Results: We included 271 patients in this study (176 female and 95 male). Median age was 52 years. The mean estimated GFR was 49.3 ml/min/1.73 m2. Several parameters including waist

circumference (r2 = 0.059, p = 0.001), systolic blood pressure (r2 = 0.048, p < 0.001), total kidney volume (r2 = 0.247, p < 0.001) were significantly inversely correlated with eGFR. There were significant correlations between eGFR and Hemoglobin level (r2 = 0.259, p < 0.001), serum alubumin (r2 = 0.081, PLX4032 datasheet p < 0.001). Conclusion: In this cohort study, we will clear the actual treatment course of PKD in Japan. YAMAMOTO JUNYA, ISHIKAWA YASUNOBU, NAKAGAKI TASUKU, SHIBAZAKI SEKIYA, NISHIO SAORI, ATSUMI TATSUYA Hokkaido University Graduate School of Medicine Department

of Medicine 2 Introduction: Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive development and enlargement of renal and liver cysts. Mammalian target of rapamycin (mTOR) cascade is one of the important pathways regulating cyst growth. It has been reported that PXD101 concentration mTOR inhibitor can inhibit cyst growth. Branched-chain amino acids (BCAA), which developed for the purpose of improving hypo-albuminemia in patients with uncompensated liver cirrhosis, have crucial role to activate mTOR cascade. However, there is little information related to the influence of BCAA on ADPKD. We investigated the effects of BCAA in Pkd1flox/flox:Mx1-Cre mice. Methods: Pkd1flox/flox:Mx1-Cre mice were intraperitoneally injected with 10 μg/g body weight of polyinosinic-polycytidylic acid for 6 consecutive days at 2 weeks of age to inactivate

Pkd1. To evaluate the effect s of BCAA, we prepared BCAA (0.250 g isoleucine/g, leucine 0.500 g/g and 0.250 g/g valine) dissolved drinking water and placebo (cornstarch alone) drinking water Tideglusib and mice were assigned to BCAA group or placebo group. BCAA or placebo was administered from 4 weeks to 16 or 22 weeks of age. We carried out a series of analyses by kidney/body weight ratio, liver/body weight ratio, cystic index (CI) which is defined as the percent of total cross-sectional area occupied by cysts, histology, cell proliferation and apoptosis at specific time points of 16 and 22 weeks of age. We investigated MAPK pathway and mTOR pathway by Western blotting. Results: The kidney/body weight ratio was signigficantly greater in BCAA group than in Placebo group at 22 weeks of age. CI was significantly greater in BCAA group than in Placebo group at 22 weeks of age in both kidney and liver.