Published in Clin J Am Soc Nephrol on October 26, 2005
Autosomal dominant polycystic kidney disease: the last 3 years. Kidney Int (2009) 3.66
Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol (2010) 3.47
Sirolimus reduces polycystic liver volume in ADPKD patients. J Am Soc Nephrol (2008) 2.26
Functional polycystin-1 dosage governs autosomal dominant polycystic kidney disease severity. J Clin Invest (2012) 1.74
A metabolomics approach using juvenile cystic mice to identify urinary biomarkers and altered pathways in polycystic kidney disease. Am J Physiol Renal Physiol (2010) 1.63
Imaging for the prognosis of autosomal dominant polycystic kidney disease. Nat Rev Nephrol (2010) 1.63
Liver and kidney disease in ciliopathies. Am J Med Genet C Semin Med Genet (2009) 1.53
Autosomal-dominant polycystic kidney disease (ADPKD): executive summary from a Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference. Kidney Int (2015) 1.50
Novel approach to estimate kidney and cyst volumes using mid-slice magnetic resonance images in polycystic kidney disease. Am J Nephrol (2013) 1.50
Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol (2013) 1.46
Evaluation of hepatic cystic lesions. World J Gastroenterol (2013) 1.43
Reducing polycystic liver volume in ADPKD: effects of somatostatin analogue octreotide. Clin J Am Soc Nephrol (2010) 1.36
Polycystic liver disease: an overview of pathogenesis, clinical manifestations and management. Orphanet J Rare Dis (2014) 1.35
Mammalian target of rapamycin regulates vascular endothelial growth factor-dependent liver cyst growth in polycystin-2-defective mice. Hepatology (2010) 1.31
ERK1/2-dependent vascular endothelial growth factor signaling sustains cyst growth in polycystin-2 defective mice. Gastroenterology (2009) 1.29
MRI-based kidney volume measurements in ADPKD: reliability and effect of gadolinium enhancement. Clin J Am Soc Nephrol (2009) 1.24
ARPKD and early manifestations of ADPKD: the original polycystic kidney disease and phenocopies. Pediatr Nephrol (2014) 1.17
Analysis of baseline parameters in the HALT polycystic kidney disease trials. Kidney Int (2011) 1.13
Determinants of renal disease variability in ADPKD. Adv Chronic Kidney Dis (2010) 1.11
Altered store operated calcium entry increases cyclic 3',5'-adenosine monophosphate production and extracellular signal-regulated kinases 1 and 2 phosphorylation in polycystin-2-defective cholangiocytes. Hepatology (2012) 1.09
Isolated polycystic liver disease. Adv Chronic Kidney Dis (2010) 1.08
Imaging approaches to patients with polycystic kidney disease. Semin Nephrol (2011) 1.07
Pathophysiology, epidemiology, classification and treatment options for polycystic liver diseases. World J Gastroenterol (2013) 1.04
Liver involvement in early autosomal-dominant polycystic kidney disease. Clin Gastroenterol Hepatol (2014) 1.03
Clinical Pattern of Tolvaptan-Associated Liver Injury in Subjects with Autosomal Dominant Polycystic Kidney Disease: Analysis of Clinical Trials Database. Drug Saf (2015) 1.02
Health-related quality of life in patients with autosomal dominant polycystic kidney disease and CKD stages 1-4: a cross-sectional study. Am J Kidney Dis (2013) 1.01
Whole-exome sequencing reveals LRP5 mutations and canonical Wnt signaling associated with hepatic cystogenesis. Proc Natl Acad Sci U S A (2014) 1.00
Diagnosis and management of childhood polycystic kidney disease. Pediatr Nephrol (2010) 0.99
CXCR2 agonists in ADPKD liver cyst fluids promote cell proliferation. Am J Physiol Cell Physiol (2008) 0.94
Renal relevant radiology: radiologic imaging in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol (2013) 0.94
Polycystic liver diseases. Dig Liver Dis (2010) 0.93
Segmentation of individual renal cysts from MR images in patients with autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol (2013) 0.91
N-glycosylation determines the abundance of the transient receptor potential channel TRPP2. J Biol Chem (2014) 0.91
Educational paper: ciliopathies. Eur J Pediatr (2011) 0.90
Evaluation and management of pain in autosomal dominant polycystic kidney disease. Adv Chronic Kidney Dis (2010) 0.86
Clinical characteristics and predictors of progression of chronic kidney disease in autosomal dominant polycystic kidney disease: a single center experience. Clin Exp Nephrol (2012) 0.85
Intermediate volume on computed tomography imaging defines a fibrotic compartment that predicts glomerular filtration rate decline in autosomal dominant polycystic kidney disease patients. Am J Pathol (2011) 0.85
Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment. Int J Nephrol Renovasc Dis (2010) 0.85
Rationale and design of the DIPAK 1 study: a randomized controlled clinical trial assessing the efficacy of lanreotide to Halt disease progression in autosomal dominant polycystic kidney disease. Am J Kidney Dis (2013) 0.84
Distinct patterns of kidney and liver cyst growth in pkd2(WS25/-) mice. Nephrol Dial Transplant (2010) 0.84
A stepwise approach for effective management of chronic pain in autosomal-dominant polycystic kidney disease. Nephrol Dial Transplant (2014) 0.82
Efficacy of 4 Years of Octreotide Long-Acting Release Therapy in Patients With Severe Polycystic Liver Disease. Mayo Clin Proc (2015) 0.82
Epidemiology and mortality of liver abscess in end-stage renal disease dialysis patients: Taiwan national cohort study. PLoS One (2014) 0.82
Everolimus and long acting octreotide as a volume reducing treatment of polycystic livers (ELATE): study protocol for a randomized controlled trial. Trials (2011) 0.82
Effect of genotype on the severity and volume progression of polycystic liver disease in autosomal dominant polycystic kidney disease. Nephrol Dial Transplant (2016) 0.81
Automated Segmentation of Kidneys from MR Images in Patients with Autosomal Dominant Polycystic Kidney Disease. Clin J Am Soc Nephrol (2016) 0.81
Treatment of autosomal dominant polycystic kidney disease (ADPKD): the new horizon for children with ADPKD. Pediatr Nephrol (2008) 0.80
From bone abnormalities to mineral metabolism dysregulation in autosomal dominant polycystic kidney disease. Pediatr Nephrol (2013) 0.80
Clinical Correlates of Mass Effect in Autosomal Dominant Polycystic Kidney Disease. PLoS One (2015) 0.79
Volume regression of native polycystic kidneys after renal transplantation. Nephrol Dial Transplant (2015) 0.78
microRNA biomarkers in cystic diseases. BMB Rep (2013) 0.78
PKD1 mono-allelic knockout is sufficient to trigger renal cystogenesis in a mini-pig model. Int J Biol Sci (2015) 0.78
Image texture features predict renal function decline in patients with autosomal dominant polycystic kidney disease. Kidney Int (2017) 0.78
Novel methodology to evaluate renal cysts in polycystic kidney disease. Am J Nephrol (2014) 0.77
The spectrum of autosomal dominant polycystic kidney disease in children and adolescents. Pediatr Nephrol (2016) 0.76
Use of Ultra-high Field MRI in Small Rodent Models of Polycystic Kidney Disease for In Vivo Phenotyping and Drug Monitoring. J Vis Exp (2015) 0.76
Outcomes and Durability of Hepatic Reduction after Combined Partial Hepatectomy and Cyst Fenestration for Massive Polycystic Liver Disease. J Am Coll Surg (2016) 0.76
Human Polycystin-2 Transgene Dose-Dependently Rescues ADPKD Phenotypes in Pkd2 Mutant Mice. Am J Pathol (2015) 0.76
Laparoscopic surgery and polycystic liver disease: Clinicopathological features and new trends in management. J Minim Access Surg (2016) 0.75
Creating an effective clinical registry for rare diseases. United European Gastroenterol J (2015) 0.75
Polycystic liver disease. BMJ Case Rep (2014) 0.75
Automated segmentation of liver and liver cysts from bounded abdominal MR images in patients with autosomal dominant polycystic kidney disease. Phys Med Biol (2016) 0.75
Acute abdomen and ascites as presenting features of autosomal dominant polycystic kidney disease. World J Hepatol (2012) 0.75
Complete heart block with diastolic heart failure and pulmonary edema secondary to enlarging previously diagnosed thrombosed aneurysm of sinus of valsalva in a patient with history of autosomal dominant polycystic kidney disease. Case Rep Cardiol (2015) 0.75
LRP5 variants may contribute to ADPKD. Eur J Hum Genet (2015) 0.75
Pancreatic Cysts in Autosomal Dominant Polycystic Kidney Disease: Prevalence and Association with PKD2 Gene Mutations. Radiology (2016) 0.75
Total kidney and liver volume is a major risk factor for malnutrition in ambulatory patients with autosomal dominant polycystic kidney disease. BMC Nephrol (2017) 0.75
Chromosomal abnormalities in hepatic cysts point to novel polycystic liver disease genes. Eur J Hum Genet (2016) 0.75
Biliary Tract and Liver Complications in Polycystic Kidney Disease. J Am Soc Nephrol (2017) 0.75
Rapamycin treatment dose-dependently improves the cystic kidney in a new ADPKD mouse model via the mTORC1 and cell-cycle-associated CDK1/cyclin axis. J Cell Mol Med (2017) 0.75
Identification and analysis of functional elements in 1% of the human genome by the ENCODE pilot project. Nature (2007) 75.09
Nivolumab plus ipilimumab in advanced melanoma. N Engl J Med (2013) 17.53
The Ocular Hypertension Treatment Study: a randomized trial determines that topical ocular hypotensive medication delays or prevents the onset of primary open-angle glaucoma. Arch Ophthalmol (2002) 16.89
The Ocular Hypertension Treatment Study: baseline factors that predict the onset of primary open-angle glaucoma. Arch Ophthalmol (2002) 11.38
Tolvaptan in patients with autosomal dominant polycystic kidney disease. N Engl J Med (2012) 9.88
Effect of constraint-induced movement therapy on upper extremity function 3 to 9 months after stroke: the EXCITE randomized clinical trial. JAMA (2006) 9.55
Effect of clopidogrel on early failure of arteriovenous fistulas for hemodialysis: a randomized controlled trial. JAMA (2008) 7.50
Volume progression in polycystic kidney disease. N Engl J Med (2006) 7.16
Comparative genomics identifies a flagellar and basal body proteome that includes the BBS5 human disease gene. Cell (2004) 6.10
The polycystic kidney disease proteins, polycystin-1, polycystin-2, polaris, and cystin, are co-localized in renal cilia. J Am Soc Nephrol (2002) 5.58
Blood pressure in early autosomal dominant polycystic kidney disease. N Engl J Med (2014) 5.01
Renal structure in early autosomal-dominant polycystic kidney disease (ADPKD): The Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) cohort. Kidney Int (2003) 4.84
Polycystic kidney disease. Annu Rev Med (2009) 4.78
The gene mutated in autosomal recessive polycystic kidney disease encodes a large, receptor-like protein. Nat Genet (2002) 4.54
Candidate exome capture identifies mutation of SDCCAG8 as the cause of a retinal-renal ciliopathy. Nat Genet (2010) 3.99
Association between adverse clinical outcome in human disease caused by novel influenza A H7N9 virus and sustained viral shedding and emergence of antiviral resistance. Lancet (2013) 3.83
Inhibition of renal cystic disease development and progression by a vasopressin V2 receptor antagonist. Nat Med (2003) 3.78
Longitudinal course and neuropathologic outcomes in original vs revised MCI and in pre-MCI. Neurology (2006) 3.69
Effect of dipyridamole plus aspirin on hemodialysis graft patency. N Engl J Med (2009) 3.69
Efficacy of maintenance treatment approaches for childhood overweight: a randomized controlled trial. JAMA (2007) 3.50
Randomized clinical trial of long-acting somatostatin for autosomal dominant polycystic kidney and liver disease. J Am Soc Nephrol (2010) 3.47
Incidental findings in imaging research: evaluating incidence, benefit, and burden. Arch Intern Med (2010) 3.40
Comprehensive molecular diagnostics in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2007) 3.26
Controlled trial of transfusions for silent cerebral infarcts in sickle cell anemia. N Engl J Med (2014) 3.25
PKHD1, the polycystic kidney and hepatic disease 1 gene, encodes a novel large protein containing multiple immunoglobulin-like plexin-transcription-factor domains and parallel beta-helix 1 repeats. Am J Hum Genet (2002) 3.21
The transmembrane protein meckelin (MKS3) is mutated in Meckel-Gruber syndrome and the wpk rat. Nat Genet (2006) 3.19
Incompletely penetrant PKD1 alleles suggest a role for gene dosage in cyst initiation in polycystic kidney disease. Kidney Int (2009) 2.99
Effectiveness of vasopressin V2 receptor antagonists OPC-31260 and OPC-41061 on polycystic kidney disease development in the PCK rat. J Am Soc Nephrol (2005) 2.92
Genomic association analysis identifies multiple loci influencing antihypertensive response to an angiotensin II receptor blocker. Hypertension (2012) 2.86
Kidney volume and functional outcomes in autosomal dominant polycystic kidney disease. Clin J Am Soc Nephrol (2012) 2.78
Vasopressin directly regulates cyst growth in polycystic kidney disease. J Am Soc Nephrol (2007) 2.71
Octreotide inhibits hepatic cystogenesis in a rodent model of polycystic liver disease by reducing cholangiocyte adenosine 3',5'-cyclic monophosphate. Gastroenterology (2006) 2.68
Volume progression in autosomal dominant polycystic kidney disease: the major factor determining clinical outcomes. Clin J Am Soc Nephrol (2005) 2.66
Cellular and subcellular localization of the ARPKD protein; fibrocystin is expressed on primary cilia. Hum Mol Genet (2003) 2.55
Cyclic AMP promotes growth and secretion in human polycystic kidney epithelial cells. Kidney Int (2004) 2.55
High urine volume and low urine osmolality are risk factors for faster progression of renal disease. Am J Kidney Dis (2003) 2.41
Influence of nitrous oxide anesthesia, B-vitamins, and MTHFR gene polymorphisms on perioperative cardiac events: the vitamins in nitrous oxide (VINO) randomized trial. Anesthesiology (2013) 2.39
Evaluation of urine biomarkers of kidney injury in polycystic kidney disease. Kidney Int (2012) 2.39
MicroRNAs as modulators of smoking-induced gene expression changes in human airway epithelium. Proc Natl Acad Sci U S A (2009) 2.37
Rates of progression in mild cognitive impairment and early Alzheimer's disease. Neurology (2002) 2.34
Clinical and molecular characterization defines a broadened spectrum of autosomal recessive polycystic kidney disease (ARPKD). Medicine (Baltimore) (2006) 2.34
Cyclic AMP activates B-Raf and ERK in cyst epithelial cells from autosomal-dominant polycystic kidneys. Kidney Int (2003) 2.32
High-sensitivity cardiac troponin T in prediction and diagnosis of myocardial infarction and long-term mortality after noncardiac surgery. Am Heart J (2013) 2.30
Validated prediction model for the development of primary open-angle glaucoma in individuals with ocular hypertension. Ophthalmology (2006) 2.28
Drug dosing consideration in patients with acute and chronic kidney disease-a clinical update from Kidney Disease: Improving Global Outcomes (KDIGO). Kidney Int (2011) 2.28
Sirolimus reduces polycystic liver volume in ADPKD patients. J Am Soc Nephrol (2008) 2.26
Calcium restriction allows cAMP activation of the B-Raf/ERK pathway, switching cells to a cAMP-dependent growth-stimulated phenotype. J Biol Chem (2004) 2.26
Polycystic liver disease: a critical appraisal of hepatic resection, cyst fenestration, and liver transplantation. Ann Surg (2009) 2.23
Methods for a multisite randomized trial to investigate the effect of constraint-induced movement therapy in improving upper extremity function among adults recovering from a cerebrovascular stroke. Neurorehabil Neural Repair (2003) 2.22
Cyst number but not the rate of cystic growth is associated with the mutated gene in autosomal dominant polycystic kidney disease. J Am Soc Nephrol (2006) 2.21