Abstract：
BACKGROUND:A sensitive, feasible and reproducible marker for renal function is necessary to evaluate the clinical efficacy of enzyme replacement therapy (ERT) in Fabry nephropathy. Serum creatinine has some limitations and cystatin C has been proposed, in other nephropathies, as a useful marker of renal function. The use of cystatin C as a marker of glomerular filtration rate (GFR) was investigated in Fabry patients receiving ERT. METHODS:Renal function was evaluated with serum creatinine, serum cystatin C and estimated GFR (through Modification of Diet in Renal Disease [MDRD], Cockcroft-Gault [C&G] and Hoek formulae) in 21 Fabry patients receiving ERT with agalsidase alfa for 3 years and in 13 Fabry patients receiving agalsidase alfa for 4 years. RESULTS:During years of ERT while serum creatinine remained stable, cystatin C values showed a significant, increasing trend right from the first year of ERT. CONCLUSIONS:In Fabry disease, cystatin C is a sensitive and reliable marker of renal function, and it should be taken into account when evaluating GFR trends during ERT.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Feriozzi S,Germain DP,Di Vito R,Legrand A,Ricci R,Barbey Fsubject
Has Abstractpub_date
2007-07-01 00:00:00pages
437-43issue
4eissn
1121-8428issn
1724-6059journal_volume
20pub_type
临床试验,杂志文章abstract：BACKGROUND:Mineral and bone parameters are actively managed in end-stage renal disease (ESRD). However, whether these undergo circadian variation is not known. We investigated the circadian variation of mineral and bone parameters in patients on long-term hemodialysis. METHODS:Seventeen ESRD patients on long-term hemo...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-014-0124-6
更新日期：2015-06-01 00:00:00
abstract：:In this paper we concentrate on the role of infections in IgA nephropathy both from a pathogenetic and clinic point of view. The current hypotheses as regards the role of infections in the pathogenesis of IgA nephropathy are: (a) role of particular pathogens, (b) chronic exposure to mucosal infections, (c) abnormal ha...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-016-0265-x
更新日期：2016-08-01 00:00:00
abstract：BACKGROUND:Drug-related problems (DRPs) are common in chronic kidney disease (CKD) patients. We developed a 2-step consultation including a clinical pharmacist (CP) session and a nephrologist conventional care consultation to explore the feasibility of a pilot drug-oriented disease management program in controlling iat...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000063
更新日期：2012-09-01 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:Cardiovascular (CV) diseases are the main cause of death in maintenance hemodialysis (MHD) patients. Muscle wasting and physical function decline are common in MHD patients, and significantly impair their quality of life. These can result from abnormalities in cardiac function, which can be further worsened ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0188-y
更新日期：2015-12-01 00:00:00
abstract：:Gonadal dysfunction is a frequent finding in men with chronic kidney disease and with end-stage renal disease. Testosterone deficiency, usually accompanied by elevation of serum gonadotropin concentrations, is present in 26-66% of men with different degrees of renal failure. Uremia-associated hypogonadism is multifact...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.5301/JN.2011.8481
更新日期：2012-01-01 00:00:00
abstract：BACKGROUND:Stenoses of vascular accesses are leading factors limiting access survival. Besides physical examination, screening of access stenoses is based mainly on the "dysfunction hypothesis", which states that progressive stenosis causes graft dysfunction, such as decreased flow. We tested whether Doppler ultrasonog...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2002-11-01 00:00:00
abstract：BACKGROUND AND AIMS:It has been well documented that gene and DNA alterations occur frequently in benign primary parathyroid adenomas as well as in parathyroid glands with secondary hyperplasia. However, it has not been shown whether a correlation exists between somatic DNA aberrations and clinical data. METHODS:We an...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2007-03-01 00:00:00
abstract：:It has been increasingly apparent that end-stage renal disease (ESRD) patients carry an inflammatory burden, which may play a pivotal role in the evolution of not only wasting but also of the massive increase in the relative risk of cardiovascular disease (CVD). Also increased oxidative stress (an impairment of the pr...
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：:IgA nephropathy (IgAN) is characterised by the mesangial deposition of polymeric IgA1 (pIgA1). pIgA1 production is reduced in the mucosal immune system in IgAN and increased in the marrow; this switch may be secondary to a defect in gammadeltaT cell control of IgA production. However this does not explain the mechanis...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1999-03-01 00:00:00
abstract：BACKGROUND:Chronic kidney disease (CKD) is an established risk factor for cardiovascular disease (CVD). In addition, patients with renal disease are exposed to a myriad of risk factors that increase their risk even further. The treatment of risk factors in these patients is paramount to reducing cardiovascular risk and...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-01-01 00:00:00
abstract：:We report a unusual case of idiopathic nodular glomerulosclerosis mimicking changes that develop in diabetic nephropathy. A 66-year-old non-diabetic female developed nephrotic range proteinuria and the work up was unremarkable. Light and electron microscopy showed mesangial expansion with Kimmelsteil-Wilson nodules an...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2005-09-01 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:Chronic renal failure (CRF) is associated with a tendency to atherosclerosis due to the enhanced oxidative stress and insufficient antioxidant enzyme activities such as superoxide dismutase (SOD), catalase (CAT) and paraoxonase (PON 1), together with abnormalities in lipid parameters. We determined the in vi...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2002-03-01 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：BACKGROUND:The prominent features of autosomal dominant polycystic kidney disease (ADPKD) are early development of hypertension, chronic kidney disease and cardiovascular problems. Thus, we aimed to investigate the role of endothelin, a vascular biomarker, in the clinical course of ADPKD, including renal and cardiovasc...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-018-0514-2
更新日期：2019-02-01 00:00:00
abstract：BACKGROUND:Dialysate calcium (Ca) concentration should be viewed as part of the integrated therapeutic regimen to control renal osteodystrophy and maintain normal mineral metabolism. Thus, a correct ionized calcium mass balance (Ca++MB) during hemodialysis (HD) is crucial in the treatment of renal osteodystrophy. The G...
journal_title：Journal of nephrology
pub_type： 杂志文章,随机对照试验
doi：10.5301/JN.2011.6385
更新日期：2011-11-01 00:00:00
abstract：BACKGROUND:We aimed to evaluate the relationship between abdominal aortic calcification (AAC) and renal resistive index (RRI), parameters associated with cardiovascular outcome, in non-dialysis chronic kidney disease (CKD) patients. METHODS:Seventy-seven stable patients mainly in CKD stages 3B and 4 (44 and 28%), medi...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-013-0021-4
更新日期：2014-04-01 00:00:00
abstract：BACKGROUND:Recently, some studies have emphasized the role of plasma 25-(OH)vitamin D (25OHD) levels in mineral metabolism dysregulation in chronic kidney diseases (CKDs). However, to date little attention has been paid to 25OHD metabolism abnormalities after renal transplantation (Tx). This cross-sectional study aimed...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-01-01 00:00:00
abstract：BACKGROUND:Adiponectin, a novel adipocytokine with antiinflammatory and antiatherosclerosis properties, has been found to be an inverse predictor of cardiovascular outcomes among uremic patients on hemodialysis. However, its role in uremic patients on continuous ambulatory peritoneal dialysis (CAPD) remains unclear. Th...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2008-09-01 00:00:00
abstract：:Toll-like receptors (TLRs) are a class of receptors of the innate immune system which detect pathogen-associated and danger-associated molecular patterns in order to initiate an inflammatory response. TLR2 and TLR4 downward signaling causes the production of proinflammatory cytokines that can induce insulin resistance...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-017-0432-8
更新日期：2017-12-01 00:00:00
abstract：:Evidence-based medicine provides tools to evaluate dialysis outcomes by integrating knowledge obtained from interventional and observational studies. This is illustrated in relation to three important topics: dialysis membranes, dialysis dose and anemia. Clinical trials and observational evidence support each other in...
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：:Patients with chronic kidney disease (CKD) have a greatly enhanced risk of cardiovascular morbidity and mortality. Over the past decade it has come clear that a disturbed calcium-phosphate metabolism, with Fibroblast Growth Factor-23 as a key hormone, is partly accountable for this enhanced risk. Numerous studies have...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-020-00715-2
更新日期：2020-06-01 00:00:00
abstract：:The pathogenetic role of adiponectin (ADPN) in kidney failure is not yet elucidated, since in vitro and in vivo studies have demonstrated that ADPN exerts both anti-inflammatory and pro-inflammatory effects. Starting from our previous findings demonstrating that HK-2 cells express and secrete ADPN, in this study we in...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0220-2
更新日期：2016-04-01 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：:Influenza is a commonly encountered and serious pathogen. Patients with end-stage renal disease are more susceptible to serious morbidity and mortality associated with influenza infection. Proper management of patients includes: vaccination, monitoring for symptoms and isolation of potentially infected patients as wel...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-017-0407-9
更新日期：2018-04-01 00:00:00
abstract：:John Allen, a British physician of the eighteenth century, deserves being remembered for a series of inventions, but most importantly -- from a 'medical' point of view -- for his highly praised manuscript, the 'Universae Medicinae Practicae,sive Doctissimorum Virorum de Morbis Eorumque Causis ac Remediis Judicia', whi...
journal_title：Journal of nephrology
pub_type： 传,历史文章,杂志文章
doi：
更新日期：2006-05-01 00:00:00
abstract：:Renal transplantation is associated with better survival and improved quality of life compared to maintenance dialysis. Although many sleep disorders improve or even disappear after a successful transplantation, sleep quality remains low, and the prevalence of sleep complaints, although lower than in dialysis patients...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2008-03-01 00:00:00
abstract：OBJECTIVE:It is uncertain whether increasing 25-hydroxyvitamin D (25-D) levels in chronic kidney disease (CKD) patients above those recommended by current guidelines result in progressive amelioration of secondary hyperparathyroidism. Our objective was to identify a potential therapeutic 25-D target which optimally low...
journal_title：Journal of nephrology
pub_type： 杂志文章,多中心研究
doi：10.1007/s40620-015-0186-0
更新日期：2016-02-01 00:00:00
abstract：BACKGROUND:Severe hyperhomocysteinemia is common in hemodialysis patients, who also present a dramatic increase in plasma concentrations of sulfate, one of the main products of methionine and cysteine catabolism. The aim of this study was to verify the relationship between high plasma sulfate levels and cysteine or hom...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2001-01-01 00:00:00
