examination to determine blood pressure, abdominal girth, heartbeat, abdominal findings, and edema; blood and urine tests, screening for urinary sediment, proteinuria, and microalbuminuria; estimated glomerular filtration rate (eGFR) and other renal function tests; and screening for intracranial aneurysm through cranial MR angiography. US represents the simplest form of diagnostic imaging for kidney diseases. Other tests to be performed, as appropriate, should include measurement of Nacetyl beta-glucosaminidase and urinary beta2 microglobulin values, MRI, and kidney CT imaging. 5) Diagnostic imaging US is the standard screening technique for ADPKD diagnosis and evaluation, but evaluation of kidney size, as opposed to function, is reportedly the better measurement in the evaluation of progression, with CT or MRI recommended for follow-up evaluation. The latter methods surpass US in detecting smaller cysts; MRI can detect cysts with a diameter of 2 mm through T2-weighted imaging. Each diagnostic imaging technique (US, CT, and MRI) plays a role in highlighting the distinctive characteristics of cysts. Diagnostic imaging is also clinically important in terms of disease complications such as cerebral aneurysms. As adverse reactions can occur, careful consideration must be given to the risk–benefit balance before utilizing contrast media. MRA is useful in screening for cerebral aneurysms and is a noninvasive test with the great benefit of not requiring contrast media. If imaging performed after a definitive ADPKD diagnosis is strictly for follow-up observation, a simple CT once every 2–5 years would be adequate if total kidney volume (TKV) is ≤1,000 mL. If TKV exceeds 1,000 mL, CT once every year or two would be appropriate. For screening purposes, diagnostic imaging at the age of 30 years is recommended. 6) Differential diagnosis A patient’s clinical manifestation and diagnostic imaging should be used to rule out possibilities such as multiple simple renal cysts, acquired cystic kidney disease, and tuberous sclerosis (Table 2). Particular caution is needed when considering tuberous sclerosis, as approximately 30% of patients with this disease are said to have no typical symptoms other than renal cysts, which are mistakenly attributed to ADPKD. Additional diseases to be ruled out include renal tubular acidosis, multicystic kidney (multicystic dysplastic kidney), multilocular cyst of the kidney, medullary cystic kidney disease, and oral–facial–digital syndrome. As rare diseases are difficult to identify and distinguish during normal medical examinations, despite reports on characteristic indicators other than renal cysts, extra care should be given during differential diagnosis. Table 2 Major non-ADPKD renal cystic diseases Disease Cyst proliferation Cyst distribution/size Typical life stage for cyst diagnosis Pathophysiological characteristics Multiple simple renal cyst Moderate Size diversity/nonuniform distribution All ages Rare under age 30 years; manifestation increases with age Acquired cystic disease of the kidney Moderate to great Diffusibility Adulthood Cyst formation precedes ESRD Tuberous sclerosis Moderate to great Uniform distribution of Per-Treatment Post Hoc Analysis of Clinical Trial Outcomes With Tolvaptan in ADPKD Andrew J. Mallett1,2 , Ronald D. Perrone3 , Gopala Rangan4,5 , Carmel Hawley6,7 , Ragada El-Damanawi8,9 , Thomas F. Hiemstra8,9 , Carolina Townsend Arellano1 , Jennifer Lee10 and Vicente E. Torres11 1 Institute for Molecular Bioscience and Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia; 2 Department of Renal Medicine, Townsville University Hospital, Townsville, Queensland, Australia; 3 Division of Nephrology, Department of Medicine, Tufts Medical Center and Tufts University School of Medicine, Boston, Massachusetts, USA; 4 Centre for Transplant and Renal Research, Westmead Institute for Medical Research, The University of Sydney, Sydney, New South Wales, Australia; 5 Department of Renal Medicine, Westmead Hospital, Western Sydney Local Health District, Sydney, New South Wales, Australia; 6 Department of Nephrology, Princess Alexandra Hospital, Brisbane, Queensland, Australia; 7 Translational Research Institute, Brisbane, Queensland, Australia; 8 Division of Experimental Medicine and Immunotherapeutics, Department of Medicine, University of Cambridge, Cambridge, UK; 9 Cambridge Clinical Trials Unit, Addenbrooke’s Hospital, Cambridge, UK; 10Biostatistics, Otsuka Pharmaceutical Development & Commercialization, Inc., Rockville, Maryland, USA; and 11Division of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA Introduction: In pivotal trials of patients with autosomal dominant polycystic kidney disease at risk of rapid progression, tolvaptan slowed estimated glomerular filtration rate (eGFR) decline in early-to-moderate (TEMPO 3:4 [NCT00428948]) and moderate- to late-stage (REPRISE [NCT02160145]) chronic kidney disease (CKD). Discontinuation was less frequent in REPRISE (15.0%) than TEMPO 3:4 (23.0%), given that in REPRISE, only subjects who tolerated tolvaptan 60/30 mg daily initiated the double-blind phase. We evaluated whether the greater treatment effect in REPRISE was attributable to different completion rates. Methods: We conducted post hoc analyses of TEMPO 3:4 and REPRISE completers, defined as subjects who took