Abstract:
:Calciphylaxis, or calcific uremic arteriolopathy, is a vascular ossification-calcification disease involving cutaneous or visceral arterioles, with ischemic damage of the surrounding tissues, usually in the setting of chronic kidney disease. Pathogenesis is still unclear and probably comprises the participation of vascular smooth muscle cells, endothelial cells and macrophages surrounded by a uremic and/or pro-calcifying environment. According to the original concept of calcific uremic arteriolopathy coined by Hans Selye, risk factors may be divided into sensitizers and challengers and their knowledge is useful in clinical practice to pre-emptively identify both uremic and non-uremic 'at risk' patients and guide treatment. Systemic calcific uremic arteriolopathy is a rarity. Cutaneous calcific uremic arteriolopathy is more frequent and clinically presents as a first phase of cutaneous hardening and erythema, followed by a second phase of ulcerations and scars; these two phases are probably associated with the initial development of arteriolar lesion and tissue ischemic damage, respectively. Clinical history, physical examination, laboratory analysis, histology and imaging are the main tools to exclude important differential diagnoses and obtain a definitive diagnosis. Treatment is generally unrewarding and consists of rigorous control of comorbid conditions, anti-oxidant, anti-inflammatory and antithrombotic strategies, avoidance of iatrogeny and wound and pain management. Prognosis remains poor in terms of morbidity and mortality. Efforts should be made towards a greater awareness of calcific uremic arteriolopathy, development of better therapies and improvement of clinical outcomes.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Oliveira TM,Frazão JMdoi
10.1007/s40620-015-0192-2subject
Has Abstractpub_date
2015-10-01 00:00:00pages
531-40issue
5eissn
1121-8428issn
1724-6059pii
10.1007/s40620-015-0192-2journal_volume
28pub_type
杂志文章,评审abstract::Recent molecular cloning experiments have identified the existence of four NBC isoforms (NBC1, 2, 3 and 4) and two NBC-related proteins AE4 and NCBE (Anion Exchanger 4 and Sodium-dependent Chloride-Bicarbonate Exchanger). Except for AE4 which does not transport Na, all are presumed to mediate the cotransport of Na+ an...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2002-03-01 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:The most frequent cause of death in hemodialysis (HD) patients is cardiovascular disease (CVD), and chronic inflammation has been identified as an epidemiologically important risk factor for CVD. Elevated levels of minor acute phase reactants, such as ceruloplasmin (Cp) and transferrin, have been related to ...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2004-09-01 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::Percutaneous transluminal angioplasty of the renal artery (PTRA) has been increasingly used over the past 20 years for treating renovascular hypertension (RVH). From the experience gathered so far it is justified to state that this technique is the first choice for patients with fibromuscular renal artery stenosis (RA...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2000-11-01 00:00:00
abstract::Organ transplantation has proven to be an effective therapeutic for a wide variety of disease states, but the chronic immunosuppression required for allograft survival increases the risk for infection and neoplasia. In the past 50 years, a wealth of experimental data has been accumulated relating to strategies to pres...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2010-05-01 00:00:00
abstract::One of the central functions of the kidney is to excrete low molecular weight, water soluble, plasma, waste products into the urine, whereas macromolecules, the size of albumin and larger, are retained. The flow of the glomerular filtrate is thought to follow an extracellular route, passing through the endothelial fen...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2003-11-01 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:To report clinical symptoms and outcome of systemic paraphenylene diamine (PPD) intoxication. METHODS:Our study was retrospective. It was conducted over 6 yrs (1994-2000) in the medical intensive care unit (ICU) of a university hospital and it concerned 19 patients hospitalized for systemic PPD intoxication...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2005-05-01 00:00:00
abstract::Membranous glomerulopathy (MGN) is a frequent cause for nephrotic syndrome in adults. In this overview, the basic pathogenic features of Heymann nephritis, a "classical" model of MGN in rats, are compared with those of human MGN. While the pathogenic antigen(s) of rat Heymann nephritis (the polyspecific receptor prote...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2000-11-01 00:00:00
abstract::We describe a patient with renal transplant who developed abdominal pain and acute nephritis in the course of the follow-up. A biopsy of colon lesions revealed CMV inclusions, renal biopsy revealed acute glomerulonephritis with positive CMV-DNA on polymerase chain reaction. The renal disease and bowel symptoms complet...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:1998-07-01 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: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:BACKGROUND:The ongoing necessity for systemic heparinization is a well-known disadvantage of continuous renal replacement therapies (CRRT), and alternative methods of anticoagulation may be required. Our aim was to evaluate, in patients with a high risk of bleeding, the possibility of an acceptable filter life with non...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2003-07-01 00:00:00
abstract:BACKGROUND:Pericardial effusion in uremic patients (UPE) was first described by R. Bright in 1836. It is generally agreed that patients require emergency pericardial drainage when tamponade signs are present, but in patients with no tamponade the optimal timing for drainage remains unclear. METHODS:To define patients ...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:10.1007/s40620-014-0107-7
更新日期:2015-02-01 00:00:00
abstract::We report the first documented case of X-linked chronic granulomatous disease (CGD) in association with IgA nephropathy in a 22 year old male with multiple soft tissue abscesses. The case highlights the potential role of staphylococcal infections in the pathogenesis of IgA nephropathy and suggests the hypothesis that ...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2002-11-01 00:00:00
abstract::The increase in progressive kidney disease, rising numbers of patients with end-stage renal disease, organ shortages for kidney transplants and poor long-term graft survival rates underline the need for better strategies to diagnose, prevent and treat renal disease. Histological analysis, based on renal biopsies and r...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:10.5301/jn.5000205
更新日期:2012-09-01 00:00:00
abstract::One of the major concerns in organ transplantation is the early detection of humoral rejection, through improved diagnostic and prognostic biomarkers. Long-term survival of renal allografts is significantly lower in recipients developing donor-specific anti-HLA antibodies (DSAs) either pretransplant or posttransplant....
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:10.5301/jn.5000240
更新日期:2013-03-01 00:00:00
abstract:BACKGROUND:The success of guidelines depends largely on effective implementation and uptake in clinical practice. The Optimal Renal Anemia Management Assessment (ORAMA) study investigated the impact of European Best Practice Guideline (EBPG) prompting on patient outcomes. METHODS:ORAMA was a prospective, international...
journal_title:Journal of nephrology
pub_type: 杂志文章,多中心研究,随机对照试验
doi:
更新日期:2009-09-01 00:00:00
abstract::Hepatitis C virus (HCV) infection is now considered a systemic disease due to the occurrence of extra-hepatic manifestations. Among these, the renal involvement is frequent. HCV infection, in fact, is strongly associated with proteinuria and chronic kidney disease (CKD) and negatively affects the prognosis of renal pa...
journal_title:Journal of nephrology
pub_type: 杂志文章,实务指引
doi:10.1007/s40620-018-0523-1
更新日期:2018-10-01 00:00:00
abstract:BACKGROUND:The therapeutic effects of immunosuppressive agents such as cyclosporin A, mycophenolate mofetil, rituximab and sirolimus on primary focal segmental glomerulosclerosis (FSGS) remain controversial. The present study was intended to review and analyze the treatment results of immunosuppressive agents for prima...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:10.5301/jn.5000092
更新日期:2012-09-01 00:00:00
abstract:BACKGROUND:Changes in podocyte phenotype and function are characteristic of proteinuric glomerular diseases. Integrin-linked kinase (ILK) functions as a common downstream effector in proteinuric diseases. In addition, ILK was shown to interact with the Wnt signaling pathway. Here, we investigated ILK expression as well...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:10.5301/jn.5000017
更新日期:2012-05-01 00:00:00
abstract:BACKGROUND:The endogenous inhibitor of nitric oxide synthase (NOS), asymmetric dimethylarginine (ADMA), is implicated in endothelial dysfunction and is a marker of renal disease progression and cardiovascular (CV) complications. Various cell species exhibit the enzymatic system that generates and degrades this methylar...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2007-09-01 00:00:00
abstract::Lupus is now regarded as a syndrome which results from several related auto-immune processes, although the exact mechanisms of how the disease arises in susceptible individuals remain obscure. When the San Carlo meetings began in 1968, much less was known about the pathogenesis of autoimmunity, but the presence of aut...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:1999-07-01 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::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:Carotid artery intima-media thickness (CIMT) has been used as a marker of atherosclerosis. An insertion/deletion (I/D) polymorphism of the angiotensin-converting enzyme (ACE) gene is associated with various cardiovascular diseases. This study is aimed at evaluating early atherosclerotic involvement of caroti...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:1999-07-01 00:00:00
abstract::Ferumoxytol is a new product approved for intravenous use by the US Food and Drug Administration (FDA) in the treatment of iron deficiency anemia in adults with chronic kidney disease. This approval was based on data from 3 open-label, randomized, controlled clinical trials. In all of these trials, ferumoxytol was wel...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:10.5301/jn.5000025
更新日期:2011-11-01 00:00:00
abstract:BACKGROUND:Phosphate control is a crucial treatment goal in end-stage renal disease, but poor patient adherence to phosphate binder therapy remains a challenge. This study aimed to estimate the extent of phosphate binder adherence in hemodialysis patients and to identify potential determinants. METHODS:Phosphate binde...
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/s40620-014-0062-3
更新日期:2014-12-01 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