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 thrombotic microangiopathy involving the glomerulus and associated with renal damage (like aHUS) cannot occur without haematuria. METHODS:The aim of this retrospective study is to analyse our experience with this approach in aHUS patients who have never previously been treated, who are currently on treatment or who have discontinued C5 inhibition. The records of all aHUS patients (children and adults) managed by or referred to our Centre from January 2009 to March 2020 were included and the analysis for the presence of haemoglobinuria was restricted to the period following primary remission. A positive test was defined as haemoglobin ≥ 1 + . Patients reporting positive urine dipstick tests underwent laboratory investigations to rule in or out the diagnosis of aHUS relapse. RESULTS:Eighty-four patients were included with 1517 determinations of haemoglobinuria during a cumulative observation period of 8904 patient-months. Haemoglobinuria for the early diagnosis of ongoing aHUS relapse shows a sensitivity of 100% and a specificity of 87.4% with a positive predictive value (PPV) of 10.5% and a negative predictive value (NPV) of 100%. The accuracy of the test was 87.6%. CONCLUSION:Haemoglobinuria is a very sensitive and acceptably specific marker of aHUS relapse. This finding and its validation may have a positive impact on patients' quality of life and on the outcome of this life threatening disease via early diagnosis of relapse.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Brambilla M,Ardissino G,Paglialonga F,Testa S,Capone V,Montini Gdoi
10.1007/s40620-021-00965-8subject
Has Abstractpub_date
2021-01-18 00:00:00eissn
1121-8428issn
1724-6059pii
10.1007/s40620-021-00965-8pub_type
杂志文章abstract：BACKGROUND:The regulation of renal hemodynamics is closely related to the L-arginine (L-Arg)/nitric oxide (NO) pathway. NO - metabolized from L-Arg - is capable of improving renal function in ischemic and toxic acute renal failure (ARF), while NO synthase (NOS) inhibition induces deterioration in renal function. The mo...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-07-01 00:00:00
abstract：:The kidney maintains systemic acid-base homeostasis through proximal tubular reclamation of filtered bicarbonate, and excretion of the daily mineral acid load by collecting duct type A intercalated cells. Impairment of either process produces renal tubular acidosis (RTA). This article will provide an overview of famil...
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：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：:The most common cause of liver disease worldwide is now non-alcoholic fatty liver disease (NAFLD). NAFLD refers to a spectrum of disease ranging from steatosis to non-alcoholic steatohepatitis, causing cirrhosis, and ultimately hepatocellular carcinoma. However, the impact of NAFLD is not limited to the liver. NAFLD h...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-020-00751-y
更新日期：2020-05-21 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: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：:Henoch-Schonlein purpura is a clinical syndrome characterized by the association of skin, joint, and gastrointestinal symptoms. Henoch-Schonlein purpura is characterized by wide-spread vasculitis. Although the clinical symptoms of this disease are characteristic, the diagnosis is not always easy to establish because o...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2005-11-01 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：:Survival analysis is the statistical method for studying the time between entry to a study and a subsequent event. Survival is often the most important outcome in both observational and intervention studies. The analysis of survival, however, is not simple because of a number of factors. The time between entry to a st...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2004-03-01 00:00:00
abstract：BACKGROUND:Accurate assessment of kidney function level is the key to the identification and management of chronic kidney disease (CKD). Glomerular filtration rate (GFR) is the best measure of overall kidney function in health and disease. There is no consensus about the method to be used routinely to measure and/or es...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2006-03-01 00:00:00
abstract：BACKGROUND:Dialysis-requiring acute kidney injury (D-AKI) is common in hospitalized patients. Many patients survive the immediate post AKI period, thus at risk of suffering long-term sequelae of AKI. Prior studies examining long term outcomes lack non-dialyzed AKI control groups. Without non-dialyzed AKI control group,...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-016-0321-6
更新日期：2016-12-01 00:00:00
abstract：BACKGROUND:IgA nephropathy is one of the most common primary glomerulonephritides, and the clinical course of almost 40% of the patients progresses to end-stage renal disease (ESRD) within 20 years. Angiotensin-converting enzyme (ACE) inhibitors and/ or angiotensin II receptor blockers (ARBs) induce a marked renoprotec...
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：2009-03-01 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：:We followed up 128 patients with crescentic glomerulonephritis (CGN), having sufficient clinical and histopathological data for a mean period of 34 +/- 28 months. There were 49 males and 79 females, mean age 22.7 +/- 14 years. We studied the effects of clinical, laboratory and histopathological parameters on kidney fu...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2002-05-01 00:00:00
abstract：BACKGROUND:Serum adiponectin is inversely linked to obesity, impaired glucose homeostasis, dyslipidemia and hypertension and has been suggested as a possible marker of cardiovascular (CV) disease in the general population. However, its role in chronic kidney disease and following renal transplantation is not well estab...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2010-01-01 00:00:00
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：: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：: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：:Rapidly progressive glomerulonephritis (RPGN) is a rare occurrence in IgA nephropathy (IgAN) in renal transplant patients on immunosuppressive therapy. RPGN post ischemia-reperfusion has not been previously reported. We report a 62 year old male patient on azathioprine therapy, 9 years after left cadaveric renal trans...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2004-01-01 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：: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：:Cyclosporine A has been empirically used for more than 12 years in the treatment of idiopathic nephrotic syndrome, in both children and adults. There is consistent evidence, from both experimental and human studies, that the highly lipophilic cyclosporine molecule diminishes or abolishes proteinuria by two differing m...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1997-01-01 00:00:00
abstract：BACKGROUND:Uremic pruritus as a symptom that affects hemodialysis (HD) patients can decrease the quality of life and increase morbidity in these patients. The aim of this study was to evaluate the effects of turmeric on uremic pruritus in HD patients. SUBJECTS AND METHODS:This was a double-blind placebo-controlled tri...
journal_title：Journal of nephrology
pub_type： 杂志文章,随机对照试验
doi：10.1007/s40620-014-0039-2
更新日期：2014-04-01 00:00:00
abstract：:IgM secreting myelomas or lymphomas, including Waldenström macroglobulinemia, are associated with a varied spectrum of renal pathology, including intracapillary hyaline deposits, cryoglobulin, membranoproliferative glomerulonephritis, amyloid, monoclonal immunoglobulin deposition disease, cast nephropathy, and lymphom...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-018-0544-9
更新日期：2018-12-01 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：AIMS:To examine the distribution of bone mineral density (BMD) in different histological groups of renal osteodystrophy. PATIENTS:We prospectively studied 62 patients, 41 men and 21 women, aged 57+/-11.5 years, who had been on hemodialysis for 60+/-55 months. The women had been amenorrheic for 13+/-4 years and 7 patie...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2000-11-01 00:00:00
abstract：INTRODUCTION:Contrast-induced acute kidney injury, also referred to as contrast-induced nephropathy (CIN), is a potentially serious renal complication associated with the use of iodinated contrast media (CM) in patients at risk. With the dramatic growth in contrast-enhanced imaging services worldwide, including procedu...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2009-05-01 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: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：:Hypertension is a risk factor for cardiovascular and renal organ damage. Environmental conditions affect the development of high blood pressure (BP), although genetic influences are also important. Current international guidelines recommend reducing dietary sodium to no more than 100 mmol (about 2.4 g sodium or approx...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2002-01-01 00:00:00
