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autosomal dominant polycystic kidney

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https://www.readbyqxmd.com/read/28340010/non-proteinuric-rather-than-proteinuric-renal-diseases-are-the-leading-cause-of-end-stage-kidney-disease
#1
Davide Bolignano, Carmine Zoccali
Proteinuria is a distinguishing feature in primary and secondary forms of chronic glomerulonephritis, which contribute to no more than the 20% of the end-stage kidney disease (ESKD) population. The contribution of non-proteinuric nephropathies to the global ESKD burden is still poorly focused and scarce research efforts are dedicated to the elucidation of risk factors and mechanistic pathways triggering ESKD in these diseases. We abstracted information on proteinuria in the main renal diseases other than glomerulonephritides that may evolve into ESKD...
March 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28332417/cost-effectiveness-of-angiotensin-converting-enzyme-inhibitors-versus-angiotensin-ii-receptor-blockers-as-first-line-treatment-in-autosomal-dominant-polycystic-kidney-disease
#2
L A Clark, S Whitmire, S Patton, C Clark, C M Blanchette, R Howden
INTRODUCTION: Autosomal dominant polycystic kidney disease (ADPKD) is a rare kidney disorder impacting approximately 1:2500 individuals among the general US population. Hypertension is a significant predictor of ADPKD progression and risk factor for development of cardiovascular disease (CVD), the most common cause for mortality among ADPKD patients. Angiotensin-converting enzymes inhibitors (ACE-I) are widely used as first-line treatment in ADPKD for the management of hypertension. However, their cost-effectiveness relative to other hypertensive medications, such as angiotensin II receptor blockers (ARB) has never been assessed...
March 23, 2017: Journal of Medical Economics
https://www.readbyqxmd.com/read/28321325/assessing-risk-of-disease-progression-and-pharmacological-management-of-autosomal-dominant-polycystic-kidney-disease-a-canadian-expert-consensus
#3
Steven Soroka, Ahsan Alam, Micheli Bevilacqua, Louis-Philippe Girard, Paul Komenda, Rolf Loertscher, Philip McFarlane, Sanjaya Pandeya, Paul Tam, Daniel G Bichet
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited renal disorder worldwide. The disease is characterized by renal cysts and progressive renal failure due to progressive enlargement of cysts and renal fibrosis. An estimated 45% to 70% of patients with ADPKD progress to end-stage renal disease by age 65 years. Although both targeted and nontargeted therapies have been tested in patients with ADPKD, tolvaptan is currently the only pharmacological therapy approved in Canada for the treatment of ADPKD...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28321323/total-kidney-volume-as-a-biomarker-of-disease-progression-in-autosomal-dominant-polycystic-kidney-disease
#4
REVIEW
Navdeep Tangri, Ingrid Hougen, Ahsan Alam, Ronald Perrone, Phil McFarlane, York Pei
PURPOSE OF REVIEW: Autosomal dominant polycystic kidney disease (ADPKD) is an inherited disorder characterized by the formation of kidney cysts and kidney enlargement, which progresses to kidney failure by the fifth to seventh decade of life in a majority of patients. Disease progression is evaluated primarily through serum creatinine and estimated glomerular filtration rate (eGFR) measurements; however, it is known that serum creatinine and eGFR values typically do not change until the fourth or fifth decade of life...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28321250/percutaneous-nephrolithotomy-under-ultrasound-guidance-in-patients-with-renal-calculi-and-autosomal-dominant-polycystic-kidney-disease-a-report-of-11-cases
#5
Xiao Wang, Xuecheng Yang, Xiulong Zhong, Zhenlin Wang, Senyao Xue, Weifeng Yu, Zhen Dong
Nephrolithiasis accelerates the renal failure in the patients with ADPKD. In order to evaluate the role of percutaneous nephrolithotomy in management of calculus in these patients, 11 patients with autosomal dominant polycystic kidney disease and renal stones were included in the study. Two patients had bilateral renal stones. All patients were treated by percutaneous nephrolithotomy under ultrasound guidance. 13 percutaneous nephrolithotomy procedures were performed in 1 stage by the urology team under ultrasound guidance...
2017: Advances in Urology
https://www.readbyqxmd.com/read/28320755/ciliary-mechanisms-of-cyst-formation-in-polycystic-kidney-disease
#6
Ming Ma, Anna-Rachel Gallagher, Stefan Somlo
Autosomal-dominant polycystic kidney disease (ADPKD) is a disease of defective tissue homeostasis resulting in active remodeling of nephrons and bile ducts to form fluid-filled sacs called cysts. The causal genes PKD1 and PKD2 encode transmembrane proteins polycystin 1 (PC1) and polycystin 2 (PC2), respectively. Together, the polycystins localize to the solitary primary cilium that protrudes from the apical surface of most kidney tubule cells and is thought to function as a privileged compartment that the cell uses for signal integration of sensory inputs...
March 20, 2017: Cold Spring Harbor Perspectives in Biology
https://www.readbyqxmd.com/read/28316316/colonic-diverticulosis-and-diverticulitis-in-renal-transplant-recipients-management-and-long-term-outcomes
#7
Ahmet Rencuzogullari, Gokhan Ozuner, Sinan Binboga, Erman Aytac, Venkatesh Krishnamurthi, Emre Gorgun
Data regarding management of colonic diverticulitis in renal transplant recipients (RTRs) are limited. This study aims to identify prevalence, risk factors, and outcomes in RTRs with colonic diverticulosis and diverticulitis. Between January 2004 and December 2013, all patients who underwent kidney transplantation were analyzed. Among all RTSs, patients who had a pretransplant colonoscopic diagnosis of diverticulosis and patients with a proven attack of diverticulitis were included in our analysis. There were 1578 RTRs with a mean age of 50 ± 14 years at the time of transplantation...
March 1, 2017: American Surgeon
https://www.readbyqxmd.com/read/28315882/comparison-of-total-kidney-volume-quantification-methods-in-autosomal-dominant-polycystic-disease-for-a-comprehensive-disease-assessment
#8
Dario Turco, Marco Busutti, Renzo Mignani, Riccardo Magistroni, Cristiana Corsi
BACKGROUND: In recent times, the scientific community has been showing increasing interest in the treatments aimed at slowing the progression of the autosomal dominant polycystic kidney disease (ADPKD). Therefore, in this paper, we test and evaluate the performance of several available methods for total kidney volume (TKV) computation in ADPKD patients - from echography to MRI - in order to optimize patient classification. METHODS: Two methods based on geometric assumptions (mid-slice [MS], ellipsoid [EL]) and a third one on true contour detection were tested on 40 ADPKD patients at different disease stage using MRI...
March 18, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28283868/intracranial-aneurysms-in-patients-with-autosomal-dominant-polycystic-kidney-disease-prevalence-risk-of-rupture-and-management-a-systematic-review
#9
REVIEW
Federico Cagnazzo, Carlo Gambacciani, Riccardo Morganti, Paolo Perrini
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) is a genetic disorder associated with high incidences of intracranial aneurysms. We performed a systematic review with the purpose of clarifying the prevalence, risk of rupture, and appropriate management of intracranial aneurysms in the ADPKD population. METHOD: PRISMA guidelines were followed. We conducted a comprehensive literature search of three databases (PubMed, Ovid MEDLINE, and Ovid EMBASE) on all series reporting ADPKD patients with intracranial aneurysms...
March 10, 2017: Acta Neurochirurgica
https://www.readbyqxmd.com/read/28271061/identification-and-expression-analysis-of-the-complete-family-of-zebrafish-pkd-genes
#10
Samantha J England, Paul C Campbell, Santanu Banerjee, Annika J Swanson, Katharine E Lewis
Polycystic kidney disease (PKD) proteins are trans-membrane proteins that have crucial roles in many aspects of vertebrate development and physiology, including the development of many organs as well as left-right patterning and taste. They can be divided into structurally-distinct PKD1-like and PKD2-like proteins and usually one PKD1-like protein forms a heteromeric polycystin complex with a PKD2-like protein. For example, PKD1 forms a complex with PKD2 and mutations in either of these proteins cause Autosomal Dominant Polycystic Kidney Disease (ADPKD), which is the most frequent potentially-lethal single-gene disorder in humans...
2017: Frontiers in Cell and Developmental Biology
https://www.readbyqxmd.com/read/28260467/outcome-of-kidney-transplant-in-patients-with-polycystic-kidney-disease-a-single-center-study-in-tunisia
#11
Samia Barbouch, Meriam Hajji, Hafedh Hedri, Fethi El Younsi, Fathi Ben Hamida, Mohammed Mongi Bacha, Mondher Ounissi, Ezzedine Abderrahim, Taieb Ben Abdallah
OBJECTIVES: Autosomal dominant polycystic kidney disease is a common cause of end-stage renal disease and a common indication for renal transplant. This study was undertaken to evaluate the demographics, outcomes, and complications of renal transplant in patients with autosomal dominant polycystic kidney disease compared with other nephropathies. MATERIALS AND METHODS: In a retrospective case-control design, we reviewed the records of 7 patients with autosomal dominant polycystic kidney disease from a total of 701 renal transplant patients over a 30-year period (1986-2016)...
February 2017: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/28249268/bile-acids-in-polycystic-liver-diseases-triggers-of-disease-progression-and-potential-solution-for-treatment
#12
Maria J Perugorria, Ibone Labiano, Aitor Esparza-Baquer, Marco Marzioni, Jose J G Marin, Luis Bujanda, Jesús M Banales
Polycystic liver diseases (PLDs) are a group of genetic hereditary cholangiopathies characterized by the development and progressive growth of cysts in the liver, which are the main cause of morbidity. Current therapies are based on surgical procedures and pharmacological strategies, which show short-term and modest beneficial effects. Therefore, the determination of the molecular mechanisms of pathogenesis appears to be crucial in order to find new potential targets for pharmacological therapy. Ductal plate malformation during embryogenesis and abnormal cystic cholangiocyte growth and secretion are some of the key mechanisms involved in the pathogenesis of PLDs...
2017: Digestive Diseases
https://www.readbyqxmd.com/read/28244683/rapamycin-treatment-dose-dependently-improves-the-cystic-kidney-in-a-new-adpkd-mouse-model-via-the-mtorc1-and-cell-cycle-associated-cdk1-cyclin-axis
#13
Ao Li, Song Fan, Yuchen Xu, Jialin Meng, Xufeng Shen, Jun Mao, Li Zhang, Xiansheng Zhang, Gilbert Moeckel, Dianqing Wu, Guanqing Wu, Chaozhao Liang
Although translational research into autosomal dominant polycystic kidney disease (ADPKD) and its pathogenesis has made considerable progress, there is presently lack of standardized animal model for preclinical trials. In this study, we developed an orthologous mouse model of human ADPKD by cross-mating Pkd2 conditional-knockout mice (Pkd2(f3) ) to Cre transgenic mice in which Cre is driven by a spectrum of kidney-related promoters. By systematically characterizing the mouse model, we found that Pkd2(f3/f3) mice with a Cre transgene driven by the mouse villin-1 promoter (Vil-Cre;Pkd2(f3/f3) ) develop overt cysts in the kidney, liver and pancreas and die of end-stage renal disease (ESRD) at 4-6 months of age...
February 28, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/28237043/tuberous-sclerosis-complex-and-polycystic-kidney-disease-contiguous-gene-syndrome-with-moyamoya-disease
#14
Jonathan Lai, Lopa Modi, Daryl Ramai, Matthew Tortora
Tuberous sclerosis complex (TSC) and autosomal dominant polycystic kidney disease (ADPKD) are two diseases sharing close genetic loci on chromosome 16. Due to contiguous gene syndrome, also known as contiguous gene deletion syndrome, the proximity of TSC2 and PKD1 genes increases the risk of co-deletion resulting in a shared clinical presentation. Furthermore, Moyamoya disease (MMD) is a rare vaso-occlusive disease in the circle of Willis. We present the first case of TSC2/PKD1 contiguous gene syndrome in a patient with MMD along with detailed histopathologic, radiologic, and cytogenetic analyses...
January 3, 2017: Pathology, Research and Practice
https://www.readbyqxmd.com/read/28218410/pharmacokinetics-and-pharmacodynamics-of-tolvaptan-in-autosomal-dominant-polycystic-kidney-disease-phase-2-trials-for-dose-selection-in-the-pivotal-phase-3-trial
#15
Susan E Shoaf, Arlene B Chapman, Vicente E Torres, John Ouyang, Frank S Czerwiec
In the pivotal TEMPO 3:4 trial, the arginine vasopressin V2-receptor antagonist tolvaptan reduced the rate of kidney growth in patients with autosomal dominant polycystic kidney disease. Tolvaptan was initiated as daily morning/afternoon doses of 45/15 mg, and uptitrated weekly to 60/30 mg and 90/30 mg according to patient-reported tolerability. The current report describes 3 phase 2 trials in adult autosomal dominant polycystic kidney disease subjects that were the basis for the titrated split-dose regimen: a single ascending-dose trial (tolvaptan 15 to 120 mg; n = 11), a multiple split-dose trial (tolvaptan 15/15 mg, 30/0 mg, 30/15 mg, and 30/30 mg; n = 37), and an 8-week open-label safety and efficacy trial in 46 of the 48 subjects who participated in the prior 2 trials (tolvaptan 30/15 mg, 45/15 mg, 60/30 mg, and 90/30 mg)...
February 20, 2017: Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/28208936/supra-hepatic-inferior-vena-cava-thrombosis-surgical-challenges
#16
Senthil Muthuraman, Anand Ramamurthy, Mahesh Gopashetty, Cuddalore Sadasivam Vijayshankar, Anand Khakhar
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is a chronic affliction characterized by numerous liver and kidney cysts. There is a gradual but progressive renal and liver impairment which may require combined liver-kidney transplantation. Compression of the retrohepatic Inferior Vena Cava (IVC) by an enlarged polycystic liver may impede clear visualization on pre-operative imaging and miss an underlying thrombosis or obliteration. This may result in an intra-operative surprise. Management can be challenging requiring modification of conventional surgical approach...
December 2016: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28207635/kidney-transplantation-rates-across-glomerulonephritis-subtypes-in-the-united-states
#17
Michelle M O'Shaughnessy, Sai Liu, Maria E Montez-Rath, Richard A Lafayette, Wolfgang C Winkelmayer
BACKGROUND: Whether kidney transplantation rates differ by glomerulonephritis (GN) subtype remains largely unknown. METHODS: Using the US Renal Data System, we identified all adult patients with ESRD attributed to 1 of 6 GN subtypes who initiated dialysis in the US (1996-2013). Patients with diabetic nephropathy (DN) and autosomal-dominant polycystic kidney disease (ADPKD) served as "external" non-GN comparators. Using Cox proportional hazards regression, with death considered a competing risk, we estimated hazard ratios [HRs (95% confidence intervals)] for first kidney transplantation, controlling for year, demographics, comorbidities, socioeconomic factors, and Organ Procurement Organization (OPO)...
February 15, 2017: Transplantation
https://www.readbyqxmd.com/read/28205547/microrna-17-family-promotes-polycystic-kidney-disease-progression-through-modulation-of-mitochondrial-metabolism
#18
Sachin Hajarnis, Ronak Lakhia, Matanel Yheskel, Darren Williams, Mehran Sorourian, Xueqing Liu, Karam Aboudehen, Shanrong Zhang, Kara Kersjes, Ryan Galasso, Jian Li, Vivek Kaimal, Steven Lockton, Scott Davis, Andrea Flaten, Joshua A Johnson, William L Holland, Christine M Kusminski, Philipp E Scherer, Peter C Harris, Marie Trudel, Darren P Wallace, Peter Igarashi, Edmund C Lee, John R Androsavich, Vishal Patel
Autosomal dominant polycystic kidney disease (ADPKD) is the most frequent genetic cause of renal failure. Here we identify miR-17 as a target for the treatment of ADPKD. We report that miR-17 is induced in kidney cysts of mouse and human ADPKD. Genetic deletion of the miR-17∼92 cluster inhibits cyst proliferation and PKD progression in four orthologous, including two long-lived, mouse models of ADPKD. Anti-miR-17 treatment attenuates cyst growth in short-term and long-term PKD mouse models. miR-17 inhibition also suppresses proliferation and cyst growth of primary ADPKD cysts cultures derived from multiple human donors...
February 16, 2017: Nature Communications
https://www.readbyqxmd.com/read/28194574/tolvaptan-treatment-for-severe-neonatal-autosomal-dominant-polycystic-kidney-disease
#19
Rodney D Gilbert, Hazel Evans, Kazeem Olalekan, Arvind Nagra, Mushfequr R Haq, Mark Griffiths
BACKGROUND: Severe neonatal autosomal-dominant polycystic kidney disease (ADPKD) is rare and easily confused with recessive PKD. Managing such infants is difficult and often unsuccessful. CASE DIAGNOSIS/TREATMENT: A female infant with massive renal enlargement, respiratory compromise and hyponatraemia was treated with the arginine vasopressin receptor 2 antagonist tolvaptan. This resolved hyponatraemia, and there was no further increase in renal size. CONCLUSION: Tolvaptan may be a useful treatment for severe neonatal PKD...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28194573/hyponatremia-and-cyst-growth-in-neonatal-polycystic-kidney-disease-a-case-for-aquaretics
#20
EDITORIAL
Detlef Bockenhauer
Hyponatremia is a common complication in neonatal polycystic kidney disease and is thought to be due to water retention. Aquaretics are drugs that promote free water excretion by blocking the arginine vasopressin receptor type 2 (AVPR2) in the collecting duct and thus impair urinary concentration. AVPR2 is also a key stimulant for cyclic AMP production in the collecting duct and in this way promotes cyst proliferation and pathologic kidney growth in autosomal dominant polycystic kidney disease (ADPKD). Consequently, the aquaretic tolvaptan is now used to slow down progression of ADPKD in adult patients...
February 13, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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