevalence 1, 2, 3, 4, 5, 6, 10 and familial clustering 11, 12, 13, 14, 15, 16, along with …