Research progress of renal damage in acute intermittent porphyria

With the development of genetics and molecular biology,the research of acute intermittent porphyria (AIP) has been studied increasingly,and its related renal damage has attracted more and more attention.However,pathogenesis of renal damage related to AIP,belonging to porphyrin-associated nephropathy(PAKD),was associated with oxidative stress,protein aggregation,renal arterial vasoconstriction,renal tubular injury,PEPT2*1 protein expression,renal epithelial cells phenotypic transformation,and inflammatory cytokines increase.Its treatment regimes include PEPT2 inhibitor use,combined liver and kidney transplantation,and reducing ALA/PBG levels.In this article,we reviewed the new progress in pathogenesis and therapies of PAKD including the targeted therapy.