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Polycystic kidney disease

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https://www.readbyqxmd.com/read/27913685/novel-biomarkers-in-kidney-disease-roles-for-cilia-wnt-signalling-and-atmin-in-polycystic-kidney-disease
#1
REVIEW
Paraskevi Goggolidou, Patricia D Wilson
Biomarkers, the measurable indicators of biological conditions, are fast becoming a popular approach in providing information to track disease processes that could lead to novel therapeutic interventions for chronic conditions. Inherited, chronic kidney disease affects millions of people worldwide and although pharmacological treatments exist for some conditions, there are still patients whose only option is kidney dialysis and kidney transplantation. In the past 10 years, certain chronic kidney diseases have been reclassified as ciliopathies...
December 15, 2016: Biochemical Society Transactions
https://www.readbyqxmd.com/read/27900973/autosomal-dominant-polycystic-kidney-disease-in-children
#2
Kiran Chandra Patro, R Dilip, S Ramakrishnan
Autosomal dominant polycystic kidney disease (ADPKD) presenting in adults is well documented, but the presentation in children is uncommon and is unclear why the disease presents early. Cases in children are identified usually while screening those with a strong family history and less commonly when symptomatic. We present here two children with ADPKD.
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27899417/emerging-roles-of-glis3-in-neonatal-diabetes-type-1-and-type-2-diabetes
#3
Xianjie Wen, Yisheng Yang
GLI-similar 3 (GLIS3), a member of the Krüppel-like zinc finger protein subfamily, is predominantly expressed in the pancreas, thyroid, and kidney. Glis3 mRNA can be initially detected in mouse pancreas at embryonic day 11.5 and is largely restricted to β cells, pancreatic polypeptide-expressing cells, as well as ductal cells at later stage of pancreas development. Mutations in GLIS3 cause a neonatal diabetes syndrome, characterized by neonatal diabetes, congenital hypothyroidism, and polycystic kidney. Importantly, genome-wide association studies showed that variations of GLIS3 are strongly associated with both type 1 diabetes (T1D) and type 2 diabetes (T2D) in multiple populations...
November 29, 2016: Journal of Molecular Endocrinology
https://www.readbyqxmd.com/read/27899079/vasopressin-related-copeptin-is-a-novel-predictor-of-early-endothelial-dysfunction-in-patients-with-adult-polycystic-kidney-disease
#4
Ismail Kocyigit, Mahmut Ilker Yilmaz, Ozkan Gungor, Eray Eroglu, Aydin Unal, Ozcan Orscelik, Bulent Tokgoz, Murat Sipahioglu, Ahmet Sen, Juan Jesús Carrero, Oktay Oymak, Jonas Axelsson
BACKGROUND: In this study, we examined the relative usefulness of serum copeptin levels as a surrogate marker of vasopressin (AVP) in adult polycystic kidney disease (ADPKD) by correlating it with baseline and longitudinal changes in markers of both renal function and common CVD manifestations (hypertensive vascular disease, atherosclerosis and endothelial dysfunction) that accompany the progression of this disease. METHODS: We studied a cohort of young and otherwise healthy ADPKD patients (n = 235) and measured cardiovascular function using flow-mediation dilatation (FMD), carotid intima media thickness (cIMT) and pulse wave velocity (PWV), as well as serum copeptin (commercial ELISA, a stable marker of AVP activity)...
November 30, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27891514/evidence-for-a-pathogenic-triumvirate-in-congenital-hepatic-fibrosis-in-autosomal-recessive-polycystic-kidney-disease
#5
REVIEW
Lu Jiang, Pingping Fang, James L Weemhoff, Udayan Apte, Michele T Pritchard
Autosomal recessive polycystic kidney disease (ARPKD) is a severe monogenic disorder that occurs due to mutations in the PKHD1 gene. Congenital hepatic fibrosis (CHF) associated with ARPKD is characterized by the presence of hepatic cysts derived from dilated bile ducts and a robust, pericystic fibrosis. Cyst growth, due to cyst wall epithelial cell hyperproliferation and fluid secretion, is thought to be the driving force behind disease progression. Liver fibrosis is a wound healing response in which collagen accumulates in the liver due to an imbalance between extracellular matrix synthesis and degradation...
2016: BioMed Research International
https://www.readbyqxmd.com/read/27883314/diagnostic-and-surgical-approach-to-prenatally-detected-urinary-tract-anomalies
#6
Mile Petrovski
Regular ultrasound examinations carried out in the second trimester of pregnancy help in detecting many anomalies in the fetal urinary tract. Their percentage ranges from 1% to 3% of all controlled pregnancies. There is a wide spectrum of anomalies that affect the urinary tract, but the most significant are: uretero/hydronephrosis (unilateral or bilateral), kidney agenesis, dysplastic kidney, polycystic and multicystic kidneys, anomalies of ascent, anomalies of kidney rotation or fusion, bladder exstrophy, posterior urethra valve etc...
November 1, 2016: Prilozi (Makedonska Akademija Na Naukite i Umetnostite. Oddelenie za Medicinski Nauki)
https://www.readbyqxmd.com/read/27881662/the-polycystins-are-modulated-by-cellular-oxygen-sensing-pathways-and-regulate-mitochondrial-function
#7
Valeria Padovano, Ivana Y Kuo, Lindsey K Stavola, Hans R Aerni, Benjamin J Flaherty, Hannah C Chapin, Ming Ma, Stefan Somlo, Alessandra Boletta, Barbara E Ehrlich, Jesse Rinehart, Michael J Caplan
Autosomal dominant polycystic kidney disease is caused by mutations in the genes encoding polycystin-1 (PC1) and polycystin-2 (PC2), which form an ion channel complex that may mediate ciliary sensory processes and regulate endoplasmic reticulum Ca(2+) release. Loss of PC1 expression profoundly alters cellular energy metabolism. The mechanisms that control the trafficking of PC1 and PC2 as well as their broader physiological roles remain poorly understood. We found that O2 levels regulate the subcellular localization and the channel activity of the polycystin complex through its interaction with the O2-sensing prolyl hydroxylase domain containing protein EGLN3 (or PHD3), which hydroxylates PC1...
November 23, 2016: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/27880955/long-term-angiotensin-ii-receptor-blockade-limits-hypertension-aortic-dysfunction-and-structural-remodeling-in-a-rat-model-of-chronic-kidney-disease
#8
Omar Z Ameer, Mark Butlin, Elena Kaschina, Manuela Sommerfeld, Alberto P Avolio, Jacqueline K Phillips
BACKGROUND/AIMS: Chronic kidney disease (CKD) is associated with large artery remodeling, endothelial dysfunction and calcification, with angiotensin II (Ang II) a known driver of these pathologies. We investigated long-term Ang II type 1 receptor inhibition with valsartan on aortic function and structure in the Lewis polycystic kidney (LPK) rat model of CKD. METHODS: Mixed sex LPK and Lewis control (total n = 28) treated (valsartan 60 mg/kg/day p.o. from 4 to 18 weeks) and vehicle groups were studied...
November 24, 2016: Journal of Vascular Research
https://www.readbyqxmd.com/read/27871310/transcriptome-analysis-reveals-manifold-mechanisms-of-cyst-development-in-adpkd
#9
Rita M C de Almeida, Sherry G Clendenon, William G Richards, Michael Boedigheimer, Michael Damore, Sandro Rossetti, Peter C Harris, Britney-Shea Herbert, Wei Min Xu, Angela Wandinger-Ness, Heather H Ward, James A Glazier, Robert L Bacallao
BACKGROUND: Autosomal dominant polycystic kidney disease (ADPKD) causes progressive loss of renal function in adults as a consequence of the accumulation of cysts. ADPKD is the most common genetic cause of end-stage renal disease. Mutations in polycystin-1 occur in 87% of cases of ADPKD and mutations in polycystin-2 are found in 12% of ADPKD patients. The complexity of ADPKD has hampered efforts to identify the mechanisms underlying its pathogenesis. No current FDA (Federal Drug Administration)-approved therapies ameliorate ADPKD progression...
November 21, 2016: Human Genomics
https://www.readbyqxmd.com/read/27862900/long-term-renal-allograft-survival-after-sequential-liver-kidney-transplantation-from-a-single-living-donor
#10
Kumiko Kitajima, Yuichi Ogawa, Katsuyuki Miki, Kotaro Kai, Akihito Sannomiya, Kazuhiro Iwadoh, Toru Murakami, Ichiro Koyama, Ichiro Nakajima, Shohei Fuchinoue
Background Combined liver-kidney transplantation (CLKT) is well-established as a definitive therapy with the potential to provide complete recovery for certain liver-kidney diseases, although the results might be contingent on the cause of transplantation. The purposes of the present study were to review the long-term outcome of renal allografts in CLKT patients from single living donors and to investigate the beneficial factors, compared with solitary renal transplantation. Materials and Methods Thirteen patients underwent sequential liver (LTx) and kidney (KTx) transplantations from single living donors...
November 10, 2016: Liver Transplantation
https://www.readbyqxmd.com/read/27862246/a-retrospective-analysis-of-mortality-in-captive-pygmy-hippopotamus-choeropsis-liberiensis-from-1912-to-2014
#11
Gabriella L Flacke, Suzana Tkalčić, Beatrice Steck, Kristin Warren, Graeme B Martin
The pygmy hippopotamus (Choeropsis liberiensis) is an IUCN Red List Endangered species (CITES Appendix II) that has been housed in zoological collections since 1912. As wild populations continue to decline throughout the species' range, successful ex situ breeding and management, including an understanding of morbidity and mortality, are of utmost importance. This study is the first comprehensive review of mortality data from the captive population since 1982 and significantly expands on previous analyses. We solicited necropsy reports from 129/187 zoological institutions worldwide that currently or previously held pygmy hippos and received data for 404 animals (177 ♂, 220 ♀, 7 undermined sex), representing 43% of pygmy hippos that have died in captivity...
November 2016: Zoo Biology
https://www.readbyqxmd.com/read/27861383/living-donor-renal-transplantation-in-patients-with-antiphospholipid-syndrome-a-case-report
#12
Ji Yoon Choi, Joo Hee Jung, Sung Shin, Young Hoon Kim, Duck Jong Han
INTRODUCTION: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. CLINICAL FINDINGS: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin...
November 2016: Medicine (Baltimore)
https://www.readbyqxmd.com/read/27858251/methodological-issues-in-clinical-trials-of-polycystic-kidney-disease-a-focused-review
#13
REVIEW
Ioan-Andrei Iliuta, Abhijat Kitchlu, York Pei
The field of therapeutics in autosomal dominant polycystic kidney disease (ADPKD) has seen a significant expansion recently, as major clinical trials have provided promising evidence in favor of new disease-modifying drugs. Though these trials are encouraging, limitations are noticeable in the form of methodological issues that restrict the interpretation of results. In this review, we discuss the methodological pitfalls of high-profile clinical interventional trials for ADPKD which have been published since 2009...
November 17, 2016: Journal of Nephrology
https://www.readbyqxmd.com/read/27856088/tolvaptan-and-kidney-pain-in-patients-with-autosomal-dominant%C3%A2-polycystic-kidney-disease-secondary-analysis-from%C3%A2-a-randomized-controlled-trial
#14
Niek F Casteleijn, Jaime D Blais, Arlene B Chapman, Frank S Czerwiec, Olivier Devuyst, Eiji Higashihara, Anna M Leliveld, John Ouyang, Ronald D Perrone, Vicente E Torres, Ron T Gansevoort
BACKGROUND: Kidney pain is a common complication in patients with autosomal dominant polycystic kidney disease (ADPKD), and data from the TEMPO 3:4 trial suggested that tolvaptan, a vasopressin V2 receptor antagonist, may have a positive effect on kidney pain in this patient group. Because pain is difficult to measure, the incidence of kidney pain leading to objective medical interventions was used in the present study to assess pain. STUDY DESIGN: Secondary analysis from a randomized controlled trial...
November 14, 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/27853247/six2crefrs2%C3%AE-knockout-mice-are-a-novel-model-of-renal-cystogenesis
#15
Pawan Puri, Daniel Bushnell, Caitlin M Schaefer, Carlton M Bates
Six2cre-mediated deletion of Frs2α (Six2creFrs2αKO), a major fibroblast growth factor receptor (Fgfr) docking protein in mouse nephron progenitors results in perinatal renal hypoplasia; however, postnatal Six2creFrs2αKO kidneys develop cysts. We sought to determine the pathogenesis of Six2creFrs2αKO cyst formation. We performed histological assays, Western blots, and quantitative PCR (qPCR). While embryonic day (E) 18.5 Six2Frs2αKO kidneys were hypoplastic and not cystic, postnatal day (P) 7 mutants had proximal tubular-derived cysts that nearly replaced the renal parenchyma by P21...
November 17, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27849017/elmo1-protects-renal-structure-and-ultrafiltration-in-kidney-development-and-under-diabetic-conditions
#16
Krishna Rakesh Sharma, Karl Heckler, Sandra J Stoll, Jan-Luuk Hillebrands, Katharina Kynast, Esther Herpel, Stefan Porubsky, Marlies Elger, Boris Hadaschik, Karen Bieback, Hans-Peter Hammes, Peter P Nawroth, Jens Kroll
Engulfment and cell motility 1 (ELMO1) functions as a guanine exchange factor for Rac1 and was recently found to protect endothelial cells from apoptosis. Genome wide association studies suggest that polymorphisms within human elmo1 act as a potential contributing factor for the development of diabetic nephropathy. Yet, the function of ELMO1 with respect to the glomerulus and how this protein contributes to renal pathology was unknown. Thus, this study aimed to identify the role played by ELMO1 in renal development in zebrafish, under hyperglycaemic conditions, and in diabetic nephropathy patients...
November 16, 2016: Scientific Reports
https://www.readbyqxmd.com/read/27843768/functional-alterations-due-to-amino-acid-changes-and-evolutionary-comparative-analysis-of-arpkd-and-adpkd-genes
#17
Burhan M Edrees, Mohammad Athar, Zainularifeen Abduljaleel, Faisal A Al-Allaf, Mohiuddin M Taher, Wajahatullah Khan, Abdellatif Bouazzaoui, Naffaa Al-Harbi, Ramzia Safar, Howaida Al-Edressi, Khawala Alansary, Abulkareem Anazi, Naji Altayeb, Muawia A Ahmed
A targeted customized sequencing of genes implicated in autosomal recessive polycystic kidney disease (ARPKD) phenotype was performed to identify candidate variants using the Ion torrent PGM next-generation sequencing. The results identified four potential pathogenic variants in PKHD1 gene [c.4870C > T, p.(Arg1624Trp), c.5725C > T, p.(Arg1909Trp), c.1736C > T, p.(Thr579Met) and c.10628T > G, p.(Leu3543Trp)] among 12 out of 18 samples. However, one variant c.4870C > T, p.(Arg1624Trp) was common among eight patients...
December 2016: Genomics Data
https://www.readbyqxmd.com/read/27836810/external-ca-2-regulates-polycystin-2-trpp2-cation-currents-in-llc-pk1-renal-epithelial-cells
#18
Xiao Qing Dai, Paula L Perez, Gonzalo Soria, Noelia Scarinci, Mariano Smoler, D Cristian Morsucci, Kunimasa Suzuki, María Del Rocío Cantero, Horacio F Cantiello
Polycystin-2 (PC2, TRPP2) is a nonselective cation channel whose dysfunction is associated with the onset of autosomal dominant polycystic kidney disease (ADPKD). PC2 contributes to Ca(2+) transport and cell signaling in renal epithelia and other tissues. Little is known however, as to the external Ca(2+) regulation of PC2 channel function. In this study, we explored the effect of external Ca(2+) on endogenous PC2 in wild type LLC-PK1 renal epithelial cells. We obtained whole cell currents at different external Ca(2+) concentrations, and observed that the basal whole cell conductance in normal Ca(2+)(1...
November 9, 2016: Experimental Cell Research
https://www.readbyqxmd.com/read/27835667/technical-evaluation-identification-of-pathogenic-mutations-in-pkd1-and-pkd2-in-patients-with-autosomal-dominant-polycystic-kidney-disease-by-next-generation-sequencing-and-use-of-a-comprehensive-new-classification-system
#19
Moritoshi Kinoshita, Eiji Higashihara, Haruna Kawano, Ryo Higashiyama, Daisuke Koga, Takafumi Fukui, Nobuhisa Gondo, Takehiko Oka, Kozo Kawahara, Krisztina Rigo, Tim Hague, Kiyonori Katsuragi, Kimiyoshi Sudo, Masahiko Takeshi, Shigeo Horie, Kikuo Nutahara
Genetic testing of PKD1 and PKD2 is expected to play an increasingly important role in determining allelic influences in autosomal dominant polycystic kidney disease (ADPKD) in the near future. However, to date, genetic testing is not commonly employed because it is expensive, complicated because of genetic heterogeneity, and does not easily identify pathogenic variants. In this study, we developed a genetic testing system based on next-generation sequencing (NGS), long-range polymerase chain reaction, and a new software package...
2016: PloS One
https://www.readbyqxmd.com/read/27829402/intracystic-magnetic-resonance-imaging-in-patients-with-autosomal-dominant-polycystic-kidney-disease-features-of-severe-cyst-infection-in-a-case-control-study
#20
Tatsuya Suwabe, Yoshifumi Ubara, Toshiharu Ueno, Noriko Hayami, Junichi Hoshino, Aya Imafuku, Masahiro Kawada, Rikako Hiramatsu, Eiko Hasegawa, Naoki Sawa, Satoshi Saitoh, Itsuko Okuda, Kenmei Takaichi
BACKGROUND: The purpose of this study was to investigate the usefulness of intracystic MRI features for detection of severe cyst infection that is usually refractory to antibiotic therapy alone in patients with autosomal dominant polycystic kidney disease. METHODS: Seventy-six patients (88 episodes) with positive cyst cultures treated from January 2006 to December 2013 were enrolled as the cases for this case-control study, while 147 patients who continued to attend our hospital from January 2011 to December 2013 and did not have cyst infection diagnosed during that period were enrolled as the controls...
November 9, 2016: BMC Nephrology
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