Abstract：
:Preemptive isolated liver transplantation (PLTX) can cure the metabolic defect in primary hyperoxaluria type 1 (PH1) but there are no uniformally accepted recommendations concerning the timing of this transplantation procedure. We have performed PLTX successfully in 4 children (age 3-9 years) with PH1 with no mortality or morbidity due to the transplantation procedure. Plasma and urinary oxalate levels normalised rapidly and renal function remained stable including one patient with advanced chronic renal failure who showed a stable course for more than 24 months. Although treatment must be individualised in this severe metabolic disorder and PLTX has to be viewed as invasive procedure, we feel PLTX should be offered and discussed not too late in the treatment of PH1 to prevent or at least delay the progression to end stage renal disease and systemic oxalosis.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Kemper MJ,Nolkemper D,Rogiers X,Timmermann K,Sturm E,Malago M,Broelsch CE,Burdelski M,Müller-Wiefel DEsubject
Has Abstractpub_date
1998-03-01 00:00:00pages
46-8eissn
1121-8428issn
1724-6059journal_volume
11 Suppl 1pub_type
杂志文章,评审abstract：BACKGROUND:Gut-derived uremic toxins have been associated with adverse outcomes in chronic kidney disease (CKD). Alterations in bowel habits, including constipation, seem to play an additional role in uremic toxicity. The aim of this study is to investigate the association of bowel habits with gut-derived uremic toxins...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-020-00819-9
更新日期：2020-10-01 00:00:00
abstract：BACKGROUND:In the general population and in hemodialysis patients epicardial adipose tissue (EAT) has been associated with increased mortality and cardiovascular events. Weight loss and lipid lowering therapies reduced EAT in the general population. It is unknown whether sevelamer, a phosphate (Pi) binder that lowers c...
journal_title：Journal of nephrology
pub_type： 杂志文章,随机对照试验
doi：10.1007/s40620-016-0310-9
更新日期：2016-10-01 00:00:00
abstract：:Sarcoidosis is a multisystem disorder of unknown etiology, which may occur in all tissues and organs of the body. Clinical presentation may be quite variable and affected by race. The most commonly affected system is the lungs with >95% of patients displaying such involvement. Renal involvement is uncommon (<2%) and m...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2003-09-01 00:00:00
abstract：BACKGROUND:Atypical haemolytic uremic syndrome (aHUS) is at high risk of relapse at any time, therefore patients require lifelong monitoring. The most appropriate way to monitor patients is not yet clear. Patients could be monitored for relapse by urine dipstick testing for haemoglobinuria based on the hypothesis that ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-021-00965-8
更新日期：2021-01-18 00:00:00
abstract：:A 59 year-old woman showed rapidly progressive glomerulonephritis during immunotherapy for metastatic renal cell carcinoma. She received unilateral nephrectomy and cytotoxic T lymphocyte (CTL) therapy for the treatment of retroperitoneal lymph node metastasis of renal cell carcinoma. With CTL therapy, her retroperiton...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-07-01 00:00:00
abstract：:Cutaneous periarteritis nodosa (PAN) is a clinical feature characterized by chronic, benign course; its pathogenesis is unknown. In patients submitted to renal transplantation cutaneous PAN is a rare complication. We report a case of cutaneous PAN associated with the reappearance of hepatitis B antigen 16 years after ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2001-09-01 00:00:00
abstract：:Sclerosing encapsulating peritonitis (SEP) is a serious complication of long-term continuous ambulatory peritoneal dialysis (CAPD), very likely related to a persisting expression of the transforming growth factor beta1 (TGFbeta1) gene on peritoneal mesothelial cells. We report the case of a 67-year-old uremic woman wh...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：1999-11-01 00:00:00
abstract：:Renal fibrogenesis can be induced by several injury mechanisms in different renal diseases, but ultimately produces identical fibrotic changes in the kidney. Recently, a number of agents that can inhibit extracellular matrix (ECM) accumulation have been studied, suggesting a therapeutic utility in the treatment of fib...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2004-07-01 00:00:00
abstract：BACKGROUND:Until now, the survival of iliac vein tunneled cuffed catheters (TCCs) used in elderly patients has not been fully investigated. Accordingly, we evaluated their use in elderly hemodialysis patients with no other venous access options. METHODS:A total of 70 elderly patients with iliac vein TCCs undergoing ch...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0198-9
更新日期：2016-02-01 00:00:00
abstract：BACKGROUND:Proteinuria results from increased glomerular permeability and is associated with retraction and effacement of the highly specialized interdigitating podocyte foot processes. In podocytes (glomerular epithelial cells [GECs]), p130Cas localizes diffusely to the cytoplasm and connects focal adhesion proteins a...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000261
更新日期：2013-09-01 00:00:00
abstract：OBJECTIVE:Chronic kidney disease (CKD) pregnancies are at high risk of developing adverse outcomes. In non-pregnant subjects with CKD, higher urinary IgM levels are associated with poor renal survival and higher rates of cardiovascular deaths. In this study, we assessed whether urinary IgM levels are associated with an...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-018-0536-9
更新日期：2019-04-01 00:00:00
abstract：:Cardiovascular disease is the leading cause of the poor long-term survival of patients with chronic kidney disease (CKD). Anemia, which is a frequent and early complication of CKD, not only impairs patients' quality of life, but also is an independent risk factor for adverse cardiovascular outcomes. This may be due to...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2007-11-01 00:00:00
abstract：INTRODUCTION:We analyzed renal histologic and immunohistologic findings in children with nephrotic syndrome (NS) who did (n=5) or did not (n=17) develop cyclosporine A (CyA) nephropathy despite appropriately low serum CyA concentrations being maintained over 2 years. METHODS:To discriminate embryonic-type from mature ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/JN.2010.5793
更新日期：2011-07-01 00:00:00
abstract：BACKGROUND:The G-1082A polymorphism of the interleukin-10 (IL-10) gene has been associated with modified gene expression and the progression of primary IgA nephropathy (IgAN). In the present study, we evaluated its influence on recurrent IgAN after renal transplantation. METHODS:We studied 103 patients who suffered fr...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2008-11-01 00:00:00
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 e...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.5301/jn.5000214
更新日期：2013-07-01 00:00:00
abstract：BACKGROUND:NKF K-DOQI clinical practice guidelines recommend a regular monitoring of vascular access (VA) through several methods, such as VA recirculation (AR). Assessment of AR is crucial to avoid inefficiency of hemodialysis (HD). A non-urea based method has been developed (ultrasound dilution Transonics Hemodialysi...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2003-11-01 00:00:00
abstract：BACKGROUND:Renal tubular epithelial cells (TECs) function as antigen-presenting cells because they constitutively express MHC class II molecules and have the ability to present peptide antigen to CD4+T cells. However, the costimulatory signals provided by TECs for optimal T-cell activation have not been fully character...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2006-07-01 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：BACKGROUND:Congenital nephrotic syndrome is an uncommon disorder that may be caused by several diseases. These may be inherited, sporadic, acquired or part of a general malformation syndrome. METHODS:We reviewed the clinical characteristics, pathologic findings, and results of medical management in 30 infants who pres...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2001-03-01 00:00:00
abstract：BACKGROUND:Ischemia-reperfusion (IR) causes acute kidney injury (AKI), and ischemia pretreatment may exert protection. Mitogen-activated protein kinase kinase 3 (MKK3), which is involved in the signal transduction pathway in IR-induced injury, is a potential target of miR-21. We aimed to verify the targeting regulation...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0217-x
更新日期：2016-02-01 00:00:00
abstract：:Clinically apparent venous thromboembolism (VTE) occurs in approximately 7% of patients with membranous nephropathy. Hypoalbuminemia at diagnosis is an independent risk factor for VTE, and risk increases significantly as albumin falls. Optimal prophylactic and treatment anticoagulation regimens in the nephrotic syndro...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-018-0552-9
更新日期：2019-08-01 00:00:00
abstract：BACKGROUND:The aim of this work was to investigate the benefit of basiliximab induction therapy in living-related-donor kidney transplantation. METHODS:One hundred adult recipients of a first kidney allograft were randomized into two treatment groups, one to receive basiliximab and the second as a control. All patient...
journal_title：Journal of nephrology
pub_type： 临床试验,杂志文章,随机对照试验
doi：
更新日期：2003-05-01 00:00:00
abstract：:The kidney does not usually present specific lesions in cystic fibrosis (CF), although in recent years renal involvement has been reported, particularly amyloidosis and immune complex glomerulonephritis. IgA nephropathy is rare. We report four cases of IgA nephropathy out of five renal biopsies performed in the last t...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：1999-01-01 00:00:00
abstract：:Recently, two independent teams detected presumed hepatitis agents, which were designated HGV and hepatitis GB virus C; they represent a new genus in the family Flaviviridae. The most accurate way to assess the epidemiology of GBV-C/HGV infection remains the combination of RT-PCR and anti-GBV-C/HGV E2 techniques, to d...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1999-05-01 00:00:00
abstract：BACKGROUND:Causes of hypertension and cardiac hypertrophy in hemodialysis (HD) patients are multiple, but the role of fluid overload appears to be crucial. Short daily HD (sDHD = 2 hr x 6/week) seems to allow reductions in left ventricular mass (LVM) through the reduction of extracellular water (ECW). Better cardiovasc...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2006-01-01 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：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 u...
journal_title：Journal of nephrology
pub_type： 临床试验,杂志文章
doi：
更新日期：2007-07-01 00:00:00
abstract：BACKGROUND:Cardiac surgery-associated acute kidney injury (CSA-AKI) is associated with high risk for complications and mortality. Whether renin-angiotensin system (RAS) inhibitor should be continued or withdrawn in patients with long-term use before cardiac surgery has been lack of consensus. METHODS:We performed this...
journal_title：Journal of nephrology
pub_type： 杂志文章,多中心研究
doi：10.1007/s40620-019-00657-4
更新日期：2019-12-01 00:00:00
abstract：BACKGROUND:The risks connected with dialysis fluid contamination are well established. Epidemiological studies have demonstrated just how difficult it is to obtain permanently satisfactory quality standards. Therefore, dialysis centers must have effective and regular quality control programs, particularly with the grow...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2004-07-01 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
