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autosomal dominant polycystic kidney

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https://www.readbyqxmd.com/read/28729967/tesevatinib-ameliorates-progression-of-polycystic-kidney-disease-in-rodent-models-of-autosomal-recessive-polycystic-kidney-disease
#1
William E Sweeney, Philip Frost, Ellis D Avner
AIM: To investigate the therapeutic potential of tesevatinib (TSV), a unique multi-kinase inhibitor currently in Phase II clinical trials for autosomal dominant polycystic kidney disease (ADPKD), in well-defined rodent models of autosomal recessive polycystic kidney disease (ARPKD). METHODS: We administered TSV in daily doses of 7.5 and 15 mg/kg per day by I.P. to the well characterized bpk model of polycystic kidney disease starting at postnatal day (PN) 4 through PN21 to assess efficacy and toxicity in neonatal mice during postnatal development and still undergoing renal maturation...
July 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28724316/molecular-diagnosis-of-autosomal-dominant-polycystic-kidney-disease
#2
Xuewen Song, Amirreza Haghighi, Ioan-Andrei Iliuta, York Pei
Autosomal dominant polycystic kidney disease (ADPKD) is the most common inherited kidney disease that accounts for 5-10% of end-stage renal disease in developed countries. Mutations in PKD1 and PKD2 account for a majority of cases. Mutation screening of PKD1 is technically challenging largely due to the complexity resulting from duplication of its first 33 exons in six highly homologous pseudogenes (i.e. PKD1P1-P6). Protocol using locus-specific long-range and nested PCR has enabled comprehensive PKD1 mutation screening but is labor-intensive and costly...
July 20, 2017: Expert Review of Molecular Diagnostics
https://www.readbyqxmd.com/read/28723471/urinary-tract-infections-in-immunocompromised-patients-with-diabetes-chronic-kidney-disease-and-kidney-transplant
#3
REVIEW
Zafer Tandogdu, Tommaso Cai, Bela Koves, Florian Wagenlehner, Truls Erik Bjerklund-Johansen
CONTEXT: This paper provides a brief overview of urinary tract infections (UTIs) in immunocompromised patients from the perspective of a practicing urologist. OBJECTIVE: The primary objective was to provide an update on UTIs in immunocompromised patients. Diabetes mellitus (DM), chronic kidney disease, and kidney transplant (KT) are the most common clinical cases encountered by urologists. Diagnosis, management, and future research needs are summarised. EVIDENCE ACQUISITION: We conducted a nonsystematic review of the literature...
October 2016: European Urology Focus
https://www.readbyqxmd.com/read/28716055/temporal-and-geographical-external-validation-study-and-extension-of-the-mayo-clinic-prediction-model-to-predict-egfr-in-the-younger-population-of-swiss-adpkd-patients
#4
Laura Girardat-Rotar, Julia Braun, Milo A Puhan, Alison G Abraham, Andreas L Serra
BACKGROUND: Prediction models in autosomal dominant polycystic kidney disease (ADPKD) are useful in clinical settings to identify patients with greater risk of a rapid disease progression in whom a treatment may have more benefits than harms. Mayo Clinic investigators developed a risk prediction tool for ADPKD patients using a single kidney value. Our aim was to perform an independent geographical and temporal external validation as well as evaluate the potential for improving the predictive performance by including additional information on total kidney volume...
July 17, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28714645/-extra-renal-manifestations-of-the-autosomal-dominant-polycystic-kidney-disease
#5
Aurélie Butscher, Olivier Phan, Olivier Bonny
Extra-renal complications of autosomal dominant polycystic kidney disease (ADPKD) are often unrecognized. Liver cysts are usually benign, but may cause chronic pain, may interfere with nutrition and may necessitate partial liver resection or even liver transplant. Somatostatin analogs may be an alternative therapy. Cerebral aneurisms are rare, but devastating. They must be investigated in case of positive familial history. Cardiac valvulopathies will be searched for by echocardiography. Abdominal wall herniae are often identified during the physical exam in ADPKD patients...
February 22, 2017: Revue Médicale Suisse
https://www.readbyqxmd.com/read/28711910/combined-renin-angiotensin-aldosterone-system-blockade-and-statin-therapy-effectively-reduces-the-risk-of-cerebrovascular-accident-in-autosomal-dominant-polycystic-kidney-disease-a-nationwide-population-based-cohort-study
#6
Pei-Hsun Sung, Hsin-Ju Chiang, Mel S Lee, John Y Chiang, Hon-Kan Yip, Yao-Hsu Yang
Fairly limited data reported the incidence and risk of cerebrovascular accident (CVA) in autosomal dominant polycystic kidney disease (ADPKD). Additionally, little is known regarding the therapeutic impact of renin-angiotensin-aldosterone system (RAAS) blockade and statin on reducing the occurrence of CVA in ADPKD. We utilized the data from Taiwan National Health Insurance Research Database (NHIRD) to perform a population-based cohort study (1997-2013). A total of 2,647 patients with ADPKD were selected from 1,000,000 general population after excluding patients with age<18, renal replacement therapy and concomitant diagnosis of CVA...
June 27, 2017: Oncotarget
https://www.readbyqxmd.com/read/28709639/ganoderma-triterpenes-retard-renal-cyst-development-by-downregulating-ras-mapk-signaling-and-promoting-cell-differentiation
#7
Limin Su, Liying Liu, Yingli Jia, Lei Lei, Jiangfeng Liu, Shuai Zhu, Hong Zhou, Ruoyun Chen, Hua Ann Jenny Lu, Baoxue Yang
Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenetic disease characterized by the progressive development of renal cysts with further need for effective therapy. Here our aim was to investigate the effect of Ganoderma triterpenes (GT) on the development of kidney cysts. Importantly, GT attenuated cyst development in two mouse models of ADPKD with phenotypes of severe cystic kidney disease. Assays for tubulogenesis showed that GT promoted epithelial tubule formation in MDCK cells, suggesting a possible effect on epithelial cell differentiation...
July 11, 2017: Kidney International
https://www.readbyqxmd.com/read/28705885/fibroblast-growth-factor-23-and-kidney-disease-progression-in-autosomal-dominant-polycystic-kidney-disease
#8
Michel Chonchol, Berenice Gitomer, Tamara Isakova, Xuan Cai, Isidro Salusky, Renata Pereira, Kaleab Abebe, Vicente Torres, Theodor I Steinman, Jared J Grantham, Arlene B Chapman, Robert W Schrier, Myles Wolf
BACKGROUND AND OBJECTIVES: Increases in fibroblast growth factor 23 precede kidney function decline in autosomal dominant polycystic kidney disease; however, the role of fibroblast growth factor 23 in autosomal dominant polycystic kidney disease has not been well characterized. DESIGN, SETTING, PARTICIPANTS & MEASUREMENTS: We measured intact fibroblast growth factor 23 levels in baseline serum samples from 1002 participants in the HALT-PKD Study A (n=540; mean eGFR =91±17 ml/min per 1...
July 13, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/28701314/cux1-promotes-cell-proliferation-and-polycystic-kidney-disease-progression-in-an-adpkd-mouse-model
#9
Binu Porath, Safia Livingston, Erica L Andres, Alexandra M Petrie, Joshua C Wright, Anna E Woo, Carol G Carlton, Richard Baybutt, Gregory B Vanden Heuvel
Autosomal Dominant Polycystic Kidney Disease (ADPKD) is one of the most common monogenic hereditary disorders in humans characterized by fluid-filled cysts, primarily in the kidneys. Cux1, a cell cycle regulatory gene highly expressed during kidney development, is elevated in the cyst-lining cells of Pkd1 mutant mice, and in human ADPKD cells. However, forced expression of Cux1 is insufficient to induce cystic disease in transgenic mice, or to induce rapid cyst formation after cilia disruption in the kidneys of adult mice...
July 12, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/28685101/end-stage-kidney-failure-in-oman-an-analysis-of-registry-data-with-an-emphasis-on-congenital-and-inherited-renal-diseases
#10
Intisar Al Alawi, Issa Al Salmi, Adhra Al Mawali, Yacoub Al Maimani, John A Sayer
Globally, end-stage kidney disease (ESKD) is a huge burden on health care systems. The aims of this study were to perform a comprehensive epidemiological and etiological report of ESKD patients commencing RRT in Oman with an emphasis on genetic causes and inherited kidney disease. All newly registered Omani patients with ESKD commencing RRT from 2001 until 2015 (n = 2,922) were analysed using the RRT register in Oman. All potentially genetic or inherited causes of ESKD were reviewed. In Oman, ESKD is more prevalent in males (57...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28679364/understanding-barriers-to-medication-dietary-and-lifestyle-treatments-prescribed-in-polycystic-kidney-disease
#11
Wen-Ching Tran, David Huynh, Tea Chan, Catherine A Chesla, Meyeon Park
BACKGROUND: Autosomal dominant polycystic kidney disease (PKD) is the most common genetic renal disease and the fourth leading cause of end-stage renal disease in the United States. Although there is no cure for PKD, several treatments are considered to be beneficial, including blood pressure control, exercise, low-salt diet, and high volume water intake. However, levels of understanding of the importance of these treatments and adherence to these recommendations vary among patients. This study explores illness perception models of patients with PKD to reveal barriers in adherence to prescribed therapies; satisfaction with medical care; and sources of medical information...
July 5, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28676509/peritoneal-dialysis-for-patients-with-autosomal-dominant-polycystic-kidney-disease
#12
REVIEW
Sana Khan, Anna Giuliani, Carlo Crepaldi, Claudio Ronco, Mitchell H Rosner
End-stage renal disease secondary to autosomal dominant polycystic kidney (ADPKD) is a common issue worldwide. Peritoneal dialysis (PD) is a reasonable option for renal replacement therapy for these patients and should not be withheld due to concerns that the patient may not tolerate the fluid volumes in the peritoneal cavity. This review covers the existing data on the outcomes and complications associated with the use of PD in the polycystic kidney disease patient. In general, PD is well tolerated and outcomes in ADPKD patients are equivalent to or better than other patient groups...
July 2017: Peritoneal Dialysis International: Journal of the International Society for Peritoneal Dialysis
https://www.readbyqxmd.com/read/28676447/profiling-conserved-biological-pathways-in-autosomal-dominant-polycystic-kidney-disorder-adpkd-to-elucidate-key-transcriptomic-alterations-regulating-cystogenesis-a-cross-species-meta-analysis-approach
#13
REVIEW
Shatakshee Chatterjee, Srikant Prasad Verma, Priyanka Pandey
PURPOSE: Initiation and progression of fluid filled cysts mark Autosomal Dominant Polycystic Kidney Disease (ADPKD). Thus, improved therapeutics targeting cystogenesis remains a constant challenge. Microarray studies in single ADPKD animal models species with limited sample sizes tend to provide scattered views on underlying ADPKD pathogenesis. Thus we aim to perform a cross species meta-analysis to profile conserved biological pathways that might be key targets for therapy. METHODS: Nine ADPKD microarray datasets on rat, mice and human fulfilled our study criteria and were chosen...
July 1, 2017: Gene
https://www.readbyqxmd.com/read/28660330/quantitative-mri-of-kidneys-in-renal-disease
#14
Timothy L Kline, Marie E Edwards, Ishan Garg, Maria V Irazabal, Panagiotis Korfiatis, Peter C Harris, Bernard F King, Vicente E Torres, Sudhakar K Venkatesh, Bradley J Erickson
PURPOSE: To evaluate the reproducibility and utility of quantitative magnetic resonance imaging (MRI) sequences for the assessment of kidneys in young adults with normal renal function (eGFR ranged from 90 to 130 mL/min/1.73 m(2)) and patients with early renal disease (autosomal dominant polycystic kidney disease). MATERIALS AND METHODS: This prospective case-control study was performed on ten normal young adults (18-30 years old) and ten age- and sex-matched patients with early renal parenchymal disease (autosomal dominant polycystic kidney disease)...
June 28, 2017: Abdominal Radiology
https://www.readbyqxmd.com/read/28645206/catheter-based-renal-denervation-as-therapy-for-chronic-severe-kidney-related-pain
#15
Rosa L de Jager, Niek F Casteleijn, Esther de Beus, Michiel L Bots, Evert-Jan E Vonken, Ron T Gansevoort, Peter J Blankestijn
Background: Loin pain haematuria syndrome (LPHS) and autosomal dominant polycystic kidney disease (ADPKD) are the most important non-urological conditions to cause chronic severe kidney-related pain. Multidisciplinary programmes and surgical methods have shown inconsistent results with respect to pain reduction. Percutaneous catheter-based renal denervation (RDN) could be a less invasive treatment option for these patients. Methods: Our aim was to explore the change in perceived pain and use of analgesic medication from baseline to 3, 6 and 12 months after RDN...
June 22, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28640449/polycystic-kidney-disease
#16
Joseph Ghata, Benjamin D Cowley
Renal cysts, which arise from renal tubules, can be seen in a variety of hereditary and nonhereditary entities. Common mechanisms associated with renal cyst formation include increased cell proliferation, epithelial fluid secretion, and extracellular matrix remodeling. Hereditary polycystic kidney disease (PKD) is seen as a component of numerous diseases. Autosomal dominant (AD) PKD is the most common potentially fatal hereditary disease in humans, causes renal failure in approximately 50% of affected individuals, and accounts for approximately 5% of end stage renal disease cases in the United States...
June 18, 2017: Comprehensive Physiology
https://www.readbyqxmd.com/read/28637589/elevated-factor-h-related-protein-1-and-factor-h-pathogenic-variants-decrease-complement-regulation-in%C3%A2-iga-nephropathy
#17
Agustín Tortajada, Eduardo Gutiérrez, Elena Goicoechea de Jorge, Jaouad Anter, Alfons Segarra, Mario Espinosa, Miquel Blasco, Elena Roman, Helena Marco, Luis F Quintana, Josué Gutiérrez, Sheila Pinto, Margarita Lopez-Trascasa, Manuel Praga, Santiago Rodriguez de Córdoba
IgA nephropathy (IgAN), a frequent cause of chronic kidney disease worldwide, is characterized by mesangial deposition of galactose-deficient IgA1-containing immune complexes. Complement involvement in IgAN pathogenesis is suggested by the glomerular deposition of complement components and the strong protection from IgAN development conferred by the deletion of the CFHR3 and CFHR1 genes (ΔCFHR3-CFHR1). Here we searched for correlations between clinical progression and levels of factor H (FH) and FH-related protein 1 (FHR-1) using well-characterized patient cohorts consisting of 112 patients with IgAN, 46 with non-complement-related autosomal dominant polycystic kidney disease (ADPKD), and 76 control individuals...
June 18, 2017: Kidney International
https://www.readbyqxmd.com/read/28628430/autosomal-dominant-polycystic-kidney-disease-and-the-heart-and-brain
#18
REVIEW
Vinod Krishnappa, Poornima Vinod, Divya Deverakonda, Rupesh Raina
Autosomal dominant polycystic kidney disease (ADPKD) has numerous systemic manifestations and complications. This article gives an overview of hypertension, cardiac complications, and intracranial aneurysms in ADPKD, their pathophysiology, and recent developments in their management.
June 2017: Cleveland Clinic Journal of Medicine
https://www.readbyqxmd.com/read/28620080/the-sorting-nexin-3-retromer-pathway-regulates-the-cell-surface-localization-and-activity-of-a-wnt-activated-polycystin-channel-complex
#19
Shuang Feng, Andrew J Streets, Vasyl Nesin, Uyen Tran, Hongguang Nie, Marta Onopiuk, Oliver Wessely, Leonidas Tsiokas, Albert C M Ong
Autosomal dominant polycystic kidney disease (ADPKD) is caused by inactivating mutations in PKD1 (85%) or PKD2 (15%). The ADPKD proteins encoded by these genes, polycystin-1 (PC1) and polycystin-2 (PC2), form a plasma membrane receptor-ion channel complex. However, the mechanisms controlling the subcellular localization of PC1 and PC2 are poorly understood. Here, we investigated the involvement of the retromer complex, an ancient protein module initially discovered in yeast that regulates the retrieval, sorting, and retrograde transport of membrane receptors...
June 15, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28616208/patient-survey-of-current-water-intake-practices-in-autosomal-dominant-polycystic-kidney-disease-the-sips-survey
#20
Ragada El-Damanawi, Tess Harris, Richard N Sandford, Fiona E Karet Frankl, Thomas F Hiemstra
Background: Autosomal dominant polycystic kidney disease (ADPKD) affects 12.5 million worldwide. Vasopressin drives cysts growth and in animal models can be suppressed through high water intake. A randomized controlled trial of 'high' versus 'standard' water intake in ADPKD is essential to determine if this intervention is beneficial. We conducted an ADPKD patient survey to gain an understanding of current fluid intake practices and the design challenges of a randomized water intake trial. Methods: In collaboration with the PKD Charity, we developed and distributed an online survey to ADPKD patients over age 16 years and not on renal replacement therapy...
June 2017: Clinical Kidney Journal
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