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Chronic kidney polycystic

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https://www.readbyqxmd.com/read/29786190/-identification-of-a-new-mutation-of-the-nphp1-gene
#1
Antonella La Russa, Rosa Anna Cifarelli, Anna Perri, Angelo Saracino, Giovanni Santarsia, Renzo Bonofiglio
Kidney cystic diseases are inherited disorders causing chronic renal failure. According to the genetic defect they are classified as diseases of the primary ciliary complex and uromodulin-associated diseases. Mutations in genes coding for ciliary proteins are the basis of a broad category of genetic diseases, called ciliopathies. To date, three important ciliopathies are known: the autosomal dominant form and the recessive shape of the polycystic kidney and the nephronophthisis (NPHP). Juvenile Nephronophthisis (NPHP) is a progressive renal tubulo-interstitial disorder with a form of autosomal recessive inheritance that progresses inexorably towards terminal renal failure...
May 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/29782632/prevalence-of-clinically-validated-primary-causes-of-end-stage-renal-disease-esrd-in-a-state-capital-in-northeastern-brazil
#2
Luana Rodrigues Sarmento, Paula Frassinetti Castelo Branco Camurça Fernandes, Marcelo Ximenes Pontes, Daniel Barros Santos Correia, Victhor Castelo Branco Chaves, Cecília Ferreira de Araújo Carvalho, Tiago Lima Arnaud, Matheus Henrique Seixas Dos Santos, Livia Cristina Barros Barreto, Larissa Alves Alexandre Moliterno
INTRODUCTION: Knowledge of validated primary causes of end-stage renal disease (ESRD) is extremely relevant in the realm of public health. The literature lacks validated studies on the primary causes of ESRD. OBJECTIVE: The aim of this study was to estimate the prevalence of the causes of ESRD in a State Capital in Northeastern Brazil. METHODS: This cross-sectional study was based on the analysis of medical records of patients on hemodialysis at five specialized centers in Fortaleza, CE, Brazil...
May 17, 2018: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29780607/renal-cell-carcinoma-in-a-cat-with-polycystic-kidney-disease-undergoing-renal-transplantation
#3
Daniel J Adams, Jolie A Demchur, Lillian R Aronson
Case summary: A 10-year-old spayed female American Shorthair cat underwent renal transplantation due to worsening chronic kidney disease secondary to polycystic kidney disease. During transplantation, the right kidney grossly appeared to be more diseased than the left and was firmly adhered to the surrounding tissues. An intraoperative fine-needle aspirate of the right native kidney revealed inflammatory cells but no evidence of neoplasia. To create space for the allograft, a right nephrectomy was performed...
January 2018: JFMS Open Reports
https://www.readbyqxmd.com/read/29774463/early-cardiovascular-manifestations-in-children-and-adolescents-with-autosomal-dominant-polycystic-kidney-disease-a-single-center-study
#4
Vasiliki Karava, Cherine Benzouid, Julien Hogan, Claire Dossier, André Pierre Denjean, Georges Deschênes
BACKGROUND: This study aims to describe the cardiovascular manifestations in children with autosomal dominant polycystic kidney disease (ADPKD) and detect their relation with kidney disease and type of gene mutation. METHODS: Twenty-one patients (7 to 19 years old) were included. Cardiovascular evaluation involved blood pressure (BP), indexed left ventricular mass (LVMI), pulse wave velocity (PWV), and carotid intima media thickness (cIMT) measurement. Patients were classified according to percentile reference values of these parameters in healthy children...
May 17, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29767399/a-decreased-soluble-klotho-level-with-normal-egfr-fgf23-serum-phosphate-and-fep-in-an-adpkd-patient-with-enlarged-kidneys-due-to-multiple-cysts
#5
Takahiro Kanai, Kazuhiro Shiizaki, Hiroyuki Betsui, Jun Aoyagi, Takanori Yamagata
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary renal disorder. ADPKD is characterized clinically by the presence of multiple bilateral renal cysts that lead to chronic renal failure. The cysts evolve from renal tubular epithelial cells that express the Klotho gene. Notably, Klotho acts as a co-receptor for fibroblast growth factor 23 (FGF23); in this context, it induces phosphaturia and maintains serum phosphate at a normal level. Many reports have shown that decreases in the soluble Klotho level and increases in the FGF23 level are associated with glomerular filtration rate (GFR) decline, but a recent study observed these changes in patient with normal eGFR...
May 16, 2018: CEN Case Reports
https://www.readbyqxmd.com/read/29763910/different-relevance-of-peripheral-central-or-nighttime-blood-pressure-measurements-in-the-prediction-of-chronic-kidney-disease-progression-in-patients-with-mild-or-no-proteinuria
#6
Piotr Kuczera, Katarzyna Kwiecień, Marcin Adamczak, Teresa Bączkowska, Jolanta Gozdowska, Katarzyna Madziarska, Hanna Augustyniak-Bartosik, Marian Klinger, Magdalena Durlik, Eberhard Ritz, Andrzej Wiecek
BACKGROUND/AIMS: Arterial hypertension is one of the leading factors aggravating the course of chronic kidney disease (CKD). It seems that the novel parameters used in the assessment of the blood pressure (BP) load (i.e. central blood pressure, nighttime blood pressure) may be more precise in predicting the cardiovascular risk and the progression of CKD in comparison with the traditional peripheral blood pressure measurements in the office conditions. The aim of the study was to assess the impact of the central, or nighttime blood pressure on the progression of CKD in patients with mild or no-proteinuria (autosomal, dominant polycystic kidney disease or IgA nephropathy)...
May 10, 2018: Kidney & Blood Pressure Research
https://www.readbyqxmd.com/read/29725632/urinary-biomarkers-to-identify-autosomal-dominant-polycystic-kidney-disease-patients-with-a-high-likelihood-of-disease-progression
#7
A Lianne Messchendorp, Esther Meijer, Wendy E Boertien, Gerwin E Engels, Niek F Casteleijn, Edwin M Spithoven, Monique Losekoot, Johannes G M Burgerhof, Dorien J M Peters, Ron T Gansevoort
Introduction: The variable disease course of autosomal dominant polycystic kidney disease (ADPKD) makes it important to develop biomarkers that can predict disease progression, from a patient perspective and to select patients for renoprotective treatment. We therefore investigated whether easy-to-measure urinary biomarkers are associated with disease progression and have additional value over that of conventional risk markers. Methods: At baseline, inflammatory, glomerular, and tubular damage markers were measured in 24-hour urine collections (albumin, IgG, kidney injury molecule-1 (KIM-1), N -acetyl-β-d-glucosaminidase (NAG), β2 microglobulin (β2MG), heart-type fatty acid binding protein (HFABP), macrophage migration inhibitory factor (MIF), neutrophil gelatinase-associated lipocalin (NGAL), and monocyte chemotactic protein-1 (MCP-1)...
March 2018: KI Reports
https://www.readbyqxmd.com/read/29707627/identifying-barriers-to-preemptive-kidney-transplantation-in-a-living-donor-transplant-cohort
#8
Ryan A Helmick, Colleen L Jay, Brittany A Price, Patrick G Dean, Mark D Stegall
Background: Despite substantial evidence demonstrating clear benefit, rates of preemptive kidney transplantation (PreKTx) remain low in the United States. Our goal was to identify barriers to PreKTx. Methods: Using a telephone-administered questionnaire including questions about barriers, timing of referral, timing of education, we retrospectively studied first living donor kidney transplant recipients (2006-2010) at Mayo Clinic, Rochester, MN. Of 235 patients, 145 (62%) responded to the questionnaire (74 PreKTx and 71 non-PreKTx)...
April 2018: Transplantation Direct
https://www.readbyqxmd.com/read/29657211/etiological-profile-of-chronic-kidney-disease-a-single-center-retrospective-hospital-based-study
#9
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
#10
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/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/29617078/complete-supine-percutaneous-nephrolithotomy-with-gopro%C3%A2-ten-steps-for-success
#12
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/29606500/preimplantation-genetic-diagnosis-counseling-in-autosomal-dominant-polycystic-kidney-disease
#13
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/29564978/determinants-of-progression-in-early-autosomal-dominant-polycystic-kidney-disease-is-it-blood-pressure-or-renin-angiotensin-aldosterone-system-blockade
#14
Godela M Brosnahan, Kaleab Z Abebe, Charity G Moore, Kyongtae T Bae, William E Braun, Arlene B Chapman, Michael F Flessner, Peter C Harris, Marie C Hogan, Ronald D Perrone, Frederic F Rahbari-Oskoui, Theodore I Steinman, Vicente E Torres, The Halt Pkd Investigators
BACKGROUND: The HALT PKD trial in early autosomal dominant polycystic kidney disease (ADPKD) showed that intensive control of systolic blood pressure to 95-110 mmHg was associated with a 14% slower rate of kidney volume growth compared to standard control. It is unclear whether this result was due to greater blockade of the renin-angiotensin-aldosterone system (RAAS) by allowing the use of higher drug doses in the low blood pressure arm, or due to the lower blood pressure per se. METHODS: In this secondary analysis of HALT PKD Study A, we categorized participants into high and low dose groups based on the median daily equivalent dose of RAAS blocking drugs used after the initial dose titration period...
March 21, 2018: Current Hypertension Reviews
https://www.readbyqxmd.com/read/29534238/targeting-epigenetic-dna-and-histone-modifications-to-treat-kidney-disease
#15
Miguel Fontecha-Barriuso, Diego Martin-Sanchez, Olga Ruiz-Andres, Jonay Poveda, Maria Dolores Sanchez-Niño, Lara Valiño-Rivas, Marta Ruiz-Ortega, Alberto Ortiz, Ana Belén Sanz
Epigenetics refers to heritable changes in gene expression patterns not caused by an altered nucleotide sequence, and includes non-coding RNAs and covalent modifications of DNA and histones. This review focuses on functional evidence for the involvement of DNA and histone epigenetic modifications in the pathogenesis of kidney disease and the potential therapeutic implications. There is evidence of activation of epigenetic regulatory mechanisms in acute kidney injury (AKI), chronic kidney disease (CKD) and the AKI-to-CKD transition of diverse aetiologies, including ischaemia-reperfusion injury, nephrotoxicity, ureteral obstruction, diabetes, glomerulonephritis and polycystic kidney disease...
March 9, 2018: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29425281/at1-receptor-antagonism-improves-structural-functional-and-biomechanical-properties-in-resistance-arteries-in-a-rodent-chronic-kidney-disease-model
#16
Ko Jin Quek, Omar Z Ameer, Jacqueline K Phillips
BACKGROUND: The renin-angiotensin system, in particular Angiotensin II (AngII), plays a significant role in the pathogenesis of hypertension in chronic kidney disease (CKD). Effects of chronic AT1 receptor antagonism were investigated in a genetic hypertensive rat model of CKD, the Lewis polycystic kidney (LPK) rat. METHODS: Mixed-sex LPK and Lewis control rats (total n = 31) were split between treated (valsartan 60 mg/kg/day p.o. from 4 to 18 weeks) and vehicle groups...
May 7, 2018: American Journal of Hypertension
https://www.readbyqxmd.com/read/29358433/randomised-controlled-trial-to-determine-the-efficacy-and-safety-of-prescribed-water-intake-to-prevent-kidney-failure-due-to-autosomal-dominant-polycystic-kidney-disease-prevent-adpkd
#17
Annette T Y Wong, Carly Mannix, Jared J Grantham, Margaret Allman-Farinelli, Sunil V Badve, Neil Boudville, Karen Byth, Jessie Chan, Susan Coulshed, Marie E Edwards, Bradley J Erickson, Mangalee Fernando, Sheryl Foster, Imad Haloob, David C H Harris, Carmel M Hawley, Julie Hill, Kirsten Howard, Martin Howell, Simon H Jiang, David W Johnson, Timothy L Kline, Karthik Kumar, Vincent W Lee, Maureen Lonergan, Jun Mai, Philip McCloud, Anthony Peduto, Anna Rangan, Simon D Roger, Kamal Sud, Vincent Torres, Eswari Vliayuri, Gopala K Rangan
INTRODUCTION: Maintaining fluid intake sufficient to reduce arginine vasopressin (AVP) secretion has been hypothesised to slow kidney cyst growth in autosomal dominant polycystic kidney disease (ADPKD). However, evidence to support this as a clinical practice recommendation is of poor quality. The aim of the present study is to determine the long-term efficacy and safety of prescribed water intake to prevent the progression of height-adjusted total kidney volume (ht-TKV) in patients with chronic kidney disease (stages 1-3) due to ADPKD...
January 21, 2018: BMJ Open
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
#18
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...
May 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
#19
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...
March 2018: Kidney International
https://www.readbyqxmd.com/read/29241197/renoprotective-effects-of-metformin
#20
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...
2018: Nephron
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