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https://www.readbyqxmd.com/read/29029121/clinical-heterogeneity-and-phenotypic-expansion-of-napi-iia-associated-disease
#1
Korcan Demir, Melek Yildiz, Hilla Bahat, Michael Goldman, Nisreen Hassan, Shay Tzur, Ayala Ofir, Daniella Magen
Context: NaPi-IIa, encoded by SLC34A1 is a key phosphate transporter in the mammalian proximal tubule, and plays a cardinal role in renal phosphate handling. NaPi-IIa impairment has been linked to various overlapping clinical syndromes, including hypophosphatemic nephrolithiasis with osteoporosis, renal Fanconi's syndrome with chronic kidney disease, and most recently, with idiopathic infantile hypercalcemia and nephrocalcinosis. Objectives: We studied the molecular basis of idiopathic infantile hypercalcemia with partial proximal tubulopathy in two apparently unrelated patients of Israeli and Turkish descent...
September 29, 2017: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/29027595/carnosinase-diabetes-mellitus-and-the-potential-relevance-of-carnosinase-deficiency
#2
REVIEW
Verena Peters, Johannes Zschocke, Claus P Schmitt
Carnosinase (CN1) is a dipeptidase, encoded by the CNDP1 gene, that degrades histidine-containing dipeptides, such as carnosine, anserine and homocarnosine. Loss of CN1 function (also called carnosinase deficiency or aminoacyl-histidine dipeptidase deficiency) has been reported in a small number of patients with highly elevated blood carnosine concentrations, denoted carnosinaemia; it is unclear whether the variety of clinical symptoms in these individuals is causally related to carnosinase deficiency. Reduced CN1 function should increase serum carnosine concentrations but the genetic basis of carnosinaemia has not been formally confirmed to be due to CNDP1 mutations...
October 13, 2017: Journal of Inherited Metabolic Disease
https://www.readbyqxmd.com/read/29027182/preoperative-management-of-arteriovenous-fistula-avf-for-hemodialysis
#3
Gaetano Alfano, Francesco Fontana, Mario Iannaccone, Patrizia Noussan, Gianni Cappelli
Native arteriovenous fistula (AVF) is the favorite access for hemodialysis (HD). The National Kidney Foundation's Kidney Disease Outcomes Quality Initiative (KDOQI) recommends its creation in most patients with renal failure. Unfortunately, intensive efforts to promote native AVF in patients with marginal vessels have increased the rate of primary fistula failure. A non-functioning fistula prompts the use of central venous catheter (CVC) that, unlike AVF, has been associated with an increased risk of morbidity and mortality among patients receiving HD...
October 11, 2017: Journal of Vascular Access
https://www.readbyqxmd.com/read/29027072/outcomes-of-acute-kidney-injury-in-a-nephrology-ward
#4
Ana R Fernandes, Márcio S R Viegas, Elsa Q Soares, Sofia S Coelho, Patricia Valério, José C Barreto, José M Vinhas
Acute kidney injury (AKI) is a global problem which predicts immediate and long-term adverse outcomes. We evaluated the AKI progression to end-stage renal disease, as well as the mortality associated with AKI and the in-hospital readmission rate because of a cardiovascular event in AKI patients admitted in a nephrology ward. A 5-year retrospective study was set in a nephrology department, with a follow-up period of up to 8 years. In a total of 191 patients, mean age was 73.83 ± 12.49 years, and 137 (71...
October 11, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/29026467/chromogranin-a-as-a-biochemical-marker-for-neuroendocrine-tumors-a-single-center-experience-at-royal-hospital-oman
#5
Elham S Al-Risi, Fatma S Al-Essry, Waad-Allah S Mula-Abed
OBJECTIVES: To evaluate the significance of serum chromogranin A (CgA) status in patients with and without different neuroendocrine tumors (NETs) by conducting a retrospective assessment of the diagnostic utility and limitations of CgA as a biomarker for NETs in a tertiary care hospital in Oman. METHODS: We conducted a retrospective analysis of CgA requests referred to the Clinical Biochemistry Laboratory, Royal Hospital, Oman over a 24-month period (April 2012 to March 2014)...
September 2017: Oman Medical Journal
https://www.readbyqxmd.com/read/29024698/pediatric-clif-sofa-score-is-the-best-predictor-of-28-day-mortality-in-children-with-decompensated-chronic-liver-disease
#6
Rishi Bolia, Anshu Srivastava, Surender Kumar Yachha, Ujjal Poddar
BACKGROUND AND AIMS: Early identification of children with decompensated chronic liver disease (DCLD) at risk of short-term mortality helps improve outcome. We evaluated the predictors of outcome and role of Child Pugh [CP], Pediatric End- stage Liver Disease [PELD] and pediatric chronic liver failure sequential organ failure assessment [pCLIF-SOFA] score in prognosticating 28 days mortality in DCLD children. METHODS: DCLD children were prospectively evaluated with a clinico-laboratory proforma and followed till 28 days to determine outcome...
October 9, 2017: Journal of Hepatology
https://www.readbyqxmd.com/read/29022104/deriving-and-understanding-the-risk-of-post-transplant-recurrence-of-nephrotic-syndrome-in-the-light-of-current-molecular-and-genetic-advances
#7
REVIEW
Agnieszka Bierzynska, Moin A Saleem
After renal transplantation, recurrence of the original disease is the second most common cause of graft loss, after rejection. The most dramatic manifestation of this phenomenon is in patients with nephrotic syndrome (NS). NS is a descriptive term describing a clinical picture centred on proteinuria arising from damage to the glomerular filtration barrier (GFB). There are many different drivers of that damage, ranging from immune dysregulation to genetic disorders and chronic disease/infections. The main categories in childhood are "idiopathic" (presumed immune mediated) and genetic NS, with further stratification of the idiopathic group according to steroid responses...
October 11, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29021843/impact-of-baseline-renal-function-on-all-cause-mortality-in-patients-who-underwent-cardiac-resynchronization-therapy-a-systematic-review-and-meta-analysis
#8
REVIEW
G Bazoukis, K P Letsas, P Korantzopoulos, C Thomopoulos, K Vlachos, S Georgopoulos, N Karamichalakis, A Saplaouras, M Efremidis, A Sideris
BACKGROUND: Cardiac resynchronization therapy (CRT) improves both morbidity and mortality in selected patients with heart failure and increased QRS duration. However, chronic kidney disease (CKD) may have an adverse effect on patient outcome. The aim of this systematic review was to analyze the existing data regarding the impact of baseline renal function on all-cause mortality in patients who underwent CRT. METHODS: Medline database was searched systematically, and studies evaluating the effect of baseline renal function on all-cause mortality in patients who underwent CRT were retrieved...
October 2017: Journal of Arrhythmia
https://www.readbyqxmd.com/read/29021382/sustained-activation-of-guanylate-cyclase-a-with-tdt-a-natriuretic-peptide-derivative-exhibits-cardiorenal-protection-in-dahl-salt-sensitive-hypertensive-rats
#9
Shohei Oishi, Naoko Suzuki, Yuri Hasui, Tsuyoshi Homma, Masanori Obana, Takahiro Nagayama, Yasushi Fujio
Heart failure often presents with prognosis-relevant impaired renal function. To investigate whether the chronic activation of guanylate cyclase-A (GC-A) protects both heart and kidney, we examined the effects of TDT, a neprilysin (NEP)-resistant natriuretic peptide (NP) derivative, on cardiac and renal dysfunction in Dahl salt-sensitive hypertensive rats (DS rats). Pretreatment with NEP or NEP inhibitor did not influence GC-A activation by TDT both in vitro and in vivo, resulting in a long-acting profile of TDT compared to native human atrial NP (hANP)...
October 11, 2017: Journal of Pharmacology and Experimental Therapeutics
https://www.readbyqxmd.com/read/29021274/prevalence-and-outcomes-of-left-sided-valvular-heart-disease-associated-with-chronic-kidney-disease
#10
Zainab Samad, Joseph A Sivak, Matthew Phelan, Phillip J Schulte, Uptal Patel, Eric J Velazquez
BACKGROUND: Chronic kidney disease (CKD) is an adverse prognostic marker for valve intervention patients; however, the prevalence and related outcomes of valvular heart disease in CKD patients is unknown. METHODS AND RESULTS: Included patients underwent echocardiography (1999-2013), had serum creatinine values within 6 months before index echocardiogram, and had no history of valve surgery. CKD was defined as diagnosis based on the International Classification of Diseases, Ninth Revision or an estimated glomerular filtration rate <60 mL/min per 1...
October 11, 2017: Journal of the American Heart Association
https://www.readbyqxmd.com/read/29020583/glecaprevir-and-pibrentasvir-in-patients-with-hcv-and-severe-renal-impairment
#11
Edward Gane, Eric Lawitz, David Pugatch, Georgios Papatheodoridis, Norbert Bräu, Ashley Brown, Stanislas Pol, Vincent Leroy, Marcello Persico, Christophe Moreno, Massimo Colombo, Eric M Yoshida, David R Nelson, Christine Collins, Yang Lei, Matthew Kosloski, Federico J Mensa
Background Chronic hepatitis C virus (HCV) infection is more prevalent among patients who have chronic kidney disease than among those who do not have the disease. Patients with chronic kidney disease who also have HCV infection are at higher risk for progression to end-stage renal disease than those who have chronic kidney disease without HCV infection. Patients with both HCV infection and advanced chronic kidney disease have limited treatment options. Methods We conducted a multicenter, open-label, phase 3 trial to evaluate the efficacy and safety of treatment with the combination of the NS3/4A protease inhibitor glecaprevir and the NS5A inhibitor pibrentasvir for 12 weeks in adults who had HCV genotype 1, 2, 3, 4, 5, or 6 infection and also had compensated liver disease (with or without cirrhosis) with severe renal impairment, dependence on dialysis, or both...
October 12, 2017: New England Journal of Medicine
https://www.readbyqxmd.com/read/29018798/tafro-syndrome-in-caucasians-a-case-report-and-review-of-the-literature
#12
Céline Louis, Sandrine Vijgen, Kaveh Samii, Yves Chalandon, Louis Terriou, David Launay, David C Fajgenbaum, Jörg D Seebach, Yannick D Muller
BACKGROUND: TAFRO syndrome has been reported in Japan among human herpesvirus 8 (HHV-8)-negative/idiopathic multicentric Castleman's disease (iMCD) patients. To date, the majority of iMCD patients with TAFRO syndrome originate from Japan. CASE PRESENTATION: Herein, we report a 67-year-old HIV/HHV-8-negative Caucasian iMCD patient diagnosed with TAFRO. He presented with marked systemic inflammation, bicytopenia, terminal renal insufficiency, diffuse lymphadenopathies, and anasarca...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/29018182/role-of-nitric-oxide-pathway-in-development-and-progression-of-chronic-kidney-disease-in-rats-sensitive-and-resistant-to-its-occurrence-in-an-experimental-model-of-5-6-nephrectomy
#13
Marek Saracyn, Katarzyna Czarzasta, Marek Brytan, Piotr Murawski, Sławomir Lewicki, Tomasz Ząbkowski, Robert Zdanowski, Agnieszka Cudnoch-Jędrzejewska, Grzegorz Wiktor Kamiński, Zofia Wańkowicz
BACKGROUND Understanding the mechanisms conditioning development of chronic kidney disease (CKD) is still a challenge. The aim of this study was to evaluate the activity of the intrarenal nitric oxide (NO) pathway in the context of sensitivity or resistance of different animal strains to the development and degree of renal failure. MATERIAL AND METHODS Two rat strains were used: Wistar (WR) and Sprague-Dawley rats (SDR) in a model of CKD - 5/6 nephrectomy. We assessed parameters of renal failure and expression of nitric oxide synthase (NOS) isoforms in renal cortex and medulla...
October 11, 2017: Medical Science Monitor: International Medical Journal of Experimental and Clinical Research
https://www.readbyqxmd.com/read/29017166/the-role-of-the-mineralocorticoid-receptor-in-inflammation-focus-on-kidney-and-vasculature
#14
Zachary Belden, Jeffrey A Deiuliis, Mirela Dobre, Sanjay Rajagopalan
BACKGROUND: The remarkable success of clinical trials in mineralocorticoid receptor (MR) inhibition in heart failure has driven research on the physiological and pathological role(s) of nonepithelial MR expression. MR is widely expressed in the cardiovascular system and is a major determinant of endothelial function, smooth muscle tone, vascular remodeling, fibrosis, and blood pressure. An important new dimension is the appreciation of the role MR plays in immune cells and target organ damage in the heart, kidney and vasculature, and in the development of insulin resistance...
October 10, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/29016969/implementation-of-a-scalable-web-based-automated-clinical-decision-support-risk-prediction-tool-for-chronic-kidney-disease-using-c-cda-and-application-programming-interfaces
#15
Lipika Samal, John D D'Amore, David W Bates, Adam Wright
Background and Objective: Clinical decision support tools for risk prediction are readily available, but typically require workflow interruptions and manual data entry so are rarely used. Due to new data interoperability standards for electronic health records (EHRs), other options are available. As a clinical case study, we sought to build a scalable, web-based system that would automate calculation of kidney failure risk and display clinical decision support to users in primary care practices...
November 1, 2017: Journal of the American Medical Informatics Association: JAMIA
https://www.readbyqxmd.com/read/29016832/contemporary-stroke-prevention-strategies-in-11%C3%A2-096-european-patients-with-atrial-fibrillation-a-report-from-the-eurobservational-research-programme-on-atrial-fibrillation-eorp-af-long-term-general-registry
#16
Giuseppe Boriani, Marco Proietti, Cécile Laroche, Laurent Fauchier, Francisco Marin, Michael Nabauer, Tatjana Potpara, Gheorghe-Andrei Dan, Zbigniew Kalarus, Igor Diemberger, Luigi Tavazzi, Aldo P Maggioni, Gregory Y H Lip
Aims: Contemporary data regarding atrial fibrillation (AF) management and current use of oral anticoagulants (OACs) for stroke prevention are needed. Methods and results: The EURObservational Research Programme on AF (EORP-AF) Long-Term General Registry analysed consecutive AF patients presenting to cardiologists in 250 centres from 27 European countries. From 2013 to 2016, 11 096 patients were enrolled (40.7% female; mean age 69 ± 11 years). At discharge, OACs were used in 9379 patients (84...
September 14, 2017: Europace: European Pacing, Arrhythmias, and Cardiac Electrophysiology
https://www.readbyqxmd.com/read/28992758/aldosterone-breakthrough-does-not-alter-central-hemodynamics
#17
Andrew Beenken, Andrew S Bomback
INTRODUCTION: Angiotensin-converting enzyme inhibitors and angiotensin receptor blockers are widely used in congestive heart failure and chronic kidney disease, but up to 40% of patients will experience aldosterone breakthrough, with aldosterone levels rising above pre-treatment levels after 6-12 months of renin-angiotensin-aldosterone system blockade. Aldosterone breakthrough has been associated with worsening congestive heart failure and chronic kidney disease, yet the pathophysiology remains unclear...
October 2017: Journal of the Renin-angiotensin-aldosterone System: JRAAS
https://www.readbyqxmd.com/read/28992319/atrial-fibrillation-in-kidney-transplant-recipients-is-there-a-place-for-the-novel-drugs
#18
Jolanta Malyszko, Paulina Lopatowska, Elzbieta Mlodawska, Dominika Musialowska, Jacek S Malyszko, Anna Tomaszuk-Kazberuk
Atrial fibrillation (AF) is the most common sustained cardiac arrhythmia of high clinical importance, occurring in 2% of the general population and in 19-24% in patients with chronic kidney disease. It is a well-known risk factor for cardiovascular morbidity and mortality. Kidney transplant recipients with a history of AF were associated with significantly higher rate of ischaemic strokes, graft failure and post-transplant mortality. AF occurs in over 7% of kidney transplant recipients in the first 3 years after transplantation and is associated with reduced graft and patient survival...
September 7, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28990414/biomarkers-of-endothelial-renal-and-platelet-dysfunction-in-stage-5-chronic-kidney-disease-hemodialysis-patients-with-heart-failure
#19
Ryan McMillan, Leonidas Skiadopoulos, Debra Hoppensteadt, Nil Guler, Vinod Bansal, Ravipresenna Parasuraman, Jawed Fareed
The aim of this study was to determine the role of endothelial, renal, and inflammatory biomarkers in the pathogenesis of heart failure (HF) in patients with stage 5 chronic kidney disease (CKD5) undergoing maintenance hemodialysis (HD). Plasma levels of biomarkers-kidney injury molecule 1 (KIM-1), N-terminal pro brain natriuretic peptide (NT-proBNP), glycated hemoglobin, neutrophil gelatinase-associated lipocalin, interleukin-18,platelet-derived growth factor, platelet factor 4 (PF4), 25-OH vitamin D, parathyroid hormone (PTH), endothelin, and endocan-were measured in CKD5-HD patients at the Loyola University Ambulatory Dialysis facility...
January 1, 2017: Clinical and Applied Thrombosis/hemostasis
https://www.readbyqxmd.com/read/28989292/diffusion-in-tube-dialyzer
#20
Yohannes Nigatie
Nowadays, kidney failure is a problem of many peoples in the world. We know that the main function of kidney is maintaining the chemical quality of blood particularly removing urea through urine. But when they malfunction, the pathologic state known as uremia results in a condition in which the urea is retained in the body. Failure of the kidney results in building up of harmful wastes and excess fluids in the body. Kidney diseases (failures) can be due to infections, high blood pressure (hypertension), diabetes, and/or extensive use of medication...
2017: Biomedical Engineering and Computational Biology
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