1/100. fibrinogen A alpha chain mutation (Arg554 Leu) associated with hereditary renal amyloidosis in a French family.A French family with hereditary renal amyloidosis (HRA) was studied. The disease presented in 7 of the 8 affected individuals with proteinuria or the nephrotic syndrome. The age of onset was in the fifth decade of life. There is currently no sign of extrarenal involvement in any affected individual. However, the nephropathy in this family is progressive and led to terminal renal failure in 4 patients. immunohistochemistry studies of glomerular amyloid deposits suggested that the amyloid protein was the fibrinogen A alpha chain. Direct dna sequencing revealed a G 4993 T transversion and subsequently Arg 554 Leu mutation in the fibrinogen A alpha chain. This is the first description of this fibrinogen A alpha chain mutation in europe. This family is of French descent and cannot be related to the previously reported Peruvian/Mexican and African-American kindreds.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
2/100. Remission of the nephrotic syndrome in a patient with renal amyloidosis due to rheumatoid arthritis treated with prednisolone and methotrexate.A 46-year-old woman developed nephrotic syndrome secondary to rheumatoid arthritis (RA). A renal biopsy showed deposition of amyloid fibrils in the subendothelial space of the glomerular capillary walls. After treatment with prednisolone (PSL, 40 mg/day), the levels of c-reactive protein (CRP) and serum amyloid A decreased to within normal limits for 2 weeks. However, the nephrotic syndrome persisted for 6 months after the therapy. To maintain the suppression of disease activity and to reduce PSL, methotrexate (5 mg/week) was added. The nephrotic syndrome resolved gradually, and the level of serum albumin returned to normal. Although renal prognosis of patients with nephrotic syndrome due to amyloidosis caused by RA has been considered poor, adequate and long-term treatment of RA with antiinflammatory drugs, including PSL and methotrexate, is useful for patients with secondary amyloidosis complicated by RA.- - - - - - - - - - ranking = 8keywords = nephrotic syndrome (Clic here for more details about this article) |
3/100. Renal amyloidosis with nephrotic syndrome in two patients with schistosomiasis mansoni and chronic salmonellosis.This report describes two Egyptian patients who presented with the nephrotic syndrome and concurrent infections with schistosoma mansoni and salmonella paratyphi a. Unlike similar cases previously reported from this unit, these patients did not respond to antimicrobial and antischistosomal therapy, and their renal biopsies demonstrated amyloidosis. These two case reports and several experimental observations suggest that chronic schistosomiasis and salmonellosis may lead to secondary amyloidosis in susceptible individuals.- - - - - - - - - - ranking = 5keywords = nephrotic syndrome (Clic here for more details about this article) |
4/100. Irreversible glomerular lesions induced by crystal precipitation in a renal transplant after foscarnet therapy for cytomegalovirus infection.AIMS: foscarnet is an antiviral agent used to treat cytomegalovirus infection in AIDS patients and in transplant recipients. In most cases, foscarnet induces reversible tubulo-interstitial lesions which can be avoided by correct hydration. We report the first case of crystal foscarnet precipitation within glomerular capillaries in a renal transplant. methods AND RESULTS: The recipient, a 49-year-old man, developed a nephrotic syndrome with haematuria and an acute renal failure after foscarnet therapy for cytomegalovirus (CMV) infection. The polarization examination of the first graft biopsy revealed the presence of birefringent crystals within glomeruli and tubules. Infrared analysis attested to the presence of trisodium foscarnet salts and mixed sodium calcium salts coloured by Von Kossa's reaction. A second biopsy showed glomerular sclerosis, interstitial fibrosis, tubular atrophy and crystal vanishing. polymerase chain reaction (PCR) in situ applied to this biopsy confirmed the diagnosis of cytomegalovirus infection. CONCLUSIONS: These adverse effects might be the result of a toxic synergy between foscarnet and other drugs. In cases with crystalline precipitation, graft biopsy remains the best mean of diagnosis and follow-up of glomerular damage.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
5/100. Transcriptional regulation of PDGF-A and TGF-beta by KTS WT1 deletion mutants and a mutant mimicking denys-drash syndrome.denys-drash syndrome (DDS) and frasier syndrome (FS) are rare diseases caused by the mutations of wilms tumor gene, WT1. The common denominator in these syndromes is a nephropathy which is manifested by early-onset proteinuria, nephrotic syndrome and end stage renal failure. Although these syndromes are genetic models of nephropathy and the mutations of WT1 gene are characterized in these patients the mechanism how mutations of WT1 gene affect the embryonic kidney adversely has not been elucidated. Recently, there was a report that FS is caused by mutations in the donor splice site of WT1. These mutations predicted loss of KTS isoform, which is one of the four splicing variants of WT1. In this study, two KTS deletion mutants of WT1 were made as well as a WT1 mutant mimicking a mutation found in a patient who had diffuse mesangial sclerosis, end stage renal failure and wilms tumor. Mutant embryonic kidney cell lines were established by transfection of 293 embryonic kidney cells with WT1 mutants. We investigated the transcription regulation of mutant WT1 among these cell lines using the reporter vectors containing PDGF-A and TGF-beta promoter sequence. Our results showed that the promoter activity of PDGF-A and TGF-beta, which are related to the progression of glomerular diseases, was modestly increased in the mutant cell mimicking the patent, while those activities were markedly increased in other two deletion mutant cell lines. This study demonstrated that KTS WT1 mutation found in DDS affected the cytokine expression adversely in vitro. From these results, we suggest that the alteration of KTS WT1 expression be responsible for the rapid progression of renal diseases in DDS and FS.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
6/100. Necrotizing fasciitis caused by serratia marcescens in two patients receiving corticosteroid therapy.Necrotizing fasciitis (NF), a devastating soft tissue infection, is rarely attributed to serratia marcescens. We here report two patients with S. marcescens NF, both of whom had underlying renal disease and had been receiving corticosteroid therapy. The first patient, a 40-year-old man with systemic lupus erythematosus and uremia on prednisolone therapy, developed fulminant cellulitis and septic shock 1 month after a skin biopsy for cutaneous vasculitis of the left foot. The cellulitis evolved to NF, and blood and necrotic tissue cultures both grew S. marcescens. The patient completely recovered after debridement and ceftazidime therapy. The second patient, a 73-year-old man receiving prednisolone therapy for nephrotic syndrome, developed right leg cellulitis that evolved to NF. blood and necrotic tissue cultures both grew S. marcescens. After aggressive debridement and ciprofloaxcin therapy, the NF improved. However, the patient died of aspiration pneumonia and massive gastrointestinal bleeding 1 month later. These findings illustrate that S. marcescens should be considered as a potential pathogen causing NF in susceptible hosts.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
7/100. Severe interstitial nephritis in a patient with renal amyloidosis and exacerbation of Crohn's disease.A 57-year-old man with long-term untreated Crohn's disease presented with exacerbation of his bowel disease, volume depletion, nephrotic syndrome and rapid decline in renal function. Renal biopsy revealed amyloidosis and extensive interstitial infiltration. Initiation of steroid therapy was associated with improvement in renal function and postponement of dialysis, suggesting that control of interstitial inflammation might have a therapeutic role in renal amyloidosis. We hypothesize that volume depletion could magnify toxicity of proteinuria, thus augmenting interstitial inflammation and accelerating the deterioration in renal function.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
8/100. Remission of nephrotic syndrome after removal of localized Castleman's disease.Renal complications of Castleman's disease are uncommon. Among the various renal disorders, including mesangial proliferative glomerulonephritis, membranous glomerulonephritis, and minimal change disease, nephrotic syndrome attributable to renal amyloidosis is very rarely reported. We report a case of mixed type of localized Castleman's disease complicated with nephrotic syndrome. Renal biopsy was performed. The deposition of AA amyloidosis was shown. After the removal of two mesenteric lymphoid masses, the proteinuria was gradually decreased and disappeared. Renal biopsy was repeated after 14 months, and, despite complete remission of nephrotic syndrome, no regression in amyloid deposition was found.- - - - - - - - - - ranking = 7keywords = nephrotic syndrome (Clic here for more details about this article) |
9/100. AL-amyloidosis of the kidney initially presenting as minimal change glomerulonephritis.Small amounts of amyloid in kidney biopsy specimens may be missed on routine examination unless specifically targeted. Occasionally, this oversight results in a diagnosis of minimal change glomerulonephritis (MCGN). This misdiagnosis may be facilitated by the fact that typical "minimal changes" with flattening and effacement of the epithelial foot processes can be found in capillary loops directly affected by amyloid deposition as well as in capillary loops of glomeruli with only mild amyloid deposition in the mesangium. Repeatedly, the diagnosis of MCGN had to be corrected to renal amyloidosis when re-examination by special techniques succeeded in detecting even small amounts of amyloid fibrils. We present the case of a previously healthy 49-year-old man who suddenly developed nephrotic syndrome. A first renal biopsy showed MCGN. proteinuria remained refractory to immunosuppressive treatments, and creatinine clearance deteriorated rapidly. Two years later, a repeat renal biopsy showed AL-amyloidosis. In this case, re-examination of the first biopsy in the light of the final diagnosis again did not show any deposition of amyloid fibrils. We suspect that proteinuria and epithelial podocyte changes in amyloidosis are caused by factors other than deposition of amyloid fibrils itself. Possibly a cytokine release during the early fibril formation leads to abnormalities even before the typical structural changes of renal amyloidosis can be detected. This is analogous to the hypothesis of a circulating factor that leads to proteinuria in focal segmental glomerulosclerosis or the speculation of altered lymphokine expression associated with the development of MCGN in Hodgkin's disease.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
10/100. Successful renal transplantation in focal glomerular sclerosis: report of two cases.The fate of renal allografts has provided much useful information about the pathogenesis of certain glomerular diseases. Idiopathic nephrotic syndrome with focal glomerular sclerosis (FGS) has been reported to recur in transplants. Two patients are reported with idiopathic nephrotic syndrome who progressed to renal failure. Immunopathologic and ultrastructural studies appeared to exclude immune pathogenic mechanisms in both patients, and light microscopic findings were characteristics of FGS. The nephrotic syndrome in one patient was initially steroid-dependent. Both patients underwent bilateral nephrectomy prior to renal transplantation. Graft function and urine protein excretion were normal in both patients 2 and 4 years respectively after transplantation, and transplant biopsies revealed no evidence of recurrent FGS. These results indicate that renal transplantation in patients with idiopathic nephrotic syndrome and FGS is not invariably followed by recurrent glomerular disease and suggest that the responsible pathogenetic mechanism(s) may not always persist.- - - - - - - - - - ranking = 4keywords = nephrotic syndrome (Clic here for more details about this article) |
| | Next -> |