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BACKGROUND Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive enlargement of cyst-filled kidneys. METHODS In a three-year study, we measured the rates of change in total kidney volume, total cyst volume, and iothalamate clearance in patients with ADPKD. Of a total of 241 patients, in 232 patients without azotemia who were(More)
BACKGROUND Autosomal-dominant polycystic kidney disease (ADPKD) is characterized by gradual renal enlargement and cyst growth prior to loss of renal function. Standard radiographic imaging has not provided the resolution and accuracy necessary to detect small changes in renal volume or to reliably measure renal cyst volumes. The Consortium for Radiologic(More)
The immunosuppressive agent sirolimus exerts an antiproliferative effect by inhibiting mammalian target of rapamycin (mTOR). Because excessive proliferation of the biliary epithelium is a prominent feature of the polycystic liver that accompanies autosomal dominant polycystic kidney disease (ADPKD), we hypothesized that sirolimus may benefit patients with(More)
A dynamic MR angiography technique, MR digital subtraction angiography (MR DSA), is proposed using fast acquisition, contrast enhancement, and complex subtraction. When a bolus of contrast is injected into a patient, data acquisition begins, dynamically acquiring a thick slab using a fast gradient echo sequence for 10-100 s. Similar to x-ray DSA, a mask is(More)
There are no proven, effective therapies for polycystic kidney disease (PKD) or polycystic liver disease (PLD). We enrolled 42 patients with severe PLD resulting from autosomal dominant PKD (ADPKD) or autosomal dominant PLD (ADPLD) in a randomized, double-blind, placebo-controlled trial of octreotide, a long-acting somatostatin analogue. We randomly(More)
Data from serial renal magnetic resonance imaging of the Consortium of Radiologic Imaging Study of PKD (CRISP) autosomal dominant polycystic kidney disease (PKD) population showed that cystic expansion occurs at a consistent rate per individual, although it is heterogeneous in the population, and that larger kidneys are associated with more rapid disease(More)
Most reports on the natural history, manifestations, and treatment of polycystic liver disease are based on the disease as it manifests in patients with autosomal dominant polycystic kidney disease (ADPKD). The purpose of this study was to develop a clinical profile of isolated autosomal dominant polycystic liver disease (ADPLD) using nonaffected family(More)
BACKGROUND Autosomal-dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by renal cyst growth, early development of hypertension, and late occurrence of renal insufficiency. Despite evidence for the importance of nephroangiosclerosis in the progression of renal insufficiency in ADPKD, evaluation of renal blood flow (RBF) as a(More)
This study evaluates reliability of current technology for measurement of renal arterial blood flow by breath-held velocity-encoded MRI. Overall accuracy was determined by comparing MRI measurements with known flow in controlled-flow-loop phantom studies. Measurements using prospective and retrospective gating methods were compared in phantom studies with(More)
Alterations in the mechanical properties or "hardness" of tissues allow physicians to detect disease by palpation. Recently, attempts have been made to quantitate and image these tissue properties with the use of magnetic resonance elastography (MRE). This technique has been validated in ex vivo specimens, including kidney, breast, and prostate. In this(More)