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:Chronic kidney disease (CKD) progression is associated with significant comorbidities and costs. In Italy, limited evidence of healthcare resource consumption and costs is available. We therefore aimed to investigate the direct healthcare costs in charge to the Lombardy Regional Health Service (RHS) for the ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-016-0291-8
更新日期：2017-04-01 00:00:00
abstract：BACKGROUND:Early initiation of therapy is warranted for patients with idiopathic membranous nephropathy (IMN) who have severe proteinuria. Therapy with tacrolimus (TAC) or intravenous cyclophosphamide (CYC) may be an option in treating such patients. METHODS:This prospective cohort study included patients with IMN who...
journal_title：Journal of nephrology
pub_type： 临床试验,杂志文章
doi：
更新日期：2008-07-01 00:00:00
abstract：BACKGROUND:Numerous data support the possible role of myeloblastin/proteinase 3 (PR3) in growth and differentiation of neutrophil granulocytes and certain monocyte subtypes. However, whether PR3 is expressed in non-myeloid cells remains a matter of debate even though recent studies clearly demonstrated its expression i...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2003-11-01 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：BACKGROUND:This study aimed to evaluate the health-related quality of life (HRQOL) and burden on family caregivers of chronic dialysis patients and to analyze which factors were associated with it. METHODS:A cross-sectional multicentric study was carried out with 221 patient/caregiver pairs. General population Short F...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2004-11-01 00:00:00
abstract：:The newly described endothelium-dependent vasoactive substances, nitric oxide and endothelins, have been the subject of intense investigative interest and have been demonstrated to promote a wide array of autocrine and paracrine functions. Recent data highlight their emerging role as potential important mediators of r...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：1997-05-01 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：:Primary renal non-Hodgkin's lymphoma is very rare in childhood. A six-year-old boy presented with bilateral non-obstructive multinodular nephromegaly and renal failure. Percutaneous needle biopsy showed large-cell lymphoma. The patient was started on chemotherapy. A right nephrectomy was done when systemic hypertensio...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2002-09-01 00:00:00
abstract：:Renal involvement is uncommon in the rare condition of Type I cryoglobulinaemia. On review of the literature most cases featured membranoproliferative glomerulonephritis on renal biopsy. In this case report, we describe a presentation of rapidly progressive glomerulonephritis that histologically demonstrated necrotisi...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：2006-05-01 00:00:00
abstract：:We report 4 cases of sepsis-induced acute renal failure (ARF) with peculiar clinical presentation in which the renal biopsy was the only clue to a correct diagnosis. We observed 66 cases of ARF in a 4-year experience. Seven (11%) were associated with sepsis; in 3 of these (4.5%) a shock was present. Clinical picture o...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：
更新日期：1998-09-01 00:00:00
abstract：:The presence of circulating permeability factors (cPFs) has been hypothesized to be associated with recurrence of focal segmental glomerulosclerosis (rFSGS) in renal allografts. The available methods to detect cPFs are complex, not easily repeatable and inappropriate to represent the anatomical characteristics of the ...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-019-00683-2
更新日期：2020-08-01 00:00:00
abstract：:Membranous nephropathy (MN) is a glomerular disease characterized by proteinuria, usually in a nephrotic range, and variable natural course. The etiology is unknown in many cases, while in some patients, MN may be secondary to infection, to other diseases, or to exposure to drugs and toxic substances. In idiopathic MN...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2007-05-01 00:00:00
abstract：:Clinical immunology laboratories play an essential role in diagnosis and monitoring of systemic lupus erythematosus (SLE). To obtain the best results in terms of diagnostic performance and clinical usefulness, the following recommendations should be fulfilled: Indirect immunofluorescence on Hep-2 cells remains the met...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2002-11-01 00:00:00
abstract：OBJECTIVE:To investigate the pregnancy outcomes for patients with stage 3-4 chronic kidney disease (CKD) and the effects of pregnancy on kidney function. METHODS:Clinical data of pregnant women with CKD in the Peking University First Hospital between January 1st 2005 and October 1st 2016 were retrospectively analysed....
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-018-0509-z
更新日期：2018-12-01 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：:Urine samples can be readily obtained from patients in everyday clinical practice. Therefore, the availability of urine allows physicians to obtain relevant clinical information in a timely manner. Since the measurement of urinary protein levels is essential in diagnosing and treating a host of diseases, the potential...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-020-00735-y
更新日期：2020-04-23 00:00:00
abstract：:To maintain divalent ion concentration within the body, a complex sensor has evolved to identify the changes in the extracellular environment and cause the surrounding cells to either secrete or absorb in response to change. An important member of these "sensors" is the calcium-sensing receptor (CaSR), which is consta...
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：: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：:Hyperuricosuric calcium urolithiasis is a condition of mixed calcium oxalate stones characterized by hyperuricosuria either in isolation or in conjunction with other risk factors for calcium oxalate stones such as hypercalciuria, hyperoxaluria, and hypocitraturia. There are three proposed physicochemical models of pat...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：10.1007/s40620-018-0469-3
更新日期：2018-04-01 00:00:00
abstract：AIM:The objective of this study was to determine whether the baseline estimated glomerular filtration rate (eGFR) level was independently associated with in-hospital mortality in generalized patients with hospital-acquired acute kidney injury (HA-AKI) in China. METHODS:All of the patients admitted to a tertiary medica...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-015-0238-5
更新日期：2016-06-01 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：:The management of rapidly progressive glomerulonephritis has been transformed over the past thirty years. It has become one of the few forms of glomerulonephritis that can be effectively treated, and today overall renal survival is as high as 70%. Effective management of patients with RPGN requires prompt and accurate...
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:Reset osmostat (RO) occurs in 36% of patients with syndrome of inappropriate antidiuretic hormone secretion (SIADH) and is not often considered when evaluating hyponatremic patients. Patients with RO are not usually treated, but recent awareness that symptoms are associated with mild hyponatremia creates a t...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.5301/jn.5000074
更新日期：2012-09-01 00:00:00
abstract：:Hereditary systemic amyloidosis is caused by deposition of genetically variant proteins as amyloid fibrils. The types that present with renal disease are usually associated with mutations in the genes for either apolipoprotein AI, apolipoprotein AII, lysozyme or fibrinogen A alpha-chain. These diseases are inherited i...
journal_title：Journal of nephrology
pub_type： 杂志文章,评审
doi：
更新日期：2003-05-01 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：BACKGROUND:Patients undergoing chronic hemodialysis (HD) are at increased risk for peripheral artery disease (PAD). Both ankle-brachial index (ABI) and ambulatory blood pressure monitoring (ABPM) in the interdialytic period have been shown to be strong predictors of all-cause mortality. METHODS:This cross-sectional st...
journal_title：Journal of nephrology
pub_type： 杂志文章
doi：10.1007/s40620-016-0322-5
更新日期：2017-06-01 00:00:00
abstract：:Cyclosporine (CsA) is among the most widely used immunosuppressants for preventing graft rejection and autoimmune diseases. However, its clinical use is hampered by its significant nephrotoxicity and effects as a cause of hypertension. The proximal tubular Na+-H+ exchanger (NHE3) is responsible for transcellular reabs...
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: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：:Highly concentrated marine polyunsaturated fatty acids (n-3 PUFA), affecting the lipids and lipophilic drugs metabolism, can interfere with cyclosporine (CyA) pharmacokinetics. This prospective, randomized and placebo-controlled, double-blind study involved 42 kidney graft recipients. From day +1, 21 pts (E) received ...
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：1998-03-01 00:00:00
abstract：BACKGROUND:Lipid peroxidation (LPO) plays a role in glomerulonephritis pathogenesis. The role of LPO in IgA nephropathy (IgAN) and the effect of prostaglandin E1 on it, is not determined. METHODS:Levels of malondialdehyde (MDA), superoxide dismutase (SOD) and vitamin E (vitE) were measured in 42 patients with IgAN and...
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：2005-05-01 00:00:00
