Abstract:
BACKGROUND:Patients with IgA nephropathy and histological vasculitic/crescentic lesions have a poor prognosis. We performed a retrospective study to assess whether treatment with steroids and immunosuppressants would preserve renal function by healing these lesions and thereby prevent progression to glomerular sclerosis and renal failure. METHODS:Sixteen patients with IgA nephropathy and a vasculitic/crescentic glomerulonephritis diagnosed by renal histology were treated with a reducing course of prednisolone (initial dose 60 mg/day). Six patients also received cyclophosphamide (2 mg/kg/day) for three months followed by azathioprine (100 mg/day) in five patients. Ten patients received azathioprine (100 mg/day) in addition to prednisolone. The median duration of treatment was 12 months (range 5-30 months). At the end of treatment each patient had a second renal biopsy. RESULTS:Following treatment there was a significant reduction in the proportion of glomeruli with acute vasculitic lesions from a median of 17.4% (range 4.8-57.5%) to 0 (range 0-15.8%) (p=0.001). There was an increase in the proportion of globally sclerosed glomeruli from a median of 13.4% (range 0-44.4%) to 21.5% (range 0-90%) after treatment but this did not significantly differ from baseline (p=0.24). The proportion of renal cortex with chronic tubular atrophy increased from 2.55% (0.4-57.7%) to 11.3% (0.3-61%) (p=0.09). The median duration of follow-up was 30 months (inter-quartile range 6-30 months). At both 12 and 24 months there was no significant increase in serum creatinine. Four patients, however, developed end-stage renal failure between 24 and 81 months. CONCLUSION:In this retrospective study we show that treatment with steroids and immunosuppressants leads to healing of vasculitic lesions and may thus arrest progression of glomerular scarring.
journal_name
J Nephroljournal_title
Journal of nephrologyauthors
Harper L,Ferreira MA,Howie AJ,Savage CO,Richards NT,Michael J,Adu Dsubject
Has Abstractpub_date
2000-09-01 00:00:00pages
360-6issue
5eissn
1121-8428issn
1724-6059journal_volume
13pub_type
临床试验,杂志文章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 pathophysiological mechanisms underlying preeclampsia, a serious complication of pregnancy, are largely unknown. Since, on the other hand, the various risk factors are known, primary and secondary prevention with pre- and inter-pregnancy counseling should be undertaken, and a follow-up should be conducted to evalu...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2008-09-01 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:BACKGROUND:This study investigated whether modifications of placental expression of endothelin-1 (ET-1), nitric oxide synthase (NOS) and tissue factor (TF) are associated with abnormal Doppler waveforms. METHODS:Fourteen pre-term severe preeclamptic (PE) women with fetal growth restriction (FGR) and 14 normal preg nan...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2003-09-01 00:00:00
abstract::Autosomal dominant polycystic kidney disease (ADPKD) may present with a variety of extrarenal manifestations. Only 1 case of spontaneous coronary artery dissection has been reported so far in ADPKD. Here we report a case of coronary artery dissection occurring in a 38-year-old woman with ADPKD and an unremarkable medi...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2009-05-01 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:INTRODUCTION:Medullary cystic kidney disease type 1 (MCKD1; OMIM #174000) is a familial progressive tubule-interstitial nephropathy belonging to the recently defined group of autosomal dominant tubulointerstitial kidney diseases (ADTKD). CASE REPORT:A specific type of cytosine insertion in the extracellular variable n...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:10.1007/s40620-016-0282-9
更新日期:2016-06-01 00:00:00
abstract::Modern nephrology developed in Poland mostly from the internal medicine department chaired in Warsaw by the great internist prof. Witold Orłowski (1874-1966). Three of his pupils Tadeusz Orłowski, Jan Roguski and Jakub Penson independently established the foundations of clinical nephrology and renal replacement therap...
journal_title:Journal of nephrology
pub_type: 历史文章,杂志文章
doi:
更新日期:2006-05-01 00:00:00
abstract:BACKGROUND:With improvements in technology, renal biopsies have become safer and could potentially be effectively carried out in the outpatient setting. The purpose of our study is to compare the safety and cost of performing outpatient versus inpatient renal biopsies at a single Northern California pediatric hospital....
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2009-01-01 00:00:00
abstract:AIMS:The management of chronic kidney disease (CKD) complications is not always adequate in patients with a failed kidney transplant. We aimed to evaluate the frequency of CKD complications and assess whether they may lead to worse outcomes in this patient population. METHOD:We analyzed 49 kidney transplant recipients...
journal_title:Journal of nephrology
pub_type: 杂志文章,多中心研究
doi:10.1007/s40620-017-0449-z
更新日期:2018-02-01 00:00:00
abstract::Klaus Hierholzer (1929-2007) dissected various functions influenced by steroids in the distal tubule and showed that aldosterone in low doses reversed the sodium and potassium transport defect in adrenalectomized rats, through a rapid activation of Na+,K+-ATPase. Subsequent studies addressed the role of 11-beta-hydrox...
journal_title:Journal of nephrology
pub_type: 传,历史文章,杂志文章
doi:
更新日期:2009-11-01 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::Inherited distal renal tubular acidosis (dRTA) is caused by impaired urinary acid excretion resulting in hyperchloremic metabolic acidosis. Although the glomerular filtration rate (GFR) is usually preserved, and hypertension and overt proteinuria are absent, it has to be considered that patients with dRTA also suffer ...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:10.1007/s40620-016-0370-x
更新日期:2017-06-01 00:00:00
abstract::This review summarizes the plausible mechanisms of carcinogenesis, critically analyzes the literature on cancer risk and discusses issues of cancer screening in chronic dialysis patients. Despite conflicting results among various studies, there is sufficient evidence to conclude that there is a heightened incidence of...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2008-03-01 00:00:00
abstract:QUESTIONS:- To what extent can ailing kidneys adapt their functions to the demands of pregnancy? - What risks is the nephropathic patient prone to throughout pregnancy compared with those of the general population, and which specific risks does her kidney disease determine? - What risks is the baby of a nephropathic pr...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2006-03-01 00:00:00
abstract::The hallmark of IgA nephropathy (IgAN) is macroscopic hematuria coinciding with, or immediately following, a mucosal infection, usually of the upper respiratory airways. The role of mucosal pathogens has been proven in different experimental models. Mucosal (or innate) immunity acts through the recognition of pathogen...
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:AIMS:This joint document of the Italian Diabetes Society and the Italian Society of Nephrology reviews the natural history of diabetic kidney disease (DKD) in the light of the recent epidemiological literature and provides updated recommendations on anti-hyperglycemic treatment with non-insulin agents. DATA SYNTHESIS:...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:10.1007/s40620-019-00650-x
更新日期:2020-02-01 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: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:Lactic acidosis is a widely recognized, though rare, side effect of metformin. This paper describes five patients admitted to Chang Gung Memorial Hospital from 1 September 1998 to 31 May 2001 suffering severe lactic acidosis caused by metformin, and reviews the literature. PATIENTS:Five cases diagnosed as h...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2002-07-01 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::Emphysematous pyelonephritis is a rare life-threatening infection of the renal parenchyma. It usually affects unilateral kidney and occurs mostly in diabetic patients. It is characterized by the presence of gas within the renal parenchyma and requires prompt diagnosis and early aggressive therapy. Bilateral emphysemat...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:
更新日期:2000-03-01 00:00:00
abstract::Rapidly progressive glomerulonephritis (RPGN) must be diagnosed and treated quickly to prevent irreversible organ injury. The commonest cause of RPGN is anti-neutrophil cytoplasm antibody (ANCA) associated small vessel vasculitis, and the most severe is anti-glomerular basement membrane (GBM) antibody disease. In this...
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::A widely diffused method to measure the reabsorption by single segments of the nephron in humans is based on water diuresis (WD), assuming that the peak urine flow rate approximates proximal delivery (PD). However, back-diffusion of an unknown volume of solute-free water (CH2O-BD) from lumen to the interstitium even i...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:1999-09-01 00:00:00
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::Various opinions have been presented about the influence of age on the health-related quality of life (HRQOL) of patients on renal replacement therapy (RRT). Some authors sustain that age worsens HRQOL, while others show the opposite. It has been seen that a psychological adjustment occurs with aging, even when chroni...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:
更新日期:2002-05-01 00:00:00
abstract:BACKGROUND AND AIMS:Reduction of intestinal load of phosphorus is important for the prevention and treatment of chronic kidney disease (CKD)-mineral and bone disorder (MBD). However, this strategy is limited by patients' poor adherence to dietary prescription and by the existence of hidden sources of phosphorus. In add...
journal_title:Journal of nephrology
pub_type: 杂志文章
doi:10.1007/s40620-016-0306-5
更新日期:2016-12-01 00:00:00
abstract::Hemolytic uremic syndrome (HUS) is a rare disease characterized by microangiopathic hemolytic anemia, thrombocytopenia and acute renal failure. It is usually secondary to infections by strains of Escherichia coli (STEC) that produce Shiga-like toxin. In about 10% of patients, no STEC infections are reported. In these ...
journal_title:Journal of nephrology
pub_type: 杂志文章,评审
doi:10.5301/jn.5000146
更新日期:2012-11-01 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::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