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https://www.readbyqxmd.com/read/28102546/ataluren-and-similar-compounds-specific-therapies-for-premature-termination-codon-class-i-mutations-for-cystic-fibrosis
#1
REVIEW
Aisha A Aslam, Colin Higgins, Ian P Sinha, Kevin W Southern
BACKGROUND: Cystic fibrosis is a common life-shortening genetic disorder in the Caucasian population (less common in other ethnic groups) caused by the mutation of a single gene that codes for the production of the cystic fibrosis transmembrane conductance regulator protein. This protein coordinates the transport of salt (and bicarbonate) across cell surfaces and the mutation most notably affects the airways. In the lungs of people with cystic fibrosis, defective protein results in a dehydrated surface liquid and compromised mucociliary clearance...
January 19, 2017: Cochrane Database of Systematic Reviews
https://www.readbyqxmd.com/read/28102499/aortic-radial-pulse-wave-velocity-ratio-in-end-stage-renal-disease-patients-association-with-age-body-tissue-hydration-status-renal-failure-etiology-and-five-years-of-hemodialysis
#2
Daniel Bia, Rodolfo Valtuille, Cintia Galli, Sandra Wray, Ricardo Armentano, Yanina Zócalo, Edmundo Cabrera-Fischer
INTRODUCTION: The etiology of the end-stage renal disease (ESRD) and the hydration status may be involved in the arterial stiffening process observed in hemodialyzed patients. The ratio between carotid-femoral and carotid-radial pulse wave velocity (PWV ratio) was recently proposed to characterize the patient-specific stiffening process. AIMS: to analyze: (1) the PWV-ratio in healthy and hemodialyzed subjects, analyzing potential changes associated to etiologies of the ESRD, (2) the PWV-ratio and hydration status using multiple-frequency bioimpedance and, (3) the effects of hemodialysis on PWV-ratio in a 5-year follow-up...
January 19, 2017: High Blood Pressure & Cardiovascular Prevention: the Official Journal of the Italian Society of Hypertension
https://www.readbyqxmd.com/read/28101838/renal-consequences-of-preterm-birth
#3
REVIEW
Amelie Stritzke, Sumesh Thomas, Harish Amin, Christoph Fusch, Abhay Lodha
BACKGROUND: The developmental origin of health and disease concept identifies the brain, cardiovascular, liver, and kidney systems as targets of fetal adverse programming with adult consequences. As the limits of viability in premature infants have been pushed to lower gestational ages, the long-term impact of prematurity on kidneys still remains a significant burden during hospital stay and beyond. OBJECTIVES: The purpose of this study is to summarize available evidence, mechanisms, and short- and long-term renal consequences of prematurity and identify nephroprotective strategies and areas of uncertainty...
December 2017: Molecular and Cellular Pediatrics
https://www.readbyqxmd.com/read/28101755/does-n-terminal-pro-brain-type-natriuretic-peptide-predict-cardiac-complications-after-hip-fracture-surgery
#4
Hiroki Ushirozako, Tsuyoshi Ohishi, Tomotada Fujita, Daisuke Suzuki, Kazufumi Yamamoto, Tomohiro Banno, Hiroyuki Takase, Yukihiro Matsuyama
BACKGROUND: Elderly patients with hip fracture are at risk for cardiac complications. N-terminal pro-brain type natriuretic peptide (NT-proBNP) has been shown to predict cardiac complications in surgical patients; however, to our knowledge, only two studies have evaluated the utility of this test in patients with hip fracture. We believe it is important to assess a more accurate cutoff value of NT-proBNP with exclusion of patients with renal failure. QUESTIONS/PURPOSES: To assess the association between preoperative NT-proBNP and cardiac complications after hip fracture surgery...
January 18, 2017: Clinical Orthopaedics and related Research
https://www.readbyqxmd.com/read/28101457/therapeutic-effects-of-omega-3-fatty-acids-on-chronic-kidney-disease-associated-pruritus-a-literature-review
#5
REVIEW
Yunes Panahi, Simin Dashti-Khavidaki, Farahnoosh Farnood, Hamid Noshad, Mahsa Lotfi, Afshin Gharekhani
Uremic pruritus remains one of the most tormenting, frequent and potentially disabling problem in chronic kidney disease (CKD) patients. However, an area of substantial etiological interest with relation to uremic pruritus is the essential fatty acids deficiency. So we performed a literature review to elucidate the efficacy of omega-3 fatty acids on uremic pruritus. This review evaluated all of the studies published in English language, focusing on the clinical effects of omega-3 fatty acids on uremic pruritus...
December 2016: Advanced Pharmaceutical Bulletin
https://www.readbyqxmd.com/read/28101452/severe-hyperkalemia-following-blood-transfusions-is-there-a-link
#6
Christos V Rizos, Haralampos J Milionis, Moses S Elisaf
Patients with gastrointestinal bleeding often require large volume blood transfusion. Among the various side effects of blood transfusion, the increase of potassium levels is a serious one which is often overlooked. We report a case of severe hyperkalemia in a patient with gastric bleeding after large volume transfusion of packed red blood cells. The patient had hyperkalemia at baseline associated with his receiving medication as well as acute renal failure following hypovolemia. The baseline hyperkalemia was further aggravated after massive transfusions of packed red blood cells in a short period of time...
January 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28101447/functional-coupling-of-v-atpase-and-clc-5
#7
REVIEW
Nobuhiko Satoh, Masashi Suzuki, Motonobu Nakamura, Atsushi Suzuki, Shoko Horita, George Seki, Kyoji Moriya
Dent's disease is an X-linked renal tubulopathy characterized by low molecular weight proteinuria, hypercalciuria and progressive renal failure. Disease aetiology is associated with mutations in the CLCN5 gene coding for the electrogenic 2Cl(-)/H(+) antiporter chloride channel 5 (CLC-5), which is expressed in the apical endosomes of renal proximal tubules with the vacuolar type H(+)-ATPase (V-ATPase). Initially identified as a member of the CLC family of Cl(-) channels, CLC-5 was presumed to provide Cl(-) shunt into the endosomal lumen to dissipate H(+) accumulation by V-ATPase, thereby facilitating efficient endosomal acidification...
January 6, 2017: World Journal of Nephrology
https://www.readbyqxmd.com/read/28100940/surgery-in-pancreatic-necrosis-challenges-and-outcomes-in-an-industrial-hospital
#8
Ashok Chattoraj, Sunil Kumar
Twenty-seven cases of pancreatic necrosis were admitted and treated at our hospital from Jan 2010 till Jan 2015. Eight of these patients (29 %) underwent pancreatic necrosectomy. Of a total number of 957 patients admitted for acute pancreatitis, 27 patients (3 %) were diagnosed to have necrotizing pancreatitis. Of the 8 patients operated on, 5 patients (62.5 %) were treated successfully. The 3 patients who died had 3 organ (renal, respiratory and cardiovascular) failures. Nineteen patients of pancreatic necrosis responded to medical management and were successfully treated...
December 2016: Indian Journal of Surgery
https://www.readbyqxmd.com/read/28100428/heart-failure-with-preserved-ejection-fraction-has-a-better-long-term-prognosis-than-heart-failure-with-reduced-ejection-fraction-in-old-patients-in-a-5-year-follow-up-retrospective-study
#9
Silvana Kontogeorgos, Erik Thunström, Magnus C Johansson, Michael Fu
BACKGROUND: The issue of whether prognosis is similar between heart failure with preserved ejection fraction (HFpEF) and heart failure with reduced ejection fraction (HFrEF) remains unresolved. Because of the problem of inconsistency in the diagnostic criteria and because there is currently no lifesaving therapy available for HFpEF, it seems to be the right time to study the outcome of a clearly defined HFpEF compared with HFrEF in contemporary heart failure (HF) therapy. This study investigates 5-year-mortality and its prognostic factors in old patients with HFpEF compared with those with HFrEF...
January 5, 2017: International Journal of Cardiology
https://www.readbyqxmd.com/read/28100426/ferumoxytol-mra-for-transcatheter-aortic-valve-replacement-planning-with-renal-insufficiency
#10
Kimberly Kallianos, Travis S Henry, Yerem Yeghiazarians, Jeffrey Zimmet, Kendrick A Shunk, Elaine E Tseng, Vaikom Mahadevan, Michael D Hope
BACKGROUND: Computed tomography angiography (CTA) is the test of choice for pre-procedure imaging of transcatheter aortic valve replacement (TAVR) candidates. The iodinated contrast required, however, increases the risk of renal dysfunction in patients with pre-existing renal failure. Ferumoxytol is a magnetic resonance imaging (MRI) contrast agent that can be used with renal failure. Its long vascular resonance time allows gated MRA sequences that approach CTA in image quality. We present respiratory and cardiac gated MRA enabled by ferumoxytol that can be post-processed in an analogous fashion to CTA...
December 30, 2016: International Journal of Cardiology
https://www.readbyqxmd.com/read/28100340/-outcome-comparison-of-different-therapy-procedures-in-surgical-high-risk-elderly-patients-with-severe-aortic-stenosis
#11
Y Q Ye, Y T Wang, Z Li, M Y Wang, H Y Xu, W J Zhang, Q R Liu, G N Niu, Y J Wu
Objective: To compare the outcome of surgical high-risk elderly patients with severe aortic stenosis(SAS) treated by different therapy procedures, including transcatheter aortic valve implantation(TAVI), surgical aortic valve replacement(SAVR), and drug therapy. Methods: We retrospectively analyzed the clinical data of 242 surgical high-risk elderly (age ≥65 years old) SAS patients hospitalized in Fuwai Hospital between September 2012 and June 2015. According to the treatment method, patients were divided into TAVI group (81 cases), SAVR group (59 cases) and drug therapy group (102 cases)...
January 25, 2017: Zhonghua Xin Xue Guan Bing za Zhi
https://www.readbyqxmd.com/read/28100174/prediction-of-ineffective-elective-cardioversion-of-atrial-fibrillation-a-retrospective-multi-center-patient-cohort-study
#12
Tapio Hellman, Tuomas Kiviniemi, Tuija Vasankari, Ilpo Nuotio, Fausto Biancari, Aissa Bah, Juha Hartikainen, Marianne Mäkäräinen, K E Juhani Airaksinen
BACKGROUND: Elective cardioversion (ECV) of atrial fibrillation (AF) is a standard procedure to restore sinus rhythm. However, predictors for ineffective ECV (failure of ECV or recurrence of AF within 30 days) are unknown. METHODS: We investigated 1998 ECVs performed for AF lasting >48 h in 1,342 patients in a retrospective multi-center study. Follow-up data were collected from 30 days after ECV. RESULTS: Median number of cardioversions was one per patient with a range of 1-10...
January 18, 2017: BMC Cardiovascular Disorders
https://www.readbyqxmd.com/read/28100111/noteworthy-literature-published-in-2016-for-abdominal-organ-transplantation-anesthesiologists
#13
Tetsuro Sakai, Jeron Zerillo
More than 3000 peer-reviewed publications on the topic of liver transplantation were published in 2016. The goal of this article is to provide a concise review of pertinent literature for anesthesiologists who participate in liver transplantation. The authors selected and presented 33 articles published in 2016 on the topics of MELD policy; cardiovascular, pulmonary, and renal issues; coagulation and transfusion; anesthetic agents; hemodynamic monitors; acute liver failure; and donor issues.
January 1, 2017: Seminars in Cardiothoracic and Vascular Anesthesia
https://www.readbyqxmd.com/read/28099971/warfarin-associated-nonuremic-calciphylaxis
#14
Wesley Yung-Hsu Yu, Tina Bhutani, Rachel Kornik, Laura B Pincus, Theodora Mauro, Michael D Rosenblum, Lindy P Fox
Importance: Classic calciphylaxis associated with renal failure is a life-threatening disease. Warfarin-associated calciphylaxis without renal injury has been described, but whether it is a subset of classic calciphylaxis or a different entity remains unknown. We describe 1 case of warfarin-associated calciphylaxis, present data from 2 others from our institution, and review all cases of warfarin-associated calciphylaxis available in the literature. Our review indicates that warfarin-associated calciphylaxis is clinically and pathophysiologically distinct from classic calciphylaxis...
January 11, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28099093/patients-with-infectious-endocarditis-and-drug-dependence-have-worse-clinical-outcomes-after-valvular-surgery
#15
Anthony Lemaire, Viktor Dombrovskiy, Siavash Saadat, George Batsides, Aziz Ghaly, Alan Spotnitz, Leonard Y Lee
BACKGROUND: Patients with infective endocarditis (IE) are at high risk for post-operative morbidity and death, which might be associated with drug abuse. The purpose of this study is to evaluate the impact of drug dependence on outcomes in patients who have IE and undergo valvular surgery (VS). PATIENTS AND METHODS: The Nationwide/National Inpatient Sample 2001-2012 was queried to select patients with IE who had elective VS using International Classification of Diseases, Ninth Revision, Clinical Modification diagnosis and procedure codes...
January 18, 2017: Surgical Infections
https://www.readbyqxmd.com/read/28098884/renin-angiotensin-system-activation-accelerates-atherosclerosis-in-experimental-renal-failure-by-promoting-endoplasmic-reticulum-stress-related-inflammation
#16
Jia Yang, Xi Zhang, Xinyi Yu, Weixue Tang, Hua Gan
In this study, we investigated the association between the renin-angiotensin system (RAS), endoplasmic reticulum (ER) stress and atherosclerosis (AS) in uremic apolipoprotein E knockout (apoE-/-) mice. Mild uremia was induced by a 5/6 nephrectomy (5/6 Nx) in 10-week-old apoE-/- mice. Four weeks after nephrectomy, the mice received losartan or no treatment for 16 weeks. Sham-operated mice served as the controls. We found that uremia accelerated AS at the aortic root. The activation of ER stress and the significant upregulation of pro-inflammatory cytokines and chemokines were observed in the uremic mice...
January 12, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28098123/typhoid-associated-acute-kidney-injury-masquerading-as-a-relapse-of-takayasu-arteritis
#17
Revanasiddappa Manjunath, Anupama Kaul, Raj Kumar Sharma, Dharmendra Singh Bhadauria, Narayan Prasad, Amit Gupta
Renal dysfunction is common in Takayasu arteritis. Uncommonly, renal failure in a case of Takayasu arteritis can be due to an unrelated disease, and if the disease is a rare complication, it is even more difficult to diagnose. We report a 21-year-old male with type IV Takayasu arteritis presenting with fever and renal failure, who was diagnosed to have enteric fever- related glomerulonephritis which was successfully treated.
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28098117/denosumab-for-the-treatment-of-bisphosphonate-resistant-hypercalcemia-in-a-hemodialysis-patient
#18
Omar Dahmani, Christine Sophoclis, Malika Kebir, Djemai Bouguern, Aboubacry Sakho, Pascale Demarchi
The acronym of malignancy, iatrogenic, intoxication and immobilization, sarcoidosis, hyperparathyroidism and hyperthyroidism, milk-alkali syndrome, and paget is very helpful in diagnosing hypercalcemia. We report on a 94-year-old patient with history of end-stage renal failure secondary to benign nephroangiosclerosis, who was on maintenance hemodialysis during dialysis, his blood chemistry revealed mild hypercalcemia (2.66 mmol/L) with normal level of intact primary hyperparathyroidism (32.37 ng/mL) mandating the discontinuation of Vitamin D[3]...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28098114/a-comparison-of-health-related-quality-of-life-in-patients-with-renal-failure-under-hemodialysis-and-healthy-participants
#19
K Hajian-Tilaki, B Heidari, A Hajian-Tilaki
The objective of this study was to evaluate the health-related quality of life (QoL) of patients under hemodialysis (HD) and to compare this to healthy participants. We compared 154 patients on HD to 308 healthy age- and sex-matched controls recruited from Shahid Beheshti Hospital in Babol, Northern Iran in 2014. The data of health-related QoL in eight subscales were collected with interview using a standard short-form questionnaire of short form-36. The demographic data and dry weight and height were measured...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28098112/autosomal-dominant-polycystic-kidney-disease-study-of-clinical-characteristics-in-an-indian-population
#20
Sanjay Vikrant, Anupam Parashar
Autosomal dominant polycystic kidney disease (ADPKD) is the most common hereditary form of kidney disease. Clinical data on this multisystem disorder are scarce from developing countries. We conducted a prospective observational study of the clinical profile of ADPKD patients at a single center over a period of six years. A total of 208 patients were studied. Majority were male (60.6%) and the mean age was 45.8 ± 14.5 years. About 61.5% had early stage (Stages 1-3) of chronic kidney disease (CKD) and 38.5% had advanced CKD (Stages 4 and 5)...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
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