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Polycystic kidney disease

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https://www.readbyqxmd.com/read/29331360/fully-automated-segmentation-of-polycystic-kidneys-from-noncontrast-computed-tomography-a-feasibility-study-and-preliminary-results
#1
Dario Turco, Maddalena Valinoti, Eva Maria Martin, Carlo Tagliaferri, Francesco Scolari, Cristiana Corsi
RATIONALE AND OBJECTIVES: Total kidney volume is an important biomarker for the evaluation of autosomal dominant polycystic kidney disease progression. In this study, we present a novel approach for automated segmentation of polycystic kidneys from non-contrast-enhanced computed tomography (CT) images. MATERIALS AND METHODS: Non-contrast-enhanced CT images were acquired from 21 patients with a diagnosis of autosomal dominant polycystic kidney disease. Kidney volumes obtained from the fully automated method were compared to volumes obtained by manual segmentation and evaluated using linear regression and Bland-Altman analyses...
January 10, 2018: Academic Radiology
https://www.readbyqxmd.com/read/29323708/microrna501-5p-induces-p53-proteasome-degradation-through-the-activation-of-the-mtor-mdm2-pathway-in-adpkd-cells
#2
Lucia de Stephanis, Alessandra Mangolini, Miriam Servello, Peter C Harris, Lucio Dell'Atti, Paolo Pinton, Gianluca Aguiari
Cell proliferation and apoptosis are typical hallmarks of autosomal dominant polycystic kidney disease (ADPKD) and cause the development of kidney cysts that lead to end-stage renal disease (ESRD). Many factors, impaired by polycystin complex loss of function, may promote these biological processes, including cAMP, mTOR and EGFR signalling pathways. In addition, microRNAs (miRs) may also regulate the ADPKD related signalling network and their dysregulation contributes to disease progression. However, the role of miRs in ADPKD pathogenesis has not been fully understood, but also the function of p53 is quite obscure, especially its regulatory contribution on cell proliferation and apoptosis...
January 11, 2018: Journal of Cellular Physiology
https://www.readbyqxmd.com/read/29322327/combined-and-sequential-liver-kidney-transplantation-in-children
#3
Ryszard Grenda, Piotr Kaliciński
Combined and sequential liver-kidney transplantation (CLKT and SLKT) is a definitive treatment in children with end-stage organ failure. There are two major indications: - terminal insufficiency of both organs, or - need for transplanting new liver as a source of lacking enzyme or specific regulator of the immune system in a patient with renal failure. A third (uncommon) option is secondary end-stage renal failure in liver transplant recipients. These three clinical settings use distinct qualification algorithms...
January 10, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29321346/mutational-screening-of-pkd2-gene-in-the-north-indian-polycystic-kidney-disease-patients-revealed-28-genetic-variations
#4
Sonam Raj, Rana Gopal Singh, Parimal Das
Polycystic kidney disease (PKD) is a systemic disorder which adds majority of renal patients to end stage renal disease. Autosomal dominant polycystic kidney disease (ADPKD) is more prevalent and leading cause of dialysis and kidney transplant. Linkage analysis revealed some closely linked loci, two of which are identified as PKD1, PKD2 and an unidentified locus to ADPKD. This study was performed using PCR and automated DNA sequencing in 84 cases and 80 controls to test potential candidature of PKD2 as underlying cause of PKD by in silico and statistical analyses...
December 2017: Journal of Genetics
https://www.readbyqxmd.com/read/29309655/european-adpkd-forum-multidisciplinary-position-statement-on-autosomal-dominant-polycystic-kidney-disease-care-european-adpkd-forum-and-multispecialist-roundtable-participants
#5
Tess Harris, Richard Sandford, Brenda de Coninck, Olivier Devuyst, Joost P H Drenth, Tevfik Ecder, Alastair Kent, Ron T Gansevoort, José Luis Górriz, Albert C M Ong, Yves Pirson, Vicente E Torres, Klemens Budde, Denis Clément, Lorenzo E Derchi, Marianna Eleftheroudi, Elena Levtchenko, Dorien Peters, Hendrik Van Poppel, Raymond Vanholder
Autosomal dominant polycystic kidney disease (ADPKD) is a chronic, progressive condition characterized by the development and growth of cysts in the kidneys and other organs and by additional systemic manifestations. Individuals with ADPKD should have access to lifelong, multidisciplinary, specialist and patient-centred care involving: (i) a holistic and comprehensive assessment of the manifestations, complications, prognosis and impact of the disease (in physical, psychological and social terms) on the patient and their family; (ii) access to treatment to relieve symptoms, manage complications, preserve kidney function, lower the risk of cardiovascular disease and maintain quality of life; and (iii) information and support to help patients and their families act as fully informed and active partners in care, i...
December 22, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29306987/3dus-as-an-alternative-to-mri-for-measuring-renal-volume-in-children-with-autosomal-dominant-polycystic-kidney-disease
#6
Luc Breysem, Stéphanie De Rechter, Frederik De Keyzer, Maria Helena Smet, Bert Bammens, Maria Van Dyck, Maarten Hofmans, Raymond Oyen, Elena Levtchenko, Djalila Mekahli
BACKGROUND: Total kidney volume, measured by magnetic resonance imaging (MRI), is a validated disease progression marker in adults with autosomal dominant polycystic kidney disease (ADPKD). However, in childhood, MRI is burdensome, explaining the need for alternatives. METHODS: Kidney volume (KV) was evaluated in 30 children with ADPKD, using three-dimensional ultrasound (3DUS), applying the ellipsoid method and manual contouring (KV3DUS-ellipsoid, KV3DUS-contour respectively); manual contouring on MRI (KVMRI), and the ellipsoid method on two-dimensional ultrasound (2DUS, KV2DUS)...
January 6, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29306857/colovesical-fistula-a-rare-complication-after-renal-transplantation
#7
Arunkumar Subbiah, Sandeep Mahajan, Raj Kanwar Yadav, Sanjay Kumar Agarwal
Colovesical fistula per se is a rare condition and most commonly occurs secondary to diverticular disease in normal patients. Colovesical fistula in the setting of post-renal transplantation is even rarer and very few cases have been reported in literature. Patients with autosomal-dominant polycystic kidney disease (ADPKD) are predisposed to diverticulosis and hence are at a higher risk for fistula formation. Herein, we report a case of colovesical fistula in a renal allograft recipient with ADPKD in the absence of diverticulosis...
January 6, 2018: BMJ Case Reports
https://www.readbyqxmd.com/read/29306517/patterns-of-kidney-function-decline-in-autosomal-dominant-polycystic-kidney-disease-a-post-hoc-analysis-from-the-halt-pkd-trials
#8
Godela M Brosnahan, Kaleab Z Abebe, Charity G Moore, Frederic F Rahbari-Oskoui, Kyongtae T Bae, Jared J Grantham, Robert W Schrier, William E Braun, Arlene B Chapman, Michael F Flessner, Peter C Harris, Marie C Hogan, Ronald D Perrone, Dana C Miskulin, Theodore I Steinman, Vicente E Torres
BACKGROUND: Previous clinical studies of autosomal dominant polycystic kidney disease (ADPKD) reported that loss of kidney function usually follows a steep and relentless course. A detailed examination of individual patterns of decline in estimated glomerular filtration rate (eGFR) has not been performed. STUDY DESIGN: Longitudinal post hoc analysis of data collected during the Halt Progression of Polycystic Kidney Disease (HALT-PKD) trials. SETTING & PARTICIPANTS: 494 HALT-PKD Study A participants (younger; preserved eGFR) and 435 Study B participants (older; reduced eGFR) who had more than 3 years of follow-up and 7 or more eGFR assessments...
January 3, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29290310/baseline-total-kidney-volume-and-the-rate-of-kidney-growth-are-associated-with-chronic-kidney-disease-progression-in-autosomal-dominant-polycystic-kidney-disease
#9
Alan S L Yu, Chengli Shen, Douglas P Landsittel, Peter C Harris, Vicente E Torres, Michal Mrug, Kyongtae T Bae, Jared J Grantham, Frederic F Rahbari-Oskoui, Michael F Flessner, William M Bennett, Arlene B Chapman
Autosomal dominant polycystic kidney disease (ADPKD) is characterized by progressive enlargement of kidney cysts leading to chronic kidney disease (CKD) and end-stage renal disease (ESRD). Identification of an early biomarker that can predict progression of CKD is urgently needed. In an earlier Consortium for Radiologic Imaging Studies of Polycystic Kidney Disease (CRISP) study (a prospective, multicenter, observational analysis of 241 patients with ADPKD initiated in 2000), baseline height-adjusted total kidney volume (htTKV) was shown to be associated with development of CKD stage 3 after eight years of follow-up...
December 28, 2017: Kidney International
https://www.readbyqxmd.com/read/29262409/early-markers-of-cardiovascular-risk-in-autosomal-dominant-polycystic-kidney-disease
#10
Silvia Lai, Daniela Mastroluca, Silvia Matino, Valeria Panebianco, Antonio Vitarelli, Lidia Capotosto, Irene Turinese, Paolo Marinelli, Marco Rossetti, Alessandro Galani, Pia Baiocchi, Anna R D'Angelo, Paolo Palange
BACKGROUND/AIMS: Cardiovascular disease is the most frequent cause of morbidity and mortality in autosomal dominant polycystic kidney disease (ADPKD) patients, often before the onset of renal failure, and the pathogenetic mechanism is not yet well elucidated. The aim of the study was to identify early and noninvasive markers of cardiovascular risk in young ADPKD patients, in the early stages of disease. METHODS: A total of 26 patients with ADPKD and 24 control group, matched for age and sex, were enrolled, and we have assessed inflammatory indexes, mineral metabolism, metabolic state and markers of atherosclerosis and endothelial dysfunction (carotid intima media thickness (IMT), ankle brachial index (ABI), flow mediated dilation (FMD), renal resistive index (RRI), left ventricular mass index (LVMI)) and cardiopulmonary exercise testing (CPET), maximal O2 uptake (V'O2max), and O2 uptake at lactic acid threshold (V'O2@LT)...
December 15, 2017: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29241197/renoprotective-effects-of-metformin
#11
Marc E De Broe, Farshad Kajbaf, Jean-Daniel Lalau
BACKGROUND/AIMS: It has become clear that metformin exerts pleiotropic actions beyond its glucose-lowering agent effect. In this review, we summarise the state of the art concerning the potential renoprotective effects of metformin in vitro, animal models and clinical nephrology. METHODS: A literature search was performed in PUBMED, ScienceDirect, between January 1957 and March 2017 using the following keywords: "metformin," "nephroprotection," "renoprotection," "survival," "renal failure," "chronic kidney diseases," "fibrosis," "polycystic kidney disease" and "microalbuminuria...
December 14, 2017: Nephron
https://www.readbyqxmd.com/read/29234037/dynamic-regulation-of-trek1-gating-by-polycystin-2-via-a-filamin-a-mediated-cytoskeletal-mechanism
#12
Steven Li Fraine, Amanda Patel, Fabrice Duprat, Reza Sharif-Naeini
Mechanosensing is essential for several physiological functions including touch and pain sensations, osmoregulation, and controlling the myogenic tone of resistance arteries. Understanding how mechanosensitive ion channels (MSCs) are gated can provide important information regarding these processes. We have previously demonstrated that during pathological conditions such as polycystic kidney disease, polycystin 2 (TRPP2) inhibits the activity of potassium-selective MSCs through a filamin A-mediated cytoskeletal effect, and renders tubular epithelial cells susceptible to apoptosis...
December 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29230688/therapeutic-effects-of-histone-deacetylase-inhibitors-on-kidney-disease
#13
REVIEW
Pusoon Chun
Increasing evidence has shown the involvement of histone deacetylases (HDACs) in the development and progression of various renal diseases, highlighting its inhibition as a promising therapeutic strategy to prevent kidney diseases. Accordingly, numerous studies have shown that HDAC inhibitors protect the kidneys from various diseases through their effects on multiple pathways, such as suppression of transforming growth factor-β signaling pathway and nuclear factor-κB signaling pathways, augmentation of apoptosis, and inhibition of angiogenesis...
December 11, 2017: Archives of Pharmacal Research
https://www.readbyqxmd.com/read/29230552/identification-of-clustered-phosphorylation-sites-in-pkd2l1-how-pkd2l1-channel-activation-is-regulated-by-cyclic-adenosine-monophosphate-signaling-pathway
#14
Eunice Yon June Park, Misun Kwak, Kotdaji Ha, Insuk So
Polycystic kidney disease 2-like-1 (PKD2L1), or polycystin-L or TRPP2, formerly TRPP3, is a transient receptor potential (TRP) superfamily member. It is a calcium-permeable non-selective cation channel that regulates intracellular calcium concentration and thereby calcium signaling. PKD2L1 has been reported to take part in hedgehog signaling in renal primary cilia and sour tasting coupling with PKD1L3. In addition to the previous reports, PKD2L1 is recently found to play a crucial role in localization with β2-adrenergic receptor (β2AR) on the neuronal primary cilia...
December 11, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/29226648/fibulin-levels-in-autosomal-dominant-polycystic-kidney-disease-and-its-relationship-to-arterial-stiffness
#15
A Inci, O Akin, H Y Ellidag, E Eren, S Dolu, F Sari
BACKGROUND: The objective of the present study was to evaluate fibulin 1 levels in different stages of patients with autosomal dominant polycystic kidney disease (ADPKD) and investigate possible connections between fibulin-1 and arterial stiffness. METHODS: For this cross-sectional study, we included 74 patients with ADPKD (mean age, 50.92 ± 15.70 years) and 32 healthy controls (mean age, 49.53 ± 7.32 years). Patients with ADPKD were classified based on CKD epidemiology collaboration (CKD-EPI) equation assessments of estimated glomerular filtration rate (eGFR)...
November 1, 2017: Clinical Laboratory
https://www.readbyqxmd.com/read/29225875/an-88-year-old-patient-with-adpkd-underscoring-the-importance-of-risk-factor-modification
#16
Abhilash Koratala, Freddy R Malpartida, Amir Kazory
The mean age of onset of end-stage renal disease (ESRD) in autosomal dominant polycystic kidney disease (ADPKD) is between 56 and 69. Our case represents one of the oldest patients with ADPKD, whose chronic kidney disease (CKD) progression rate could be successfully reduced by optimal risk factor modification.
December 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/29224797/kidney-transplant-with-and-without-native-nephrectomy-for-polycystic-kidney-disease-results-of-the-national-inpatient-sample-and-the-rationale-for-a-2-staged-procedure
#17
Raymond A Jean, Mehida Alexandre, Peter S Yoo
BACKGROUND: Polycystic kidney disease (PKD) is one of the most common causes of end-stage renal disease requiring hemodialysis or transplantation. In patients requiring transplant, there are several indications for native nephrectomy, including recurrent cyst infection, bleeding, or to provide room for the graft. There is disagreement about whether it is advisable to perform kidney transplant alone (KT), or to perform kidney transplant with simultaneous native nephrectomy (KTN). We compared postoperative outcomes of KTN and KT in a large national cohort...
December 7, 2017: Journal of the American College of Surgeons
https://www.readbyqxmd.com/read/29217884/how-harmful-can-herbal-remedies-be-a-case-of-severe-acute-tubulointerstitial-nephritis
#18
P Beniwal, N Gaur, S K Singh, N Raveendran, V Malhotra
Acute interstitial nephritis (AIN) is a condition in which acute kidney injury (AKI) is characterized by the histological finding of interstitial inflammation. Hyponidd is an ayurvedic drug containing Momordica charantia, Gymnema sylvestre, Swertia chirata, etc., used for the treatment of Type 2 diabetes mellitus (DM) and polycystic ovarian disease as an insulin sensitizer. There are no case reports of AIN caused by this drug yet. We report a biopsy-proven case of AKI due to severe AIN associated with the use of hyponidd tablet in a 60-year-old male with DM and hypertension...
November 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/29215110/quercetin-inhibits-renal-cyst-growth-in-vitro-and-via-parenteral-injection-in-a-polycystic-kidney-disease-mouse-model
#19
Yangyang Zhu, Tian Teng, Hu Wang, Hao Guo, Lei Du, Baoxue Yang, Xiaoxing Yin, Ying Sun
Autosomal dominant polycystic kidney disease (ADPKD) is a common monogenic disease characterized by massive enlargement of fluid-filled cysts in the kidney. There is an urgent need to develop effective ADPKD therapies. We used an in vitro Madin-Darby canine kidney (MDCK) cyst model and a murine embryonic kidney cyst model to evaluate whether quercetin inhibits cyst development. We then used a polycystic kidney disease (PKD) mouse model to further determine the in vivo effects of quercetin (100 mg per kg body weight twice per day) on PKD mice via subcutaneous injections...
December 7, 2017: Food & Function
https://www.readbyqxmd.com/read/29208861/etiology-of-early-renal-allograft-dysfunction-and-comparison-between-dysfunction-and-function-group-a-single-center-study
#20
K M Hadiuzzaman, S F Islam, M O Faroque, R M Hossain, M Munirunnessa, S I Selim, M Morshed
Over a period of two years thirty five renal allograft recipients & donors were evaluated to find out the aetiology of early renal allograft dysfunction, in the Department of Nephrology, Bangabandhu Sheikh Mujib Medical University, Dhaka, Bangladesh from March 2010 to February 2012. A comparison was made between dysfunction & functioning graft group. Mean age of recipients were (36.4±9.4) years, mean age of donors were (41.7±8.3) years, with a male and female ratio of 3:1. Fifty percent recipients showed one heliotype match, ninety percent recipients were anti CMV antibody IgG positive, few were anti CMV antibody IgM positive...
October 2017: Mymensingh Medical Journal: MMJ
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