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https://www.readbyqxmd.com/read/28329394/intermediate-follow-up-of-pediatric-patients-with-hemolytic-uremic-syndrome-during-the-2011-outbreak-caused-by-e-coli-o104-h4
#1
Sebastian Loos, Wiebke Aulbert, Bernd Hoppe, Thurid Ahlenstiel-Grunow, Birgitta Kranz, Charlotte Wahl, Hagen Staude, Alexander Humberg, Kerstin Benz, Martin Krause, Martin Pohl, Max C Liebau, Raphael Schild, Johanna Lemke, Ortraud Beringer, Dominik Müller, Christoph Härtel, Marianne Wigger, Udo Vester, Martin Konrad, Dieter Haffner, Lars Pape, Jun Oh, Markus J Kemper
Background.: In 2011 Escherichia coli O104:H4 caused an outbreak with over 800 cases of hemolytic uremic syndrome (HUS) in Germany, including 90 children. Data on the intermediate outcome in children after HUS due to E. coli O104:H4 have been lacking. Methods.: Follow-up data were gathered retrospectively from the medical records of patients who had been included in the German Pediatric HUS Registry during the 2011 outbreak. Results.: A total of 72/89 (81%) of the patients was included after a median follow-up of 3...
March 13, 2017: Clinical Infectious Diseases: An Official Publication of the Infectious Diseases Society of America
https://www.readbyqxmd.com/read/28329085/renal-sodium-avidity-in-heart-failure-from-pathophysiology-to-treatment-strategies
#2
Wilfried Mullens, Frederik Hendrik Verbrugge, Petra Nijst, Wai Hong Wilson Tang
Increased neurohumoral stimulation resulting in excessive sodium avidity and extracellular volume overload are hallmark features of decompensated heart failure. Especially in case of concomitant renal dysfunction, the kidneys often fail to elicit effective natriuresis. While assessment of renal function is generally performed by measuring serum creatinine-a surrogate for glomerular filtration-, this only represents part of the nephron's function. Alterations in tubular sodium handling are at least equally important in the development of volume overload and congestion...
February 23, 2017: European Heart Journal
https://www.readbyqxmd.com/read/28328764/simultaneous-determination-of-protein-unbound-cyclosporine-a-and-mycophenolic-acid-in-kidney-transplant-patients-using-liquid-chromatography-tandem-mass-spectrometry
#3
Heike Bittersohl, Juliane Herbinger, Ming Wen, Lutz Renders, Werner Steimer, Peter B Luppa
BACKGROUND: Therapeutic drug monitoring (TDM) of immunosuppressants is essential to optimize patient care after organ transplantation. In blood, most immunosuppressive drugs are bound to plasma proteins or located inside blood cells. However, it is generally assumed that only protein-unbound (free) drug concentrations are pharmacologically active and could therefore better reflect the clinical outcome. Study data are still limited due to lacking rapid analytical methods. Therefore, a simple multiplex method for direct measurement of free cyclosporine A (CsA) and mycophenolic acid (MPA) has been developed...
March 21, 2017: Therapeutic Drug Monitoring
https://www.readbyqxmd.com/read/28328650/exosomes-in-critical-illness
#4
Nora Terrasini, Vincenzo Lionetti
OBJECTIVE: Exosomes are small, cell-released vesicles (40-100 nm in size) with the potential to transfer proteins, lipids, small RNAs, messenger RNAs, or DNA between cells via interstitial fluids. Due to their role in tissue homeostasis, exosomes have emerged as a new type of therapeutic and diagnostic (theranostic) tool in the noninvasive assessment of organ response to injury or treatment and in the development of reliable organ-protective intensive therapy. Our review provides current insights into the role of exosomes in the personalized management of injury and repair responses in critical illness...
March 21, 2017: Critical Care Medicine
https://www.readbyqxmd.com/read/28328253/a-comprehensive-index-for-predicting-risk-of-anemia-from-patients-diagnoses
#5
Matthew G Tuck, Farrokh Alemi, John F Shortle, Sanj Avramovic, Charles Hesdorffer
This article demonstrates how time-dependent, interacting, and repeating risk factors can be used to create more accurate predictive medicine. In particular, we show how emergence of anemia can be predicted from medical history within electronic health records. We used the Veterans Affairs Informatics and Computing Infrastructure database to examine a retrospective cohort of 9,738,838 veterans over an 11-year period. Using International Clinical Diagnoses Version 9 codes organized into 25 major diagnostic categories, we measured progression of disease by examining changes in risk over time, interactions in risk of combination of diseases, and elevated risk associated with repeated hospitalization for the same diagnostic category...
March 2017: Big Data
https://www.readbyqxmd.com/read/28328233/long-term-neurodevelopmental-outcome-of-neonates-with-hypernatremic-dehydration
#6
Hassan Boskabadi, Javad Akhondian, Maliheh Afarideh, Gholamali Maamouri, Sepideh Bagheri, Seyyed Mostafa Parizadeh, Majid Ghayour Mobarhan, Shabnam Mohammadi, Gordon A A Frens
BACKGROUND: Neonatal hypernatremic dehydration (NHD) is a dangerous condition that can lead to severe weight loss, renal impairment, and central nervous system complications. We aimed to evaluate the consequences of NHD in infants in their second year of life. MATERIALS AND METHODS: This was a prospective case-control study in Ghaem hospital, Mashhad, Iran. Sixty-five healthy breastfed neonates (serum sodium concentration <150 mmol/L) and 65 hypernatremic (serum sodium concentration ≥150 mmol/L) neonates were followed up from 2008 to 2011...
March 22, 2017: Breastfeeding Medicine: the Official Journal of the Academy of Breastfeeding Medicine
https://www.readbyqxmd.com/read/28328097/markers-of-iron-status-in-chronic-kidney-disease
#7
Adam E Gaweda
Anemia is one of the main comorbidities related to chronic kidney disease (CKD). Until the advent of erythropoiesis stimulating agents (ESA), endogenous erythropoietin deficiency has been thought to be the main culprit of anemia in CKD patients. The use of ESAs has shed new light on the physiology of CKD anemia, where iron homeostasis plays an increasingly important role. Disorders of iron homeostasis occurring in CKD turn the anemia management in those patients into a complex multifactorial therapeutic task, where ESA and Iron dose must be properly balanced to achieve the desired outcome without exposing the patients to the risk of serious adverse events...
March 22, 2017: Hemodialysis International
https://www.readbyqxmd.com/read/28327570/puf60-variants-cause-a-syndrome-of-id-short-stature-microcephaly-coloboma-craniofacial-cardiac-renal-and-spinal-features
#8
Karen J Low, Morad Ansari, Rami Abou Jamra, Angus Clarke, Salima El Chehadeh, David R FitzPatrick, Mark Greenslade, Alex Henderson, Jane Hurst, Kory Keller, Paul Kuentz, Trine Prescott, Franziska Roessler, Kaja K Selmer, Michael C Schneider, Fiona Stewart, Katrina Tatton-Brown, Julien Thevenon, Magnus D Vigeland, Julie Vogt, Marjolaine Willems, Jonathan Zonana, D D D Study, Sarah F Smithson
PUF60 encodes a nucleic acid-binding protein, a component of multimeric complexes regulating RNA splicing and transcription. In 2013, patients with microdeletions of chromosome 8q24.3 including PUF60 were found to have developmental delay, microcephaly, craniofacial, renal and cardiac defects. Very similar phenotypes have been described in six patients with variants in PUF60, suggesting that it underlies the syndrome. We report 12 additional patients with PUF60 variants who were ascertained using exome sequencing: six through the Deciphering Developmental Disorders Study and six through similar projects...
March 22, 2017: European Journal of Human Genetics: EJHG
https://www.readbyqxmd.com/read/28326799/glomerular-loss-after-renal-radiofrequency-ablation-are-comparable-to-30-minutes-of-warm-ischemia
#9
Leonardo A S Abreu, José A Damasceno-Ferreira, Waldemar S Costa, Marco Aurélio Pereira-Sampaio, Francisco José Sampaio, Diogo B de Souza
PURPOSE: To compare, with stereological methods, the glomerular loss in kidneys submitted to radiofrequency ablation (RFA) or warm ischemia. METHODS: Twenty-six male pigs were divided into three groups. Eight animals were allocated in the sham group, which was submitted to laparoscopic dissection of the left renal hilum, without renal ischemia. Eight animals were allocated in the ischemia group, which had the left renal hilum clamped for 30 minutes under laparoscopic access...
March 22, 2017: Journal of Endourology
https://www.readbyqxmd.com/read/28325846/soluble-flt1-gene-therapy-alleviates-brain-arteriovenous-malformation-severity
#10
Wan Zhu, Fanxia Shen, Lei Mao, Lei Zhan, Shuai Kang, Zhengda Sun, Jeffrey Nelson, Rui Zhang, Dingquan Zou, Cameron M McDougall, Michael T Lawton, Thiennu H Vu, Zhijian Wu, Abraham Scaria, Peter Colosi, John Forsayeth, Hua Su
BACKGROUND AND PURPOSE: Brain arteriovenous malformation (bAVM) is an important risk factor for intracranial hemorrhage. Current therapies are associated with high morbidities. Excessive vascular endothelial growth factor has been implicated in bAVM pathophysiology. Because soluble FLT1 binds to vascular endothelial growth factor with high affinity, we tested intravenous delivery of an adeno-associated viral vector serotype-9 expressing soluble FLT1 (AAV9-sFLT1) to alleviate the bAVM phenotype...
March 21, 2017: Stroke; a Journal of Cerebral Circulation
https://www.readbyqxmd.com/read/28325753/mouse-model-for-inherited-renal-fibrosis-associated-with-endoplasmic-reticulum-stress
#11
Sian E Piret, Eric Olinger, Anita A C Reed, M Andrew Nesbit, Tertius A Hough, Liz Bentley, Olivier Devuyst, Roger Cox, Rajesh V Thakker
Renal fibrosis is a common feature of renal failure resulting from multiple aetiologies, including diabetic nephropathy, hypertension and inherited renal disorders. However, the mechanisms of renal fibrosis are incompletely understood and we therefore explored these by establishing a mouse model for a renal tubular disorder, referred to as autosomal dominant tubulointerstitial kidney disease (ADTKD) due to missense uromodulin (UMOD) mutations (ADTKD-UMOD). ADTKD-UMOD, which is associated with retention of mutant uromodulin in the endoplasmic reticulum (ER) of renal thick ascending limb cells, is characterized by hyperuricemia, interstitial fibrosis, inflammation, and renal failure, and we used targeted homologous recombination to generate a knock-in mouse model with an ADTKD-causing missense cysteine to arginine uromodulin mutation (C125R)...
March 21, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28325740/proximal-tubulopathy-prime-mover-and-key-therapeutic-target-in-diabetic-kidney-disease
#12
Richard E Gilbert
The current view of diabetic kidney disease, based on meticulously acquired ultrastructural morphometry and the utility of measuring plasma creatinine and urinary albumin, has been almost entirely focused on the glomerulus. While clearly of great importance, changes in the glomerulus are not the major determinant of renal prognosis in diabetes and may not be the primary event in the development of diabetic kidney disease either. Indeed, advances in biomarker discovery and a greater appreciation of tubulointerstitial histopathology and the role of tubular hypoxia in the pathogenesis of chronic kidney disease have given us pause to reconsider the current "glomerulocentric" paradigm and focus attention on the proximal tubule that by virtue of the high energy requirements and reliance on aerobic metabolism render it particularly susceptible to the derangements of the diabetic state...
April 2017: Diabetes
https://www.readbyqxmd.com/read/28325697/alcam-a-novel-biomarker-in-patients-with-type-2-diabetes-mellitus-complicated-with-diabetic-nephropathy
#13
Alba Sulaj, Stefan Kopf, Elisabeth Gröne, Hermann-Josef Gröne, Sigrid Hoffmann, Erwin Schleicher, Hans-Ulrich Häring, Vedat Schwenger, Stephan Herzig, Thomas Fleming, Peter P Nawroth, Rüdiger von Bauer
BACKGROUND & AIM: Activated leukocyte cell adhesion molecule (ALCAM/CD166) functions analogue to the receptor of advanced glycation end products, which has been implicated in the development of diabetic nephropathy (DN). We investigated the expression of ALCAM and its ligand S100B in patients with DN. METHODS: A total of 34 non-diabetic patients, 29 patients with type 2 diabetes and normal albuminuria and 107 patients with type 2 diabetes complicated with DN were assessed for serum concentration of soluble ALCAM (sALCAM) by ELISA...
January 21, 2017: Journal of Diabetes and its Complications
https://www.readbyqxmd.com/read/28325651/genitourinary-paraganglioma-demographic-pathologic-and-clinical-characteristics-in-the-surveillance-epidemiology-and-end-results-database-2000-2012
#14
Stephanie Purnell, Abhinav Sidana, Mahir Maruf, Campbell Grant, Piyush K Agarwal
BACKGROUND: Extra-adrenal paragangliomas (PGLs) are infrequent, benign, and neuroendocrine tumors arising from chromaffin cells of the autonomic nervous system. Most PGLs are sporadic, but up to 32% are associated with inherited syndromes such as neurofibromatosis type 1, von Hippel-Lindau disease, and familial PGL. Although most PGLs develop above the umbilicus, they have been reported in the genitourinary (GU) tract. Owing to the paucity of literature on the rates of GU PGL, the objective of our study is to describe the demographic, pathologic, and clinical characteristics of GU PGL, and compare them to non-GU sites of PGL using the surveillance, epidemiology, and end results (SEER) database...
March 18, 2017: Urologic Oncology
https://www.readbyqxmd.com/read/28325303/inhibition-of-egf-uptake-by-nephrotoxic-antisense-drugs-in%C3%A2-vitro-and-implications-for-preclinical-safety-profiling
#15
Annie Moisan, Marcel Gubler, Jitao David Zhang, Yann Tessier, Kamille Dumong Erichsen, Sabine Sewing, Régine Gérard, Blandine Avignon, Sylwia Huber, Fethallah Benmansour, Xing Chen, Roberto Villaseñor, Annamaria Braendli-Baiocco, Matthias Festag, Andreas Maunz, Thomas Singer, Franz Schuler, Adrian B Roth
Antisense oligonucleotide (AON) therapeutics offer new avenues to pursue clinically relevant targets inaccessible with other technologies. Advances in improving AON affinity and stability by incorporation of high affinity nucleotides, such as locked nucleic acids (LNA), have sometimes been stifled by safety liabilities related to their accumulation in the kidney tubule. In an attempt to predict and understand the mechanisms of LNA-AON-induced renal tubular toxicity, we established human cell models that recapitulate in vivo behavior of pre-clinically and clinically unfavorable LNA-AON drug candidates...
March 17, 2017: Molecular Therapy. Nucleic Acids
https://www.readbyqxmd.com/read/28325251/interferon-induced-thrombotic-microangiopathy-tma-analysis-and-concise-review
#16
REVIEW
Ajay Kundra, Jen Chin Wang
Interferon (IFN) has been associated with development of thrombotic microangiopathy including thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). We reviewed literature from the earliest reported association in 1993, to July 2016 and found 68 cases. Analysis of this data shows: (1) Mean age at diagnosis was 47 years (95% CI, 44-50). (2) Majority of cases were seen where IFN was used for the treatment of chronic myelogenous leukemia (CML), multiple sclerosis (MS), chronic hepatitis C virus infection (HCV) and one case each for hairy cell leukemia (HCL) and Sezary syndrome...
April 2017: Critical Reviews in Oncology/hematology
https://www.readbyqxmd.com/read/28325081/lipid-deposition-in-kidney-diseases-interplay-among-redox-lipid-mediators-and-renal-impairment
#17
Hua Su, Cheng Wan, Chun-Tao Lei, Chun-Yun Zhang, Chen Ye, Hui Tang, Yue Qiu, Chun Zhang
Lipids encompass a large variety of molecules representing a highly efficient energy resource and forming structural components of membranes or signaling molecules that regulate diverse cellular events underlying not only physiological homeostatic processes but also undesirable pathogenic outcomes. The relationship between lipid disturbances and renal diseases has been studied for several decades, and it is well-recognized that when the balance of renal lipid uptake, synthesis, oxidation and outflow is disrupted, lipids will undergo oxidation, be sequestrated as lipid droplets, generate toxic metabolites and cause nephrotoxicity in diverse renal diseases...
March 22, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/28323627/effects-of-xuebijing-injection-for-patients-with-sepsis-induced-acute-kidney-injury-after-wenchuan-earthquake
#18
Qin Yuxi, Hanlai Zhang, Yu Baili, Shihua Shi
Context • Earthquakes are devastating disasters that have claimed countless lives in the past, partially owing to the danger of direct and indirect injuries to vital organs. In the deadly earthquake that occurred in Sichuan Province of China on May 12, 2008, some victims suffered from severe damage to multiple organs and tissue and developed sepsis, a systematic inflammatory reaction resulting from infection and trauma. Xuebijing injection (CCXG) could beneficial for improvement of clinical symptoms in those patients, but no research has yet examined its potential...
March 2017: Alternative Therapies in Health and Medicine
https://www.readbyqxmd.com/read/28323153/application-and-development-of-a-taqman-real-time-pcr-for-detecting-infectious-spleen-and-kidney-necrosis-virus-in-siniperca-chuatsi
#19
Qiang Lin, Xiaozhe Fu, Lihui Liu, Hongru Liang, Huizhi Guo, Shuwen Yin, Venkatesh Kumaresan, Zhibin Huang, Ningqiu Li
Infectious spleen and kidney necrosis virus (ISKNV) is one of the major epidemiological agents that had caused great economic loss in Chinese perch (Siniperca chuatsi). In this study, a specific TaqMan real-time PCR was developed using a pair of primers and a TaqMan probe specific to the ORF007 gene of ISKNV to rapidly detect ISKNV copies in Chinese perch samples. This assay was optimized to produce linearity from 8.75 × 10(8) to 8.75 × 10(1) copies in standard curve with an efficiency of 98% and a R(2) value of 0...
March 16, 2017: Microbial Pathogenesis
https://www.readbyqxmd.com/read/28322484/tfe3-positive-renal-cell-carcinoma-occurring-in-three-children-with-dysfunctional-kidneys-on-immunosuppression
#20
Beverly A Schaefer, Theodore S Johnson, David K Hooper, Jaimie D Nathan, James I Geller
Pediatric RCC is a rare pediatric neoplasm and is distinctly different compared to adult RCC, often demonstrating translocation morphology evidenced by unique histopathological features and TFE3 or TFEB nuclear expression. We report three cases of pediatric TFE3 positive RCC (TFE3-RCC) occurring in the setting of chronic kidney disease and long-term pharmacological immunosuppression, including two cases that developed in the native kidney following kidney transplantation. Together, these cases suggest that the kidney microenvironment in combination with immune dysregulation is likely contributing factors in the pathogenesis of some pediatric RCC, warranting further study...
March 21, 2017: Pediatric Transplantation
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