Crescentic glomerulonephritis in a patient with heterozygous Fabry's disease.

Abstract:

:A 58-year-old woman who suffered from a heterozygous Fabry's disease and immune complex crescentic glomerulonephritis (GN) is reviewed. The diagnosis was made on the basis of the pathologic findings and peripheral leukocyte alpha-galactosidase activity. Light microscopy revealed a vacuolization of epithelial cells and electron microscopy showed myelin figures in the cytoplasm of visceral epithelial cells typical of Fabry's disease at the first renal biopsy. During the following 4 months she developed progressive renal failure and a second renal biopsy disclosed the formation of cellular crescents in 7 of 11 glomeruli observed. A rare case of combined Fabry's disease and crescentic glomerulonephritis is discussed.

journal_name

Nephron

journal_title

Nephron

authors

Shimazu K,Tomiyoshi Y,Aoki S,Sakemi T,Sugihara H

doi

10.1159/000063287

subject

Has Abstract

pub_date

2002-10-01 00:00:00

pages

456-8

issue

2

eissn

1660-8151

issn

2235-3186

pii

63287

journal_volume

92

pub_type

杂志文章

相关文献

NEPHRON文献大全
  • Renal cell carcinoma presenting as nephrotic syndrome.

    abstract::A 64-year-old woman presenting with a history of increasing oedema was found to have nephrotic syndrome with a 24-hour urinary protein excretion of 20.7 g and renal impairment with an initial serum creatinine level of 197 mumol/l (2.16 mg/dl). A renal tumour was demonstrated by ultrasound scanning and subsequent nephr...

    journal_title:Nephron

    pub_type: 杂志文章,评审

    doi:10.1159/000188434

    authors: Woodrow G,Innes A,Ansell ID,Burden RP

    更新日期:1995-01-01 00:00:00

  • The effect of sodium intake on cystinuria with and without tiopronin treatment.

    abstract::As with many other amino acids the transport of cystine across the tubular epithelium is coupled to a parallel transport of sodium. We have studied the effect of a sodium-restricted diet on the urinary excretion of cystine in 13 patients with cystinuria, 7 of whom were treated with the SH compound tiopronin (2-mercapt...

    journal_title:Nephron

    pub_type: 临床试验,杂志文章

    doi:10.1159/000188760

    authors: Lindell A,Denneberg T,Edholm E,Jeppsson JO

    更新日期:1995-01-01 00:00:00

  • Should We STOP Angiotensin Converting Enzyme Inhibitors/Angiotensin Receptor Blockers in Advanced Kidney Disease?

    abstract::Chronic kidney disease (CKD) is a worldwide public health problem associated with a high prevalence of cardiovascular disease (CVD) and impaired quality of life. Previous research for preventing loss of glomerular filtration rate (GFR) has focused on reducing blood pressure (BP) and proteinuria. Angiotensin converting...

    journal_title:Nephron

    pub_type: 杂志文章,评审

    doi:10.1159/000447068

    authors: Ahmed A,Jorna T,Bhandari S

    更新日期:2016-01-01 00:00:00

  • Concomitant presence of three different glomerular diseases in the same patient. Report of a case and review of the literature.

    abstract::A 51-year-old man with diabetes mellitus and the nephrotic syndrome on renal biopsy was found to have diabetic glomerulosclerosis, amyloidosis and membranous glomerulopathy. The presence of three distinct glomerular diseases in the same patient is unique. Possible factors involved in their pathogenesis are discussed a...

    journal_title:Nephron

    pub_type: 杂志文章,评审

    doi:10.1159/000183026

    authors: Bertani T,Olesnicky L,Abu-Regiaba S,Glasberg S,Pirani CL

    更新日期:1983-01-01 00:00:00

  • Effect of levamisole on skin reactions to DNCB in chronically dialyzed patients.

    abstract::The local cutaneous inflammatory response to DMCB sensitization was positive in only 35% of 20 uremic patients, and the delayed skin reaction was positive in only 15%. The reaction to DMCB challenge was markedly delayed in some patients. Improved response to DNCB re-challenge followed levamisole administration in only...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000181858

    authors: Modai D,Sagi DB,Maor J

    更新日期:1980-01-01 00:00:00

  • Do Endocannabinoids Regulate Glucose Reabsorption in the Kidney?

    abstract::Diabetic nephropathy (DN), a distinct manifestation of diabetic kidney disease, affects approximately 30% of patients with diabetes. While most attention has been focused on glomerular changes related to DN, there is growing evidence that tubulopathy is a key feature in the pathogenesis of this disease. The renal prox...

    journal_title:Nephron

    pub_type: 杂志文章,评审

    doi:10.1159/000494512

    authors: Hinden L,Tam J

    更新日期:2019-01-01 00:00:00

  • Serum erythropoietin levels in the immediate kidney-posttransplant period.

    abstract::Following successful kidney transplantation, renal anemia is gradually corrected during the first 3-4 months. Serum erythropoietin (EPO) levels are increased after the first postoperative day following grafting. In this study, the serum EPO levels in the early posttransplant phase were investigated in 8 living-donor a...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000186701

    authors: Goch J,Birgegård G,Wikström B,Tufveson G,Danielson BG

    更新日期:1992-01-01 00:00:00

  • Acute Kidney Injury and Hemolytic Anemia Secondary to Mycoplasma pneumoniae Infection.

    abstract::Glomerulonephritis as well as kidney injury secondary to fulminant intravascular hemolysis are rare extrapulmonary manifestations of Mycoplasma pneumoniae infection. We describe a 50-year-old female diagnosed with M. pneumoniae infection-associated hemolytic anemia, characterized by negative cold agglutinin tests but ...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000478991

    authors: Carrara C,Abbate M,Sabadini E,Remuzzi G

    更新日期:2017-01-01 00:00:00

  • Effects of rhubarb tannins on uremic toxins.

    abstract::The effects of each of several tannins purified from Rhei Rhizoma on serum constituents were investigated in rats with adenine-induced renal failure. Blood levels of urea nitrogen, methylguanidine (MG), and guanidinosuccinic acid (GSA) were significantly decreased in rats given (-)-epicatechin 3-O-gallate at a dose of...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000186406

    authors: Yokozawa T,Fujioka K,Oura H,Nonaka G,Nishioka I

    更新日期:1991-01-01 00:00:00

  • Beta-2-microglobulin in the assessment of renal function of the transplanted kidney.

    abstract::Plasma and urine beta-2-microglobulin (B2m) were measured in 37 renal transplant recipients and 34 healthy subjects. Serum B2m was found to be a more sensitive index of renal function than serum creatinine. Renal transplant recipients had significantly higher urine B2m when compared with normal controls. Normal subjec...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000183376

    authors: Woo KT,Lee EJ,Lau YK,Lim CH

    更新日期:1985-01-01 00:00:00

  • Renal artery stenosis associated with epidermal nevus syndrome.

    abstract::Epidermal nevus syndrome is an unusual neurocutaneous disorder in which epidermal nevi are associated with abnormalities of the skeleton and central nervous system, including the eyes and somtimes the cardiovascular system. We treated a patient in whom the latter included renal artery stenosis. An 18-year-old man with...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000045540

    authors: Aizawa K,Nakamura T,Ohyama Y,Saito Y,Hoshino J,Kanda T,Sumino H,Nagai R

    更新日期:2000-01-01 00:00:00

  • IgA nephropathy occurring in the context of previous acute glomerulonephritis.

    abstract::A 37-year-old female presented with acute onset of glomerulonephritis 10 days following an upper respiratory infection. Serum complement components were depressed and proteinuria exceeded 3.0 g daily. Renal biopsy revealed granular staining of IgG and C3 along the basement membrane as well as small amounts of IgA in a...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000183980

    authors: Yoshizawa N,McClung JA,Ohshima S,Takahashi K,Treser G

    更新日期:1986-01-01 00:00:00

  • Acute renal failure from inhalation of mycotoxins.

    abstract::Mysterious deaths of archeologists after opening Egyptian tombs have been suspected to be secondary to inhalation of mycotoxin, however, the hypothesis has never been verified. Recently, we observed a case of acute renal failure (ARF) undeniably due to inhalation of ochratoxin of Aspergillus ochraceus. After spending ...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000187411

    authors: Di Paolo N,Guarnieri A,Loi F,Sacchi G,Mangiarotti AM,Di Paolo M

    更新日期:1993-01-01 00:00:00

  • Renal failure in familial lecithin-cholesterol acyltransferase deficiency.

    abstract::Familial lecithin-cholesterol acyltransferase deficiency is a hereditary disorder of lipid metabolism. Lipid material is deposited in the kidneys, the glomerular capillary basement membrane is irregularly thickened, detachment and even loss of endothelial cells are seen in the glomeruli. Proteinuria was present in 8 o...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000180835

    authors: Myhre E,Gjone E,Flatmark A,Hovig T

    更新日期:1977-01-01 00:00:00

  • Serum leptin concentrations in patients on hemodialysis.

    abstract::Serum leptin concentrations in normal humans have been reported to correlate with the body mass index (BMI) as well as with the body fat mass. In this study, we measured serum leptin concentrations in 107 patients on hemodialysis, 30 of whom had diabetes mellitus as the cause, and examined the clinical significance. F...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000045122

    authors: Nakazono H,Nagake Y,Ichikawa H,Makino H

    更新日期:1998-09-01 00:00:00

  • Effect of vitamin C supplementation on renal oxalate deposits in five-sixths nephrectomized rats.

    abstract::We have previously reported that hyperoxalemia can be aggravated by vitamin C supplementation in regular hemodialysis patients. The present study was undertaken to examine the validity of this observation in an experimental setting. Fifty five-sixths nephrectomized rats were divided into two groups: 30 rats were allow...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000185389

    authors: Ono K,Ono H,Ono T,Kikawa K,Oh Y

    更新日期:1989-01-01 00:00:00

  • Platelet-leukocyte activation during hemodialysis detected with a monoclonal antibody to leukocyte integrin CD11b.

    abstract::Platelet activation is commonly monitored with a battery of monoclonal antibodies against different platelet epitopes with controversial results. The transient expression of platelet markers and their role mediating interactions with other cells could easily explain these discrepancies. The present study has evaluated...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000045167

    authors: Hernández MR,Galán AM,Lozano M,Bozzo J,Cases A,Escolar G,Calls J,Ordinas A

    更新日期:1998-10-01 00:00:00

  • C1Q complexes with beta-2-microglobulin and amyloid P-component in sera of patients with dialysis-associated amyloidosis.

    abstract::We studied complexes composed of C1q and beta 2-microglobulin (C1q.beta 2M) or serum amyloid P-component (C1q.SAP) in the sera of 38 patients receiving hemodialysis (HD) and 20 healthy control subjects using an enzyme-linked immunoassay. We also compared complex levels in HD patients with and without symptoms of HD-as...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000188506

    authors: Hasegawa M,Kawashima S,Shikano M,Kojima K,Yanai T,Hassegawa H,Tomita M,Murakami K,Kawamura N,Katsumata H

    更新日期:1995-01-01 00:00:00

  • Cultural Challenges in the Care of Refugees with End-Stage Renal Disease: What Western Nephrologists Should Know.

    abstract::Forced migration is on the rise, mainly from the Middle East to western countries, resulting in unprecedented moral, political, and economic challenges for both refugees and host communities. Recent research showed that refugees represent around 1.5% of the dialysis population in several European and Middle Eastern co...

    journal_title:Nephron

    pub_type: 杂志文章,评审

    doi:10.1159/000477362

    authors: Sekkarie MA,Abdel-Rahman EM

    更新日期:2017-01-01 00:00:00

  • Renal tubular sodium and water excretion in antibiotic-induced nephrotoxicity. Renal function in antibiotic nephrotoxicity.

    abstract::Clearance techniques were used to evaluate renal tubular sodium and water excretion in 4 patients with antibiotic-induced acute renal failure (ARF). Creatinine clearances and maximal urine flow rates of patients with ARF (22.6 and 5.23 ml/min, respectively) were significantly lower than control values during hypotonic...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000181226

    authors: Hsu CH,Maletz RM,Rozas VV,Kurtz TW

    更新日期:1978-01-01 00:00:00

  • Exercise renal rehabilitation program: psychosocial effects.

    abstract::The aim of this study was to assess the psychosocial effects of exercise training on hemodialysis (HD) patients. Thirty-one uremic patients, aged 50.6+/-11.6 years, on maintenance HD were studied. Twenty patients were selected at random for a 6-month exercise renal rehabilitation program (ERRP) consisting of 3 weekly ...

    journal_title:Nephron

    pub_type: 临床试验,杂志文章,随机对照试验

    doi:10.1159/000190266

    authors: Kouidi E,Iacovides A,Iordanidis P,Vassiliou S,Deligiannis A,Ierodiakonou C,Tourkantonis A

    更新日期:1997-01-01 00:00:00

  • Reversible nephrotic range proteinuria with renal artery stenosis: a clinical example of renin-associated proteinuria.

    abstract::Nephrotic range proteinuria occurred in a 60-year-old woman with renal artery stenosis and marked hyperreninemia. Treatment by nephrectomy produced resolution of both proteinuria and hypertension. The gradual resolution of the proteinuria postoperatively suggested the proteinuria, at least in part, came from the contr...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000182521

    authors: Eiser AR,Katz SM,Swartz C

    更新日期:1982-01-01 00:00:00

  • Proliferative glomerulonephritis in monoclonal gammopathy.

    abstract::A 62-year-old white male suffering from plasmacytosis with monoclonal gammopathy developed gastrointestinal bleeding, microscopic hematuria, proteinuria and progressive azotemia. A renal biopsy demonstrated capillary wall thickening, mesangial hyperplasia, crescent formation and subepithelial humps. Serum immunoglobul...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000180902

    authors: Dhar SK,Smith EC,Fresco R

    更新日期:1977-01-01 00:00:00

  • Circulating anti-tubular basement membrane antibody in a variety of human renal diseases: detection and significance.

    abstract::Serum specimens from 99 selected renal patients were examined over a 3-year period by both a hemagglutination technique (HA) and an indirect immunofluorescent (IIF) method for the presence of circulating antibody against tubular basement membrane (TBM). 11 patients were found with anti-TBM antibodies. 6 of them also h...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000181943

    authors: Cattran DC

    更新日期:1980-01-01 00:00:00

  • Efficiency of hemodialysis of pyrimidine compounds in patients with chronic renal failure.

    abstract::The accumulation in blood plasma and efficiency of hemodialysis of pyrimidine compounds (orotic acid, orotidine, pseudouridine, uridine, thymine) as well as uric acid and creatinine in 23 patients with chronic renal failure (CRF) was investigated. As a reference, the analysis of the above metabolites in the plasma of ...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000187313

    authors: Daniewska-Michalska D,Motyl T,Gellert R,Kukulska W,Podgurniak M,Opechowska-Pacocha E,Ostrowski K

    更新日期:1993-01-01 00:00:00

  • SGLT-2 Inhibition: A Potential New Treatment for Diabetic Kidney Disease?

    abstract::Diabetic nephropathy is the leading cause of kidney failure. Treatments with drugs that block the renin-angiotensin system have proven beneficial in slowing kidney disease progression among those with diabetes; their benefit is limited and they do not stop disease progression. Despite multiple clinical trials of vario...

    journal_title:Nephron

    pub_type: 杂志文章,评审

    doi:10.1159/000450895

    authors: Toto RD

    更新日期:2017-01-01 00:00:00

  • Uremic pruritus and skin mast cells.

    abstract::The number of mast cells in the skin was evaluated in 25 patients with end-stage renal failure on different treatment modality (conservative, hemodialysis and peritoneal dialysis). According to the presence of pruritus, uremic patients were divided into two groups: group A, 13 patients with diffuse pruritus, and group...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000186826

    authors: Dimković N,Djukanović L,Radmilović A,Bojić P,Juloski T

    更新日期:1992-01-01 00:00:00

  • Increased Risk for Cardiovascular Events in Patients with Diabetic Kidney Disease and Non-Alcoholic Fatty Liver Disease.

    abstract:BACKGROUND/AIMS:Non-alcoholic fatty liver disease (NAFLD) is recognised to be a strong risk factor for causing cardiovascular disease (CVD) and chronic kidney disease (CKD), independent of the presence of diabetes mellitus. The association of NAFLD with outcomes in patients with diabetic kidney disease (DKD) has been v...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000493472

    authors: Chinnadurai R,Chrysochou C,Kalra PA

    更新日期:2019-01-01 00:00:00

  • Renal Injury During Preclampsia: Role of Extracellular Vesicles.

    abstract::Preeclampsia (PE) represents an important cause of acute kidney injury during pregnancy with a high rate of both maternal and fetal morbidity and mortality. The presence of endothelial dysfunction is the main cause of PE development; however, the pathogenic mechanisms are not still fully elucidated. A plethora of harm...

    journal_title:Nephron

    pub_type: 杂志文章,评审

    doi:10.1159/000502456

    authors: Pagani F,Cantaluppi V

    更新日期:2019-01-01 00:00:00

  • Transcapillary oncotic pressure in the edema of congestive heart failure.

    abstract::Tissue fluid and plasma oncotic pressure levels were measured in an unselected group of 13 patients presenting with congestive heart failure. Patients had a mean serum albumin of 3.6 g/dl +/- (SD) 0.35; serum oncotic pressure was 22.23 +/- 1.8 mm Hg; tissue fluid (lower leg) oncotic pressure was 0.985 +/- 0.34 mm Hg. ...

    journal_title:Nephron

    pub_type: 杂志文章

    doi:10.1159/000185804

    authors: Kwan T,Pintea M,Garcia Morino F,Preston RR,Li J,Caruso C,Berlyne SD,Berlyne GM

    更新日期:1990-01-01 00:00:00