The Japanese Journal of Clinical Dialysis Vol.32 No.4(4-1)

Theme Designated intractable kidney diseases:The control in non-dialysis CKD and renal replacement therapy
Title Autosomal dominant polycystic kidney disease
Publish Date 2016/04
Author Mitsuru Ichii Department of Metabolism, Osaka City University Graduate School of Medicine
Author Eiji Ishimura Department of Endocrinology & Molecular Medicine, Osaka City University Graduate School of Medicine
[ Summary ] Autosomal dominant polycystic kidney disease (ADPKD) is the most common form of hereditary kidney disease, in which PKD1 and PKD2 genes are identified as the cause. About half of the ADPKD patients need maintenance hemodialysis until the age of 60. For the diagnosis of ADPKD, imaging techniques, such as ultrasounds, computed tomography and magnetic resonance imaging, are needed. Only conservative treatments using medications such as antihypertensives were available, before, tolvaputan was approved for ADPKD treatment in 2014 in Japan. It has been reported that tolvaputan can suppress cyst swelling and deterioration of renal function. For renal replacement therapy, not only hemodialsysis, but also peritoneal dialysis therapy may be employed. Renal ADPKD cysts may be reduced in size after renal transplantation.
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