Abstract
Recently, a large randomized placebo-controlled trial indicated a beneficial effect of tolvaptan on the progression of autosomal dominant polycystic kidney disease (ADPKD) with near-normal kidney function. Meanwhile, the evidence of tolvaptan's efficacy in ADPKD with severe renal insufficiency was limited and higher frequency of liver enzyme elevations were observed in patients taking tolvaptan. Liver transplantation (LT) is the only curative treatment for patients with severe polycystic liver disease associated with ADPKD, but considering that liver injuries should be avoided particularly in patients who underwent LT, we must be careful to start tolvaptan in post-LT ADPKD patients. We describe the case of a patient who had developed severe renal insufficiency after living donor LT, for whom tolvaptan therapy showed marked reduction of total kidney volume and maintenance of renal function without any serious adverse events. This is the first report to show the beneficial effect and safety of tolvaptan, in a post-LT ADPKD patient with severe renal insufficiency, and hopefully will help broaden the spectrum of patients who will benefit from tolvaptan.