BACKGROUND: There is a growing literature regarding sclerotic and panniculitic cutaneous conditions seen in patients with end-stage renal disease (eg, calciphylaxis and soft tissue calcification). Nephrogenic fibrosing dermopathy (NFD) is a recent designation to describe cutaneous findings in patients with end-stage renal disease who developed sclerotic plaques with scleromyxedema-like histologic ...

BACKGROUND: The identification of nephrin and CD2AP, podocyte proteins which modulate the properties of glomerular barrier selectivity, has made it possible to unravel the mechanisms undergoing foot process effacement and proteinuria. Here we explored the role of nephrin and CD2AP together with the integrity of the slit diaphragm in the ...

There are few studies that examine, prospectively, the epidemiological profile of glomerulopathy (GP) and its clinicopathological correlation. All patients referred to Al-Amiri renal center in Kuwait from January 1st, 1995 to December 31st, 2001 were screened for GP. Detailed clinical data were collected and serological markers were done. Renal biopsy ...

The aim of the study was to determine the efficacy of an individual treatment schedule in patients with systemic vasculitis. Clinical, laboratory, morphological and immunological data before and after treatment were followed in 18 patients: eight with microscopic polyangiitis, two with Wegener's granulomatosis, four with leukocytoclastic vasculitis and four with ...

We determined the natural history of immunoglobulin A nephropathy (IgAN) among patients who presented with mild proteinuria (0.2 to 0.4 g/day), and factors associated with development of adverse clinical events, defined as proteinuria 竕ｧ 1.0g/day, blood pressure > 130/80mmHg, serum creatinine 竕ｧ 1.4mg/dl. We did analyzed data from 27 patients(mean ...

The aim of this study was to investigate the effect of colchicine therapy on the outcome of amyloid nephropathy of familial Mediterranean fever (FMF) in childhood. The diagnosis of amyloidosis type AA was confirmed by renal biopsy in 38 patients. During a mean follow-up period of 30.5 months (range 6-88 ...

PURPOSE: To determine the relapse rate and outcome in patients with Wegener's granulomatosis treated with daily cyclophosphamide and glucocorticoids to induce remission followed by methotrexate for remission maintenance. METHODS: We performed an open-label prospective study in 42 patients with active Wegener's granulomatosis. All patients were treated with a standardized regimen. ...

BACKGROUND: The spectrum of proteinuric renal disease in older adults remains incompletely defined. The purpose of the present study was to determine if differences exist in diagnostic approach, etiology, therapy, and outcome between older (> or = 60 years) and younger (<60 years) patients referred for evaluation of proteinuria. METHODS: ...

BACKGROUND: Chronic kidney diseases, particularly if presenting with significant proteinuria, are commonly associated with substantial alteration of serum lipid levels. Experimental evidence suggests that lipid abnormalities may contribute to the progression of kidney disease. However, studies in humans on the subject are scarce. METHODS: In a prospective, controlled open-label study, ...

We report the long-term follow-up of two patients with type II membranoproliferative glomerulonephritis (MPGN). One Patients was treated with high-dose alternate-day prednisolone and the other with dipyridamole. Both had favorable clinical courses over 8.5 and 15.5 years of follow-up, respectively. One patient who showed diffuse proliferative changes lost the urinary ...

BACKGROUND: Hypertensive renal damage has become one of the most important causes of end-stage renal failure (ESRF) in Western countries. Affected patients rarely have a kidney biopsy and their diagnoses therefore remain uncertain. The objective of the present study was to examine patients suspected of renal glomerular disease, which at ...

BACKGROUND: Immunoglobulin M (IgM) nephropathy is an idiopathic glomerulonephritis with mesangial hypercellularity and diffuse IgM deposits. METHODS: We studied clinical presentation, morphological findings, and prognostic factors in 110 patients with IgM nephropathy without systemic diseases. The series included both pediatric and adult patients with nephrotic syndrome (NS) or minor urinary ...

Hypercalcemic nephropathy has been classified as a tubulointerstitial renal disease. The presence of glomerular pathologic findings attributable to hypercalcemia has been observed in only a few patients and therefore has been considered an unusual finding. In the current study, calcium deposition within glomeruli was investigated in 2 patients with extreme ...

BACKGROUND: Excess cardiovascular mortality complicates systemic rheumatic disease, suggesting an accelerated atheromatous process, which it has been proposed relates to the vascular inflammation common in such diseases. Impaired endothelium dependent vasodilatation is an early marker of atheromatous disease. It has previously been shown that such endothelial cell dysfunction (ECD) occurring ...

Continuous efforts have been made to find out precise and simple method for determination of glomerular filtration rate (GFR). Cystatin C (cysteine proteinase inhibitor = CyC) is a low molecular weight (LMW) protein which is produced constantly by all nucleated cells independently of different pathological conditions and eliminated from the ...

BACKGROUND: Cocaine-induced lesions may cause extensive destruction of the osteocartilaginous structures of the nose, sinuses, and palate that mimics the clinical picture of other diseases. METHODS: From January 1991 to September 2001 25 patients with cocaine-induced midline destructive lesions were observed at the Department of Otorhinolaryngology of the University of ...

BACKGROUND: Schamberg's purpura (progressive pigmentary dermatosis or pigmented purpuric dermatosis) is uncommonly described in preadolescent children. PATIENTS AND METHODS: A retrospective review of cases of Schamberg's purpura with onset before 10 years of age was undertaken. Clinical and histopathologic data, as well as analytical studies including hemogram, erythrocyte sedimentation rate, ...

Three hundred fifteen (315) elderly (> or = 60 years) patients with clinical renal diseases were evaluated for the evidence of glomerular diseases between November 1998 to June 2002. Glomerular diseases (GN) were observed in 20.6% (65/315) of the elderly patients. The age of the patients (male 56; female 9) ...

We determined the natural history of IgA nephropathy (IgAN) among patients who presented with proteinuria, and factors associated with the development of clinical events, namely blood pressure(BP)竕ｧ130/85 mmHg, serum creatinine 竕ｧ1.4mg/dl. We analyzed data from 16 patients(mean age 35 ﾂｱ 14 years) with IgAN accompanied by proteinuria between 1990 and ...

To minimize the adverse effects of high-dose administration of steroids and cyclophosphamide in patients with myeloperoxidase (MPO) antineutrophil cytoplasmic antibody (ANCA), granulocytapheresis (GCAP) or leukocytapheresis (LCAP) was performed to reduce inflammation. Four patients with rapidly progressive glomerulonephritis (RPGN) and one patient with pulmonary hemorrhage due to MPO-ANCA-associated vasculitis were treated ...

OBJECTIVE: To assess the influence of interleukin-8 (IL-8), epithelial cell-derived neutrophil-activating peptide (ENA-78), and regulated upon activation normal T cell expressed and secreted (RANTES) gene polymorphisms in the susceptibility and clinical expression of patients fulfilling classification criteria for Henoch-Schönlein purpura (HSP). METHODS: Fifty patients (25 men) from Northwest Spain with ...

OBJECTIVE: To study the effect of the chimeric monoclonal anti-tumour necrosis factor alpha (TNF-alpha) antibody infliximab in the induction of remission in six patients refractory to standard treatment with cyclophosphamide and corticosteroids. In four patients, other measures for treating refractory Wegener's granulomatosis (WG) that have been advocated previously, i.e. intensified ...

BACKGROUND: The clinical relevance of distinguishing two types of glomerulonephritis (GN) with non-amyloid organized immunoglobulin (Ig) deposits-fibrillary GN (FGN) and immunotactoid (microtubular) GN (IT/MTGN)-on the basis of ultrastructural organization, is debated. METHODS: Twenty-three patients with organized glomerular Ig deposits were classified into two groups based on the fibrillar or microtubular ...

OBJECTIVES: Wegener's granulomatosis is a granulomatous and necrotizing vasculitis that classically involves the respiratory and renal systems. The goal of the study was to define clinical and pathological characteristics in a subgroup of patients with the changes of Wegener's granulomatosis involving the orbit. STUDY DESIGN: Retrospective study. METHODS: A database ...

We investigated whether leukocyte cell surface TNF receptors (cTNFR) and plasma soluble TNF receptors (sTNFR) could serve as predictors of childhood steroid-resistant nephrotic syndrome (SRNS) during the early stage of diagnosis. We recruited 39 nephrotic children for studies correlating plasma sTNFR and leukocyte cTNFR with disease activity and therapeutic response. ...

Anti-myeloperoxidase (MPO) antibodies are associated with the development of anti-neutrophil cytoplasmic antibody (ANCA)-related vasculitis. The imbalance between the protease-antiprotease activity in the neutrophils has been implicated in the pathogenesis of ANCA-related vasculitis. Ceruloplasmin is an acute-phase protein that has antiproteinase and antioxidant properties and inhibits MPO activity. We attempted to ...

OBJECTIVE: The aim of the present study is to review the clinical manifestations associated with small vessel vasculitis (SVV) as they pertain to the head and neck region. METHODS: A retrospective analysis was performed, including 34 individuals that filled the American College of Rheumatology criteria for the diagnosis of necrotizing ...

BACKGROUND: Acute renal failure (ARF) caused by rifampicin typically occurs on intermittent administration. There are isolated case reports and only one series reported in the literature. Systematic data, especially from countries endemic for tuberculosis and leprosy, are sparse. METHODS: We studied demographic, clinical, biochemical, and histopathologic features and prognosis of ...

Controversy exists regarding the level of proteinuria in patients with nephrosclerosis. MATERIAL AND METHODS: We retrospectively examined the clinical parameters of 67 patients with the histologic diagnosis of nephrosclerosis defined as arteriolar hyalinization and/or arterial intimal fibrosis in the absence of other findings in an adequate renal biopsy. Biopsies were ...

OBJECTIVE: To investigate the clinicopathological features and outcome of idiopathic IgA nephropathy with diffuse crescent formation in Chinese patients. METHODS: Twenty-five patients with diffuse crescentic IgA nephropathy (DCIgAN), 15 males and 10 females with median age of 28.5, and median disease duration of 5.1 months, were studied. Their clinical, laboratory ...

Polyoma virus (PV) can cause interstitial nephritis and lead to graft failure in renal transplant recipients. The clinical course of patients with polyoma virus nephritis (PVN) is not well understood, partially due to its relatively low incidence. This study is a retrospective analysis of our experience over 4 yr. The ...

In this study 43 patients with idiopathic nephrotic syndrome were randomly distributed into 2 age- and sex-matched groups. The first group was given fluvastatin while the second was used as control. The cases in the 2 groups were evaluated clinically, biochemically (creatinine clearance, albumin, 24-hour proteinuria, and lipogram), neurologically, and ...

AIMS: Nephropathy has long been recognized as a potential complication of cyanotic congenital heart disease (CCHD). There have been few large-scale studies or clinical reports on renal impairment in patients with CCHD; similarly, very few studies have examined the drug treatment of nephropathy in CCHD. We examined the clinical characteristics ...

Two siblings, a boy age 12 and his sister age 4 years, presented with proteinuria and hematuria, hypertension, and chronic hemolytic anemia. At age 13 years, the boy developed an episode of severe hypertensive encephalopathy and transient renal failure. Both children are attending normal school, have no neurologic symptoms, and ...

Recent genetic studies indicate that Alport syndrome and thin glomerular basement membrane disease (TMD) may both be due to COL4A3, COL4A4, and COL4A5 mutations, but there is continuing uncertainty concerning the diagnosis and management of patients without classic family history and symptoms. We examined kidney pathology and collagen alpha 3 ...

Recently, the authors reported that the ratio of serum IgA to C3 (serum IgA/C3 ratio) is a good marker to distinguish patients with IgA nephropathy from non-IgA nephropathy patients together with serum IgA levels using an international reference preparation (IFCC/CRM470). In this study, the authors investigated whether the serum IgA/C3 ...

BACKGROUND: The reason of variability of clinical course and progression to end-stage renal failure (ESRF) of two widespread chronic nephropathies-autosomal dominant polycystic kidney disease (ADPKD) and IgA nephropathy (IGAN) is not clear. The endothelial dysfunction is considered in the number of factors possibly influencing the prognosis of these nephropathies. Our ...

BACKGROUND: The purpose of the present study was to evaluate the clinical significance and the accuracy of the diagnosis of renal calculi incidentally found by ultrasonography (US). METHODS: A total of 906 subjects (639 men and 267 women) aged 18-78 years were referred for asymptomatic microscopic hematuria. Of these, 743 ...

BACKGROUND: Leptospirosis (LS) and Hantavirus (HV) infection have many common clinical manifestations, including acute renal failure. In as much as clinical experience with these diseases is quite limited in urban centers, we identified cases of Leptospira and HV-induced acute renal failure and compared the clinical course and evolution of these ...

Patients with Graves' disease (n = 61) treated with propylthiouracil (PTU) or thiamazole (MMI) were studied retrospectively to investigate differences in the prevalence of anti-myeloperoxidase anti-neutrophil cytoplasmic antibodies (MPO-ANCA) in relation to treatment with anti-thyroid drugs. The patients were divided into two groups: PTU-treated group (n = 32) and MMI-treated ...

Fifty-seven children (f/m=31/26) who survived diarrhea (D) + hemolytic uremic syndrome (HUS) were evaluated. The examinations were performed 1-27 years (median 7 years) from the onset of the acute disease. Patients aged 2.3-27 years (median 10 years) were allocated to three groups: Recovery (R, complete recovery), Residual renal symptoms (RRS, ...

BACKGROUND: Mycophenolate mofetil (MMF) has been used successfully in patients with ANCA-associated vasculitis as maintenance therapy. Only transient and moderate side effects have been reported with a daily dose of 2 g. Since all the treated patients who have been reported so far had no or only moderate renal insufficiency ...

Seven patients with type III membranoproliferative glomerulonephritis (MPGN) were followed for 9-17 years. Their mean age at presentation was 11.0 years. Their urinary abnormalities were detected by school urinary screening in five, and two patients presented with nephrotic syndrome. With time, urinalysis became normal in five, proteinuria persisted in one ...

Although systemic hypertension is very common in patients with glomerulonephritis there is a dispute if this alteration is consequence of the glomerulonephritis "per se" or is a consequence of the renal failure secondary to the glomerular lesion. With the aim to analyze the factors associated with systemic hypertension, 196 patients ...

PURPOSE: To evaluate renal US findings in patients with nephropathia epidemica (NE) and to determine whether changes in these findings are related to the clinical course of NE. MATERIAL AND METHODS: Renal US was undertaken in 23 hospital-treated NE patients during the acute phase of their disease (first study). The ...

OBJECTIVE: To investigate the implications of the HLA-B locus in the susceptibility to Henoch-Schönlein purpura (HSP) and determine if there are associations with renal and gastrointestinal (GI) manifestations of the disease. METHODS: A retrospective study was performed on an unselected population of patients with HSP from Northwest Spain. Forty-eight Caucasian ...

Approximately 75% of patients with tuberous sclerosis complex develop renal angiomyolipomas. These hamartomatous lesions distort and damage renal parenchyma and can lead to hemorrhage. To reduce the risk of hemorrhage, transarterial embolization is used to necrose the angiomyolipoma while sparing normal renal tissue. Although an effective renal-sparing procedure, embolization most ...

A considerable permeability factor (or factors) derived from circulating T cells has a crucial role in proteinuria of nephrotic syndrome (NS). We attempted to remove pathogenic T cells through lymphocytapheresis (LCAP) in 6 patients with primary NS, 2 patients with minimal change nephrotic syndrome (MCNS), 2 patients with focal segmental ...

Tubulointerstitial involvement seems to have a decisive influence on the progression of glomerular diseases. We have prospectively evaluated the levels of urinary retinol-binding protein (urRBP), a marker of proximal tubular dysfunction, in patients with different glomerulopathies (GPs) and correlated these levels with disease progression. By studying 238 patients with GPs, ...

Plasminogen activator inhibitor type 1 (PAI-1) and tissue plasminogen activator (tPA) are the major regulators of plasmin generation. Glomerular PAI-1/tPA balance is involved in extracellular matrix turnover, as well as fibrin deposition in glomeruli. Renal biopsy specimens were obtained from 80 patients with either primary or secondary glomerulonephritis (10 patients, ...