Kidney transplant patient with immunoglobulin A nephropathy subsequently diagnosed as concurrent autosomal dominant polycystic kidney disease during 17-year follow-up

Abstract

A 14-year-old Japanese boy was diagnosed with immunoglobulin A nephropathy resulting in end-stage kidney disease (ESKD). He underwent ABO-compatible living kidney transplantation from his father at the age of 27. In the process of selecting a donor before the transplantation, it turned out that his mother had polycystic kidneys and that her family had a history of hypertension and cerebrovascular diseases. The patient himself also had bilateral multiple kidney cysts, with a normal-sized kidney, confusing us to make the diagnosis of acquired cystic kidney disease (ACKD) or ADPKD difficult at that point. Seventeen years later, his native kidneys showed bilateral swelling with multiple cysts. This, along with the histories of his mother and her relatives and with the existence of multiple liver cysts, led us to confirm the diagnosis of autosomal dominant polycystic kidney disease, not of ACKD. Contrary to previous studies that have suggested the size of cysts both in ADPKD and ACKD reduced with time, the present case showed an increase of 3.0% per year in total kidney volume (TKV) by computed tomography. It suggested the possibility that TKV, after decreasing in the relatively early stage after transplantation, may later increase in the long term after ESKD due to another kidney injury.