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Polycystic kidney disease

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https://www.readbyqxmd.com/read/28212688/incidence-and-survival-of-end-stage-kidney-disease-due-to-polycystic-kidney-disease-in-australia-and-new-zealand-1963-2014
#1
Mangalee R Fernando, Hannah Dent, Stephen P McDonald, Gopala K Rangan
BACKGROUND: The aim of this study was to determine whether the incidence and survival of patients with end-stage kidney disease (ESKD) due to polycystic kidney disease (PKD) has changed in Australia and New Zealand. METHODS: Data for all PKD patients who developed ESKD and commenced renal replacement therapy (RRT) was assessed using the Australia and New Zealand Dialysis and Transplant Registry from 1963 to 2014. RESULTS: A total 4678 patients with ESKD due to PKD received RRT during the study period...
February 17, 2017: Population Health Metrics
https://www.readbyqxmd.com/read/28208936/supra-hepatic-inferior-vena-cava-thrombosis-surgical-challenges
#2
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
#3
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
#4
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/28202165/ghetting-to-know-adpkd-proliferative-signaling-stat
#5
Ching-Hsien Chen, Robert H Weiss
Due to their common pathogenesis and parallel proliferative signaling pathways, the cystic diseases have been recently studied in the context of cancer biology. The present study continues this paradigm by identifying signal transducer and activator of transcription (STAT5) and growth hormone (GH) as potentially modifiable pathways in polycystic kidney disease. GH, which is a potent activator of STAT5, has the additional possibility of being a biomarker, as well as providing a potential mechanism of action of somatostatin analogs in clinical trials...
March 2017: Kidney International
https://www.readbyqxmd.com/read/28201848/new-advances-in-polycystic-liver-diseases
#6
A Santos-Laso, L Izquierdo-Sánchez, P Y Lee-Law, M J Perugorria, M Marzioni, J J G Marin, L Bujanda, J M Banales
Polycystic liver diseases (PLDs) include a heterogeneous group of congenital disorders inherited as dominant or recessive genetic traits; they are manifested alone or in association with polycystic kidney disease. Ductal plate malformation during embryogenesis and the loss of heterozygosity linked to second-hit mutations may promote the dilatation and/or development of a large number (> 20) of biliary cysts, which are the main cause of morbidity in these patients. Surgical procedures aimed to eliminate symptomatic cysts show short-term beneficial effects, but are not able to block the disease progression...
February 2017: Seminars in Liver Disease
https://www.readbyqxmd.com/read/28198143/demographics-of-polycystic-kidney-disease-and-captive-population-viability-in-pygmy-hippopotamus-choeropsis-liberiensis
#7
Gabriella L Flacke, Joseph L Tomkins, Robert Black, Beatrice Steck
Polycystic kidney disease (PKD) was previously diagnosed at necropsy in several pygmy hippopotami (Choeropsis liberiensis) from the Smithsonian National Zoo and Zoo Basel, suggesting a threat to the long-term viability of the captive population. We determined the incidence and demographics of PKD in the captive population historically; we tested if the condition is linked to pedigree; we investigated mode of inheritance; we examined effects of PKD on longevity; we conducted survival analysis; and we examined long-term population viability...
February 15, 2017: Zoo Biology
https://www.readbyqxmd.com/read/28197024/nanotechnology-in-urology
#8
REVIEW
Sudhindra Jayasimha
INTRODUCTION: Nanotechnology has revolutionized our approach to medical diagnostics as well as therapeutics and has spanned an entirely new branch of research. This review addresses the potential applications of Nanotechnology in Urology. This article is based on the Dr. Sitharaman Best Essay award of the Urological Society of India for 2016. METHODS: A PubMed search was performed for all relevant articles using the terms, "nanotechnology, nanoparticles, nanoshells, nanoscaffolds, and nanofibers...
January 2017: Indian Journal of Urology: IJU: Journal of the Urological Society of India
https://www.readbyqxmd.com/read/28195403/self-care-on-hemodialysis-behaviors-with-the-arteriovenous-fistula
#9
Clemente Neves Sousa, Patrícia Marujo, Paulo Teles, Marta Nunes Lira, Maria Eulália Leite Mota Novais
End stage renal disease (ESRD) patients should be educated to maintain and preserve the arteriovenous fistula (AVF) in the best condition. The purpose of this work was to evaluate self-care frequency and factors that influenced such frequency. A prospective study was performed in 101 hemodialysis patients. Self-care behaviors were measured with the Scale of Assessment of Self-Care Behaviours with Arteriovenous Fistula in Hemodialysis. A regression model was used to determine the relevant predictors of self-care frequency and their influence...
February 14, 2017: Therapeutic Apheresis and Dialysis
https://www.readbyqxmd.com/read/28194574/tolvaptan-treatment-for-severe-neonatal-autosomal-dominant-polycystic-kidney-disease
#10
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
#11
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
https://www.readbyqxmd.com/read/28191534/the-relation-of-anthropometric-measurements-and-insulin-resistance-in-patients-with-polycystic-kidney-disease
#12
Bennur Esen, Emel Sağlam Gokmen, Mahmut Kaya, Burak Ozkan, Ahmet Engin Atay
OBJECTIVE: To examine the frequency of insulin resistance (IR) and its relation with anthropometric measurements in patients with autosomal dominant polycystic kidney disease (ADPKD). MATERIAL AND METHODS: Nonobese 82 patients with ADPKD and 58 age matched healthy controls were enrolled into the study. None of participants were diabetic or receiving renal replacement therapies (RRT). IR was determined by homeostasis model assessment of insulin resistance (HOMA-IR) formula...
September 1, 2016: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/28191533/inhibition-of-cyst-growth-in-pck-and-wpk-rat-models-of-polycystic-kidney-disease-with-low-doses-of-peroxisome-proliferator-activated-receptor-%C3%AE-agonists
#13
Stephanie M Flaig, Vincent H Gattone, Bonnie L Blazer-Yost
BACKGROUND AND OBJECTIVES: The studies were designed to test the efficacy of two peroxisome proliferator-activated receptor γ (PPARγ) agonists in two rodent models of polycystic kidney disease (PKD). MATERIALS AND METHODS: The PCK rat is a slowly progressing cystic model while the Wpk(-/-) rat is a rapidly progressing model. PCK rats were fed with a pharmacological (0.4 mg/kg body weight [BW]) and a sub-pharmacological (0.04 mg/kg BW) dose of rosiglitazone (week 4-28)...
September 1, 2016: Journal of Translational Internal Medicine
https://www.readbyqxmd.com/read/28186577/vascular-dysfunction-in-children-and-young-adults-with-autosomal-dominant-polycystic-kidney-disease
#14
Kristen L Nowak, Heather Farmer, Melissa A Cadnapaphornchai, Berenice Gitomer, Michel Chonchol
No abstract text is available yet for this article.
February 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28185403/traf3-delays-cyst-formation-induced-by-nf-%C3%AE%C2%BAb-signaling
#15
Liping Sun, Chaofeng Hu, Xinzhou Zhang
This study aims to investigate the effects of TNF receptors associated factor 3 (TRAF3) on the signaling pathway and expression of downstream products of nuclear factor kappa B (NF-κB) in the epithelial cells of renal ducts in individuals with polycystic kidney disease (PKD). We observe the TRAF3 genic overexpression of the epithelial cells, which form a tubular branch structure, in polycystic kidneys and to explore the protective effect of TRAF3 on the cystogenesis and progression of PKD. Western blotting analysis was conducted to examine the signaling changes of NF-κB in PKD the epithelial cells and TRAF3 transgenic PKD epithelial cells...
February 10, 2017: IUBMB Life
https://www.readbyqxmd.com/read/28179401/high-serum-soluble-cd200-levels-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#16
Funda Sari, Saadet Gumuslu, Ramazan Cetinkaya, Metin Sarikaya, Arzu Didem Yalcin
CD200 is a novel immune-effective molecule, existing in a cell membrane-bound form, as well as in a soluble form in serum, which performs to modulate inflammatory and acquired immune responses. Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of large renal cysts and progressive loss of renal function. As defects in cell cycle arrest and apoptosis of renal tubular epithelial cells occur in ADPKD, we asked whether serum soluble CD200 might underlie and effect on ADPKD...
February 8, 2017: Journal of Investigative Medicine: the Official Publication of the American Federation for Clinical Research
https://www.readbyqxmd.com/read/28177097/-spontaneous-rupture-of-a-simple-renal-cyst-clinical-management
#17
Luca Estienne, Maria Valentina Domenech, Elena Caramella, Marta Calatroni, Alessandro Avella, Ilaria Massa, Stefania Bianzina, Nicoletta Serpieri, Pasquale Esposito
Spontaneous renal bleeding mainly occurs in patients with polycystic kidney diseases or cancer. Indeed, despite the high prevalence of simple cysts, their spontaneous atraumatic rupture is a rare event. Underlying mechanisms may involve the increase of intracystic pressure and/or the development of a haemorrhage into the cyst. Management of this condition includes surgery, interventional radiology or conservative strategies. Here, we report a case of spontaneous rupture of a simple renal cyst, successfully managed with conservative treatment...
January 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28168444/fluid-shear-stress-induced-tgf-%C3%AE-alk5-signaling-in-renal-epithelial-cells-is-modulated-by-mek1-2
#18
Steven J Kunnen, Wouter N Leonhard, Cor Semeins, Lukas J A C Hawinkels, Christian Poelma, Peter Ten Dijke, Astrid Bakker, Beerend P Hierck, Dorien J M Peters
Renal tubular epithelial cells are exposed to mechanical forces due to fluid flow shear stress within the lumen of the nephron. These cells respond by activation of mechano-sensors located at the plasma membrane or the primary cilium, having crucial roles in maintenance of cellular homeostasis and signaling. In this paper, we applied fluid shear stress to study TGF-β signaling in renal epithelial cells with and without expression of the Pkd1-gene, encoding a mechano-sensor mutated in polycystic kidney disease...
February 6, 2017: Cellular and Molecular Life Sciences: CMLS
https://www.readbyqxmd.com/read/28166521/rationale-and-design-of-a-clinical-trial-investigating-tolvaptan-safety-and-efficacy-in-autosomal-dominant-polycystic-kidney-disease
#19
Vicente E Torres, Olivier Devuyst, Arlene B Chapman, Ron T Gansevoort, Ronald D Perrone, John Ouyang, Jaime D Blais, Frank S Czerwiec, Olga Sergeyeva
BACKGROUND: In TEMPO 3:4, the vasopressin V2-receptor antagonist tolvaptan slowed kidney growth and function decline in autosomal dominant polycystic kidney disease (ADPKD) patients with relatively preserved kidney function. METHODS: Prospective, phase 3b, multi-center, randomized-withdrawal, placebo-controlled, double-blind trial of tolvaptan in ADPKD patients with late stage 2 to early stage 4 chronic kidney disease (CKD). The primary endpoint was estimated glomerular filtration rate (eGFR) change from pre-treatment baseline to post-treatment follow-up...
February 7, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28159317/novel-treatment-protocol-for-ameliorating-refractory-chronic-pain-in-patients-with-autosomal-dominant-polycystic-kidney-disease
#20
Niek F Casteleijn, Maatje D A van Gastel, Peter J Blankestijn, Joost P H Drenth, Rosa L de Jager, Anna M Leliveld, Ruud Stellema, Andreas P Wolff, Gerbrand J Groen, Ron T Gansevoort
Autosomal dominant polycystic kidney disease (ADPKD) patients can suffer from chronic pain that can be refractory to conventional treatment, resulting in a wish for nephrectomy. This study aimed to evaluate the effect of a multidisciplinary treatment protocol with sequential nerve blocks on pain relief in ADPKD patients with refractory chronic pain. As a first step a diagnostic, temporary celiac plexus block with local anesthetics was performed. If substantial pain relief was obtained, the assumption was that pain was relayed via the celiac plexus and major splanchnic nerves...
January 31, 2017: Kidney International
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