Abstract：
:During Fabry disease, progressive glycosphingolipid deposition in the kidney causes gradual deterioration of renal function with proteinuria, uremia and hypertension. This results in end-stage renal disease (ESRD) which is one of the leading causes of morbidity and premature mortality in affected patients. Given the excellent graft and patient survival generally nowadays, kidney transplantation is the first choice to correct renal dysfunction and improve the overall prognosis of patients with renal failure because of Fabry disease. The benefit of enzyme-replacement therapy (ERT) in kidney transplanted Fabry patients has been controversially discussed and long-term trials focusing on the effectiveness of agalsidase in this patient population are needed.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Cybulla M,Kurschat C,West M,Nicholls K,Torras J,Sunder-Plassmann G,Feriozzi Sdoi
10.5301/jn.5000214subject
Has Abstractpub_date
2013-07-01 00:00:00pages
645-51issue
4eissn
1121-8428issn
1724-6059pii
C211B1BD-9B5A-4CEF-AA92-1C3836B63484journal_volume
26pub_type
杂志文章,评审abstract：BACKGROUND:Inadvertent sodium (Na(+)) flux may occur during renal replacement therapy (RRT) in ICU. The objective of this study was to estimate sodium flux during RRT. METHODS:Between September 2011 to December 2012 we studied 60 ICU patients receiving extended daily dialysis (EDD, Fresenius 4008S) or continuous renal...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-014-0041-8
更新日期：2014-08-01 00:00:00
abstract：:Aortic intimal sarcoma is a rare tumor with poor prognosis. The most common manifestations are thromboembolic phenomena and vascular obstruction. We present a case of aortic intimal sarcoma causing bilateral renal artery stenosis which manifested as resistant hypertension and acute kidney inury. Multiple attempts to s...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000283
更新日期：2013-09-01 00:00:00
abstract：:The abnormal metabolism of uric acid results in many disease such as chronic kidney disease, hyperuricemia, nephrolithiasis, gout, hypertension, vascular disease and so on. Serum uric acid levels are maintained by the balance between production and elimination. There are many factors that maintain the balance of serum...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-018-0546-7
更新日期：2019-04-01 00:00:00
abstract：:Rapidly progressive glomerulonephritis (RPGN) must be diagnosed and treated quickly to prevent irreversible organ injury. The commonest cause of RPGN is anti-neutrophil cytoplasm antibody (ANCA) associated small vessel vasculitis, and the most severe is anti-glomerular basement membrane (GBM) antibody disease. In this...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2004-11-01 00:00:00
abstract：BACKGROUND:The recent multicenter, randomized, open-label INDEPENDENT study demonstrated that sevelamer improves survival in new to hemodialysis (HD) patients compared with calcium carbonate. The objective of this study was to determine the cost-effectiveness of sevelamer versus calcium carbonate for patients new to HD...
journal_title：Journal of nephrology
pub_type： 杂志文章,多中心研究,随机对照试验
doi：10.1007/s40620-014-0122-8
更新日期：2015-10-01 00:00:00
abstract：:Some patients with essential hypertension manifest increased urinary albumin excretion (UAE). Hypertensive patients with microalbuminuria manifest abnormal circadian variation of blood pressure, increased serum levels of LDL-cholesterol and lipoprotein(a), a greater rise of serum insulin in response to an oral glucose...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1997-07-01 00:00:00
abstract：BACKGROUND:Reduced bone mineral density (BMD) is common in end-stage renal disease (ESRD) patients and predicts outcomes. The chronic kidney disease-mineral bone disorder contributes to low BMD in ESRD; however, the impact of classical risk factors for osteoporosis in the general population, such as body weight and fat...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000185
更新日期：2013-05-01 00:00:00
abstract：:Inherited distal renal tubular acidosis (dRTA) is caused by impaired urinary acid excretion resulting in hyperchloremic metabolic acidosis. Although the glomerular filtration rate (GFR) is usually preserved, and hypertension and overt proteinuria are absent, it has to be considered that patients with dRTA also suffer ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-016-0370-x
更新日期：2017-06-01 00:00:00
abstract：:Gitelman syndrome (GS), an inherited disorder due to loss of function in mutations of the gene encoding the distal convoluted tubule Na-Cl cotransporter (NCCT), is characterized by hypokalemia metabolic alkalosis, hypomagnesemia and hypocalciuria. A 18-year-old girl was admitted to our hospital with a history of muscl...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2010-09-01 00:00:00
abstract：AIMS:Nonsteroidal anti-inflammatory drugs (NSAIDs) are the cornerstone of pain management. There are no detailed data on NSAIDs use in Poland, especially in patients with chronic kidney disease (CKD). The aim of this study was to evaluate the frequency, circumstances, and causes of NSAIDs use as well as knowledge of th...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-016-0352-z
更新日期：2017-12-01 00:00:00
abstract：OBJECTIVE:Intradialytic exercise (IDE) is not yet a routine practice for hemodialysis patients, the lack of guidelines supporting it being a major reason. This systematic review and meta-analysis of aerobic IDE interventions examined the efficacy of IDE regarding quality of life (QOL), serum phosphorus, dialysis effici...
journal_title：Journal of nephrology
pub_type： 杂志文章,meta分析
doi：10.1007/s40620-018-00565-z
更新日期：2019-08-01 00:00:00
abstract：:Von Hippel-Lindau (VHL) disease is a rare autosomal dominant disorder characterized by multifocal and bilateral renal cell carcinoma and cysts, retinal angiomas, hemangioblastoma of the central nervous system, pheochromocytoma, epididymis cystoadenoma, pancreatic cysts and/or islet cell tumors. However, phenotypic man...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-03-01 00:00:00
abstract：BACKGROUND:This study was undertaken to examine whether there is an association between parity and age at first birth and risk of death from chronic renal failure (CRF). STUDY DESIGN:Prospective cohort study. SETTING AND PARTICIPANTS:The study cohort consisted of 1 292 462 women who had a first and singleton childbir...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000171
更新日期：2013-03-01 00:00:00
abstract：:Although several in vitro studies and clinical observations suggest that ACE-inhibitors (ACE-I) are a promising treatment for IgA nephropathy (IgAN), meta-analysis of published data is not yet conclusive. Therefore, a European double-blinded, prospective, randomized therapeutic trial was designed to evaluate ACE-I tre...
journal_title：Journal of nephrology
pub_type： 临床试验,杂志文章,多中心研究,随机对照试验
doi：
更新日期：2001-11-01 00:00:00
abstract：BACKGROUND:Peripheral artery disease (PAD) represents a major complication in chronic kidney disease (CKD) patients, but its early diagnosis is still problematic. Traditionally, ankle-arm blood pressure index (API) determined by means of a simple hand-held unidimensional Doppler device has been used for noninvasive dia...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2009-07-01 00:00:00
abstract：QUESTIONS:- To what extent can ailing kidneys adapt their functions to the demands of pregnancy? - What risks is the nephropathic patient prone to throughout pregnancy compared with those of the general population, and which specific risks does her kidney disease determine? - What risks is the baby of a nephropathic pr...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2006-03-01 00:00:00
abstract：:A 64-year-old man was hospitalized in 2002 with symptoms of stupor, weakness, and renal colic. The clinical examination indicated borderline hypertension, small masses in the glutei, and polyuria. Laboratory tests evidenced high serum concentrations of creatinine, calcium, and phosphate. Imaging assessments disclosed ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0228-7
更新日期：2016-02-01 00:00:00
abstract：:Hyperlipidaemia complicating the nephrotic syndrome is characterised by elevated levels of total and LDL cholesterol, often with hypertriglyceridaemia and low HDL cholesterol levels. The underlying mechanisms are complex but involve abnormalities of both lipoprotein synthesis and catabolism. Experience to date suggest...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2001-11-01 00:00:00
abstract：BACKGROUND:The relationship between malnutrition in hemodialysis (HD) patients with a chronic inflammatory condition and the expression levels of leukocyte integrins and their adhesiveness to fibronectin was investigated. METHODS:Subjective global assessment, albumin and body mass index were used as nutritional marker...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2010-03-01 00:00:00
abstract：BACKGROUND:Functional failure of the peritoneal membrane is the most serious problem in long-term continuous ambulatory peritoneal dialysis (CAPD). Transforming growth factor-beta (TGF-ss) is one of the key mediators of fibrosis in some organs, and is thought to be involved in peritoneal alterations. In this study, we ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2003-01-01 00:00:00
abstract：:A symposium on kidney stones and mineral metabolism held on December 2017 in Brussels, Belgium was the first international multidisciplinary conference of the International Collaborative Network on Kidney Stones and Mineral Metabolism. This meeting addressed epidemiology, underlying pathophysiological mechanisms, gene...
journal_title：Journal of nephrology
pub_type：
doi：10.1007/s40620-019-00587-1
更新日期：2019-10-01 00:00:00
abstract：BACKGROUND:The response of proximal convoluted tubules (PCTs) to angiotensin II is mediated by specific type 1 receptors found on both apical and basolateral surface membrane cells. After ligand association with type 1 receptors, different signaling pathways are triggered and determine changes in fluid absorption (Jv)....
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2007-01-01 00:00:00
abstract：:The combination of hypomagnesemia and hypocalciuria is the phenotypic signature of two distinct genetic renal tubular transport disorders: Gitelman's syndrome and autosomal dominant isolated renal magnesium wasting. In the past 5 years the genetic defects underlying these disorders have been elucidated through positio...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2003-03-01 00:00:00
abstract：BACKGROUND:Acute kidney injury (AKI) following acute ST elevation myocardial infarction (STEMI) is associated with adverse outcomes. The recently proposed KDIGO criteria suggested modifications to the consensus classification system for AKI, namely lowering the threshold of increase in absolute serum creatinine and ext...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-017-0461-3
更新日期：2018-06-01 00:00:00
abstract：:The hallmark of IgA nephropathy (IgAN) is macroscopic hematuria coinciding with, or immediately following, a mucosal infection, usually of the upper respiratory airways. The role of mucosal pathogens has been proven in different experimental models. Mucosal (or innate) immunity acts through the recognition of pathogen...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2010-11-01 00:00:00
abstract：:Some antihypertensive drugs may have a renoprotective effect, that is partially independent of their ability to reduce blood pressure. ACE-inhibitors are safe and effective agents that are capable of reducing proteinuria and preventing CRF progression. The results of the AIPRI extension study suggest that they may als...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1998-11-01 00:00:00
abstract：BACKGROUND:The effect of uric acid on nocturnal dipping in hypertensive patients is unknown. We analyzed the specific relationship between uric acid and nocturnal dipping status in newly diagnosed essential hypertensive patients with normal renal function. METHODS:Two hundred fifteen patients with newly diagnosed esse...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2008-05-01 00:00:00
abstract：INTRODUCTION:The literature on the clinical effectiveness of treatments for steroid-resistant nephrotic syndrome (SRNS) is very limited. The available evidence suggests a beneficial effect of cyclosporine on remission rates. Mycophenolate mofetil (MMF) represents a promising therapeutic alternative without nephrotoxici...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.2011.6327
更新日期：2011-03-01 00:00:00
abstract：:Chronic renal failure (CRF) is associated with a 20-fold increased risk of cardiovascular death, two principal mechanisms being: sudden, arrhythmic death associated with left ventricular hypertrophy, and ischaemic heart disease, associated with accelerated atherosclerosis. In recent years, the vascular endothelium has...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2000-03-01 00:00:00
abstract：BACKGROUND:Uromodulin (Tamm-Horsfall protein) is the most abundant urinary protein in healthy individuals. Despite 60 years of research, its physiological role remains rather elusive. Familial juvenile hyperuricemic nephropathy and medullary cystic kidney disease Type 2 are autosomal dominant tubulointerstitial nephrop...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-014-0079-7
更新日期：2014-06-01 00:00:00
