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https://www.readbyqxmd.com/read/29675630/saikosaponin-d-inhibits-proliferation-by-up-regulating-autophagy-via-the-camkk%C3%AE-ampk-mtor-pathway-in-adpkd-cells
#1
Weiwei Shi, Dechao Xu, Junhui Gu, Cheng Xue, Bo Yang, Lili Fu, Shuwei Song, Dongmei Liu, Wei Zhou, Jiayi Lv, Ke Sun, Meihan Chen, Changlin Mei
Autosomal dominant polycystic kidney disease (ADPKD) is a common heritable human disease. Recently, the role of repressed autophagy in ADPKD has drawn increasing attention. Here, we investigate the mechanism underlying the effect of Saikosaponin-d (SSd), a sarcoplasmic/endoplasmic reticulum Ca2+ ATPase pump (SERCA) inhibitor. We show that SSd suppresses proliferation in ADPKD cells by up-regulating autophagy. We found that treatment with SSd results in the accumulation of intracellular calcium, which in turn activates the CaMKKβ-AMPK signalling cascade, inhibits mTOR signalling and induces autophagy...
April 19, 2018: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/29674338/prevalence-of-hypertension-in-children-with-early-stage-adpkd
#2
Laura Massella, Djalila Mekahli, Dušan Paripović, Larisa Prikhodina, Nathalie Godefroid, Anna Niemirska, Ayşe Ağbaş, Karolina Kalicka, Augustina Jankauskiene, Malgorzata Mizerska-Wasiak, Alberto Caldas Afonso, Rémi Salomon, Georges Deschênes, Gema Ariceta, Z Birsin Özçakar, Ana Teixeira, Ali Duzova, Jérôme Harambat, Tomáš Seeman, Gabriela Hrčková, Adrian Catalin Lungu, Svetlana Papizh, Amira Peco-Antic, Stéphanie De Rechter, Ugo Giordano, Marietta Kirchner, Teresa Lutz, Franz Schaefer, Olivier Devuyst, Elke Wühl, Francesco Emma
BACKGROUND AND OBJECTIVES: Autosomal dominant polycystic kidney disease is the most common inheritable kidney disease, frequently thought to become symptomatic in adulthood. However, patients with autosomal dominant polycystic kidney disease may develop signs or symptoms during childhood, in particular hypertension. Although ambulatory BP monitoring is the preferred method to diagnose hypertension in pediatrics, data in children with autosomal dominant polycystic kidney disease are limited...
April 19, 2018: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29664556/clinical-problems-in-hemodialysis-patients-with-autosomal-dominant-polycystic-kidney-disease
#3
REVIEW
Ismail Kocyigit, Eray Eroglu, Ozkan Gungor
Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disease characterized by massive enlargement of fluid-filled cysts in the kidney. Due to its genetic pattern, the disease differs from other CKD. ADPKD is a multi-system, progressive disorder which is frequently complicated with hypertension, cardiovascular events and cerebrovascular disease. Thus, there are many clinical problems specific to ADPKD. In this article, we reviewed these clinical problems and their management in ADPKD with hemodialysis patients...
April 17, 2018: Seminars in Dialysis
https://www.readbyqxmd.com/read/29657211/etiological-profile-of-chronic-kidney-disease-a-single-center-retrospective-hospital-based-study
#4
Manjuri Sharma, Prodip Doley, Himanab Jyoti Das
Chronic kidney disease (CKD) is one of the leading causes of chronic diseases globally, with rising incidence and prevalence. It is a major risk factor for cerebrovascular disease and coronary artery disease, which are the main causes of death in this population. The etiology of CKD is varied. This study was performed to evaluate the various etiologies of CKD among patients presenting to the Department of Nephrology, Guwahati Medical College, a tertiary referral center. A total of 5718 CKD patients were evaluated to identify the cause of CKD...
March 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29655960/systemic-redox-biomarkers-and-their-relationship-to-prognostic-risk-markers-in-autosomal-dominant-polycystic-kidney-disease-and-iga-nephropathy
#5
Ambreen Tariq, Mohammad A Mansoor, Hans-Peter Marti, Grete Jonsson, Audun Slettan, Pabasara Weeraman, Terje Apeland
BACKGROUND: Oxidative stress is evident from an early stage in chronic kidney disease (CKD). Therefore, we investigated redox biomarkers in polycystic kidney disease (ADPKD) and IgA nephropathy (IGAN). METHODS: This is a case-control study with three groups: ADPKD (n = 54), IGAN (n = 58) and healthy controls (n = 86). The major plasma aminothiols with their redox species were examined: homocysteine (Hcy), cysteinglycine (CG), cysteine (Cys) and glutathione (GSH)...
April 12, 2018: Clinical Biochemistry
https://www.readbyqxmd.com/read/29652208/age-based-ultrasonographic-criteria-for-diagnosis-of-autosomal-dominant-polycystic-kidney-disease-in-persian-cats
#6
Juliana M Guerra, Mariana F Freitas, Alexandre Gt Daniel, Arine Pellegrino, Natália C Cardoso, Isac de Castro, Luiz F Onuchic, Bruno Cogliati
Objectives The aim of this study was to establish ultrasound criteria for the diagnosis of autosomal dominant polycystic kidney disease (ADPKD) in Persian cats. Methods Eighty-two Persian cats were assessed using renal ultrasound and genotyped for the C→A transversion in exon 29 of PKD1. The animals were also submitted to hematological characterization, serum biochemistry analyses and urinalysis. Results Age, sex and neutering status did not differ between ADPKD (n = 12) and non-ADPKD (n = 70) cats. After integrated molecular genetics/ultrasonographic analysis, the presence of at least one renal cyst was sufficient to establish a diagnosis of ADPKD in animals up to 15 months of age...
April 1, 2018: Journal of Feline Medicine and Surgery
https://www.readbyqxmd.com/read/29650765/prospective-cardiovascular-genetics-evaluation-in-spontaneous-coronary-artery-dissection
#7
M Ihsan Kaadan, Carolyn MacDonald, Francesca Ponzini, Jessica Duran, Kelsey Newell, Linda Pitler, Angela Lin, Ido Weinberg, Malissa J Wood, Mark E Lindsay
BACKGROUND: Previous studies describing genetics evaluation in spontaneous coronary artery dissection (SCAD) have been retrospective in nature or presented as single case reports. As part of a dedicated clinical program, we evaluated patients in cardiovascular genetics clinic to determine the role of genetically triggered vascular disease and genetic testing in SCAD. METHODS AND RESULTS: Patient data were entered prospectively into the Massachusetts General Hospital SCAD registry database from July 2013 to September 2017...
April 2018: Circulation. Genomic and precision medicine
https://www.readbyqxmd.com/read/29644060/infected-renal-cyst-presented-with-pleural-effusion-in-a-woman-with-autosomal-dominant-polycystic-kidney-disease
#8
Ester Casillas-Sagrado, Victor Burguera, Maria Eugenia Rioja-Martín, Maite Rivera-Gorrín
We present a 64-year-old woman with autosomal dominant polycystic kidney disease and hepatic cysts admitted to our hospital for high fever, intense coughing and right abdominal pain. The chest X-ray showed right pleural effusion suggestive of pneumonia. An abdominal ultrasound and computed tomography (CT) were done but did not show evidence of cyst infection or other abdominal complications. A gallium-67-citrate single-photon emission CT/CT, a relatively cheaper technique than positron emission tomography/CT was performed...
April 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29643536/-genetic-diagnosis-of-caroli-syndrome-with-autosomal-recessive-polycystic-kidney-disease-a-case-report-and-literature-review
#9
X Y Yang, L P Zhu, X Q Liu, C Y Zhang, Y Yao, Y Wu
This case report is about one genetically specified diagnosed infant case of Caroli syndrome with autosomal recessive polycystic kidney disease (ARPKD) in China. The patient in this case report was an eight-month infant boy with an atypical onset and the main clinical manifestation was non-symptomatic enlargement of the liver and kidneys. The imaging study demonstrated a diffused cystic dilatation of intrahepatic bile ducts as well as polycystic changes in bilateral kidneys. The basic blood biochemical tests indicated a normal hepatorenal function...
April 18, 2018: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29629868/computed-tomography-identified-factors-that-preclude-living-kidney-donation
#10
Katerina Mastrocostas, Christina M Chingkoe, Kenneth T Pace, Joseph J Barfett, Anish Kirpalani, Gevork N Mnatzakanian, Paraskevi A Vlachou, Errol Colak
INTRODUCTION: The purpose of this study was to determine the variety and prevalence of renal and non-renal abnormalities detected on multi-detector computed tomography (MDCT) that precluded patients from donating a kidney. METHODS: Institutional review board approval was obtained and the requirement for informed consent was waived. A retrospective, single-centre review of 701 patients (444 female, 257 male; age range 18-86 years; mean age 43.2±11.9 years) that underwent renal donor protocol MDCT was conducted...
April 6, 2018: Canadian Urological Association Journal, Journal de L'Association des Urologues du Canada
https://www.readbyqxmd.com/read/29628139/drug-repurposing-in-kidney-disease
#11
REVIEW
Usha Panchapakesan, Carol Pollock
Drug repurposing, is the re-tasking of known medications for new clinical indications. Advantages, compared to de novo drug development, include reduced cost and time to market plus the added benefit of a known pharmacokinetic and safety profiles. Suitable drug candidates are identified through serendipitous observations, data mining, or increased understanding of disease mechanisms. This review highlights drugs suited for repurposing in kidney disease. The main cause of mortality in patients with chronic kidney disease is cardiovascular disease...
April 6, 2018: Kidney International
https://www.readbyqxmd.com/read/29623269/cystic-kidney-diseases-from-the-adult-nephrologist-s-point-of-view
#12
REVIEW
Roman-Ulrich Müller, Thomas Benzing
Cystic kidney diseases affect patients of all age groups with the onset spanning from prenatal disease to late adulthood. Autosomal-dominant polycystic kidney disease (ADPKD) is by far the most common renal cystic disease. However, there are various cystic kidney diseases, the onset of which occurs at different times in life and depends on the type of the disease and the causative genes involved. When genetic kidney diseases are discussed in the adult setting this view is usually limited on autosomal-dominant kidney disease, the most frequent genetic disorder causing adult onset ESRD...
2018: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/29622386/long-term-outcome-of-transjugular-intrahepatic-portosystemic-shunt-for-portal-hypertension-in-autosomal-recessive-polycystic-kidney-disease
#13
Sarah Verbeeck, Djalila Mekhali, David Cassiman, Geert Maleux, Peter Witters
BACKGROUND: Autosomal recessive polycystic kidney disease (ARPKD) with congenital hepatic fibrosis (CHF) causes portal hypertension and its complications. A transjugular intrahepatic portosystemic shunt (TIPSS) could serve as a symptomatic treatment for portal hypertension-related symptoms in these children. AIMS: To study the effect of TIPSS on portal hypertension, liver and kidney function and the long term complications. MATERIALS AND METHODS: We report on 5 children with CHF treated with a TIPSS to manage severe portal hypertension related symptoms...
March 15, 2018: Digestive and Liver Disease
https://www.readbyqxmd.com/read/29618028/hepatic-production-of-fibroblast-growth-factor-23-in-autosomal-dominant-polycystic-kidney-disease
#14
Frank Bienaimé, Ariane Ambolet, Béatrice Aussilhou, François Brazier, Marie Fouchard, Amandine Viau, Pauline Barre, Anne-Marie Tissier, Jean-Michel Correas, Valérie Paradis, Fabiola Terzi, Gérard Friedlander, Bertrand Knebelmann, Dominique Joly, Dominique Prié
Context: the bone-derived hormone fibroblast growth factor 23 (FGF23) controls phosphate homeostasis and urinary phosphate excretion. FGF23 plasma levels increase at the early step of renal insufficiency to prevent hyperphosphatemia. Recent evidence suggests that this increase has off-target effects on cardiac and immune cells that compromises patients' health. Subjects with autosomal dominant polycystic kidney disease (ADPKD) have been reported to display higher FGF23 concentrations than non-ADPKD patients with similar renal function...
March 29, 2018: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29617078/complete-supine-percutaneous-nephrolithotomy-with-gopro%C3%A2-ten-steps-for-success
#15
Fabio Carvalho Vicentini, Hugo Daniel Barone Dos Santos, Carlos Alfredo Batagello, Julia Rothe Amundson, Evaristo Peixoto Oliveira, Giovanni Scala Marchini, Miguel Srougi, Willian Carlos Nahas, Eduardo Mazzucchi
OBJECTIVE: To show a video of a complete supine Percutaneous Nephrolithotomy (csPCNL) performed for the treatment of a staghorn calculus, from the surgeon's point of view. The procedure was recorded with a GoPro® camera, demonstrating the ten essential steps for a successful procedure. MATERIALS AND METHODS: The patient was a 38 years-old woman with 2.4cm of left kidney lower pole stone burden who presented with 3 months of lumbar pain and recurrent urinary tract infections...
March 15, 2018: International Braz J Urol: Official Journal of the Brazilian Society of Urology
https://www.readbyqxmd.com/read/29615724/dual-mtor-pi3k-inhibition-limits-pi3k-dependent-pathways-activated-upon-mtor-inhibition-in-autosomal-dominant-polycystic-kidney-disease
#16
Yang Liu, Martin Pejchinovski, Xueqi Wang, Xuebin Fu, Deborah Castelletti, Terry J Watnick, Alexandre Arcaro, Justyna Siwy, William Mullen, Harald Mischak, Andreas L Serra
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by the development of kidney cysts leading to kidney failure in adulthood. Inhibition of mammalian target of rapamycin (mTOR) slows polycystic kidney disease (PKD) progression in animal models, but randomized controlled trials failed to prove efficacy of mTOR inhibitor treatment. Here, we demonstrate that treatment with mTOR inhibitors result in the removal of negative feedback loops and up-regulates pro-proliferative phosphatidylinositol 3-kinase (PI3K)-Akt and PI3K-extracellular signal-regulated kinase (ERK) signaling in rat and mouse PKD models...
April 3, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29606501/crispr-gene-editing-in-the-kidney
#17
Nelly M Cruz, Benjamin S Freedman
CRISPR is a nuclease guidance system that enables rapid and efficient gene editing of specific DNA sequences within genomes. We review applications of CRISPR for the study and treatment of kidney disease. CRISPR enables functional experiments in cell lines and model organisms to validate candidate genes arising from genetic studies. CRISPR has furthermore been used to establish the first models of genetic disease in human kidney organoids derived from pluripotent stem cells. These gene-edited organoids are providing new insight into the cellular mechanisms of polycystic kidney disease and nephrotic syndrome...
March 30, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29606500/preimplantation-genetic-diagnosis-counseling-in-autosomal-dominant-polycystic-kidney-disease
#18
Erin L Murphy, Madeline L Droher, Miriam S DiMaio, Neera K Dahl
Autosomal dominant polycystic kidney disease (ADPKD) is one of the most common hereditary forms of chronic kidney disease. Mutations within PKD1 or PKD2 lead to innumerable fluid-filled cysts in the kidneys and in some instances, end-stage renal disease (ESRD). Affected individuals have a 50% chance of passing the mutation to each of their offspring. Assisted reproductive technology using preimplantation genetic diagnosis (PGD) allows these individuals to reduce this risk to 1% to 2%. We assess the disease burden of 8 individuals with ADPKD who have undergone genetic testing in preparation for PGD...
March 30, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29602400/targeting-glucosylceramide-synthesis-in-the-treatment-of-rare-and-common-renal-disease
#19
REVIEW
James A Shayman
Sphingolipids, including ceramides, glycosphingolipids, sphingomyelin, and sphingosine-1-phosphate, have been recognized as important molecules that regulate critical cellular functions. Although originally studied in the context of lysosomal storage diseases, the roles of these compounds in more common disorders involving metabolism, vascular disease, and aberrant growth has been the focus of recent studies, including in disorders that affect the kidneys. These efforts have led to new insights into Fabry disease, a classic disorder of lysosomal function that results in renal failure as well as in more common renal diseases including diabetic nephropathy and polycystic kidney disease...
March 2018: Seminars in Nephrology
https://www.readbyqxmd.com/read/29600752/recent-advances-of-mtor-inhibitors-use-in-autosomal-dominant-polycystic-kidney-disease-is-the-road-still-open
#20
Pei Kou, Shuang Wei, Fei Xiong
Autosomal dominant polycystic kidney disease (ADPKD), the most common monogenic kidney disease, is caused by mutations in the PKD1, PKD2 or, in a very limited number of families, GANAB genes. Although cellular and molecular mechanisms of this disease have been understood in the past 20 years, specific therapy approaches remain very little. Both experimental and clinical studies show that the mammalian or mechanistic target of rapamycin (mTOR) pathway plays an important role during cyst formation and enlargement in ADPKD...
March 29, 2018: Current Medicinal Chemistry
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