1/143. A renal allograft recipient with late recurrence of focal and segmental glomerulosclerosis after switching from cyclosporine to tacrolimus.BACKGROUND: Focal and segmental glomerulosclerosis (FSGS) is one of the most frequent and severe primary glomerulonephritis that recurs in transplanted kidneys. Although cyclosporine seems to have no effect on the frequency of FSGS recurrence, there is evidence that cyclosporine reduces proteinuria and prolongs graft survival in patients with recurrent glomerulonephritis after renal transplantation. The effect of tacrolimus on nephrotic syndrome after renal transplantation is controversial. methods: We describe the case of a 30-year-old man with steroid-resistant nephrotic syndrome due to FSGS who developed nephrotic syndrome 5 years after renal transplantation due to recurrent disease when he was switched from cyclosporine to tacrolimus. RESULTS: He was given pulses of methylprednisolone and returned to cyclosporine. His proteinuria decreased, but he rapidly developed chronic renal failure. CONCLUSIONS: This observation strongly suggests that tacrolimus should be given with considerable care in renal transplant recipients with FSGS.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
2/143. recurrence of focal segmental glomerulosclerosis associated with Kimura's disease after kidney transplantation.A 13-year-old Brazilian boy with Kimura's disease (eosinophylic lymphoid granuloma) and nephrotic syndrome is reported. Native kidney biopsy showed focal segmental glomerulosclerosis (FSGS). Treatment with prednisolone resulted in partial remission of proteinuria, and he had a progressive loss in renal function, requiring initiation of chronic dialysis, which he underwent for 46 months. After kidney transplantation, the patient developed proteinuria. A renal biopsy showed recurrence of focal segmental glomerulosclerosis, and subsequently he developed renal insufficiency.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
3/143. Effect of pituitary microsurgery on acromegaly complicated nephrotic syndrome with focal segmental glomerulosclerosis: report of a rare clinical case.A case of nephrotic syndrome complicated by acromegaly is presented. The first renal biopsy specimen showed minor glomerular abnormalities with glomerular hypertrophy, corresponding with minimal change nephrotic syndrome. Corticosteroid therapy led to a partial remission, followed by frequent relapses after reduction of the drug. A diagnosis of atypical focal segmental glomerulosclerosis (FSGS) was made based on the second renal biopsy results 6 months after the first. We combined steroid therapy with the administration of an anticoagulant, cytotoxic agents, angiotensin-converting enzyme inhibitor, and low-density lipoprotein adsorption. Except for the angiotensin-converting enzyme inhibitor, these medications were not effective in terms of allowing a reduction in the high dosage of steroid, which in turn threatened progressive osteoporosis and lumbar vertebrae fracture. Administering the steroid at a moderate dosage, treatment was focused on the complicating acromegaly from pituitary microadenoma. Subcutaneous injections of octreotide acetate, a somatostatin analogue, reduced proteinuria and increased urine volume. Subsequent transsphenoidal microsurgery of the adenoma resulted in the normalization of the elevated creatinine clearance and the further reduction in steroid dosage while maintaining a remission state. This is the first reported clinical case with acromegaly followed by FSGS, and it is suggested that hypersecretion of growth hormone participates in the development and progression of glomerular disease.- - - - - - - - - - ranking = 2keywords = nephrotic syndrome (Clic here for more details about this article) |
4/143. Focal segmental glomerulosclerosis in a 32-year-old kidney allograft after 7 years without immunosuppression.In kidney allografts, focal segmental glomerulosclerosis (FSGS) has been described as recurrent, de novo, or a histological variant of chronic transplant glomerulopathy. We describe a unique case of de novo FSGS in a renal transplant not accompanied by any feature of rejection in a patient who had not been immunosuppressed for several years. A 58-year-old woman received a histoidentical living-related kidney transplant for end-stage renal disease due to chronic pyelonephritis. Twenty-four years after the transplant she voluntarily discontinued all immunosuppressive medication. Seven years later she presented with nephrotic syndrome, mild renal failure, and positive serology for hepatitis C virus (HCV) antibody. The kidney transplant biopsy disclosed de novo FSGS. Features of acute or chronic rejection, including chronic transplant glomerulopathy, were not seen. The pathogenesis of this lesion is probably related to sustained and prolonged glomerular hyperfiltration; alternatively, HCV infection may have triggered or accelerated the appearance of FSGS.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
5/143. Renal complications in patients with diabetes mellitus associated with an A to G mutation of mitochondrial dna at the 3243 position of leucine tRNA.The substitution of guanine for adenine at position 3243 of the leucine tRNA gene of mitochondrial dna was originally described in association with MELAS (mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes). diabetes mellitus associated with the mutation (mitochondrial diabetes) is a different phenotype from MELAS. We identified 11 patients with the mutation among 385 Japanese diabetic patients: two had MELAS and nine had mitochondrial diabetes. We present data on a male patient with mitochondrial diabetes who developed the nephrotic syndrome at the age of 23. light microscopy revealed mesangial expansion, PAS-positive deposits and segmental sclerosis in the glomeruli. Scattered mesangial electron-dense deposits and thickening of the basement membrane were found on electron microscopy, suggesting that diabetic glomerulosclerosis accompanied by focal glomerulosclerosis (FGS). Mitochondrial diabetes may pre-dispose patients to renal complications, including forms of glomerulonephritis, such as FGS.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
6/143. Mother-to-child transmitted WT1 splice-site mutation is responsible for distinct glomerular diseases.Mutations in the Wilms' tumor suppressor gene (WT1) are linked with denys-drash syndrome (DDS), a rare childhood disease characterized by diffuse mesangial sclerosis and renal failure of early onset, XY pseudohermaphroditism, and high risk of Wilms' tumor. KTS (lysine-threonine-serine) splice site mutations in WT1 intron 9 have been described in patients with frasier syndrome, another rare syndrome defined by focal and segmental glomerulosclerosis (FSGS), XY pseudohermaphroditism, and frequent occurrence of gonadoblastoma. Cases of frasier syndrome raise the question whether splice site mutations may also be found in XX females with isolated FSGS. A girl (index case) presented with the nephrotic syndrome at 9 mo of age. The diagnosis of DDS was based on the finding of diffuse mesangial sclerosis in the kidney biopsy and of a XY karyotype. The index case's mother had had proteinuria since she was 6 years of age. A renal biopsy was performed when she was 28 and disclosed FSGS. The same splice site mutation in intron 9 (WT1 1228 5 G-->A) involving one allele was found in the child and in her mother, but not in other members of the kindred (including the parents, the two brothers, and the two sisters of the index case's mother) who were free of renal symptoms. Quantification of WT1 KTS/-KTS isoforms in the index case's father and one index case's maternal uncle showed a normal KTS/-KTS ratio of 1.50. In contrast, the index case and her mother had a low ratio (0.40 and 0.34, respectively), within the range reported in frasier syndrome. In conclusion, this study shows that the KTS splice site mutation is not specific for frasier syndrome, but that it can also be found in DDS and in a normal female (XX) with FSGS, a woman who achieved normal pregnancy. It is suggested that WT1 splice site mutations should be sought in phenotypically normal females who present with FSGS or with related glomerulopathies of early onset.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
7/143. nail-patella syndrome and IgA nephropathy in a Chinese woman.nail-patella syndrome (NPS), also known as hereditary onycho-osteodysplasia, is an autosomal dominant pleiotropic disorder characterized by nailbed dysplasia or hypoplasia, absent or hypoplastic patellae, iliac horns and deformation or luxation of the radial head. Nephropathy is a known serious complication associated with NPS. In this report, we describe an adult Chinese woman with the clinical and radiological features of NPS who presented with the nephrotic syndrome. Renal biopsy disclosed focal segmental glomerulosclerosis on light microscopy, while immunofluorescence revealed predominant staining for IgA in the glomerular mesangium and along some capillary walls. Ultrastructural study confirmed the presence of paramesangial deposits as well as subendothelial collagen fibrils in the glomeruli. The histological findings were those of combined NPS and IgA disease, an association which has rarely been described.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
8/143. Acute rejection presenting as nephrotic syndrome.BACKGROUND: early diagnosis and treatment of acute rejection is important to prevent continued renal injury. Acute rejection most commonly presents with asymptomatic rise in serum creatinine. proteinuria associated with acute rejection is well established; however, there is limited documentation of the presentation of acute rejection as nephrotic syndrome in the literature. methods AND RESULTS: We report a renal transplant patient who presented with early onset nephrotic syndrome without change in serum creatinine, whose allograft biopsy confirmed acute glomerulitis and vascular rejection. Treatment of the acute rejection was accompanied by resolution of the nephrotic syndrome. A second episode of acute rejection was also manifested as nephrotic range proteinuria. CONCLUSION: The nephrotic syndrome in early post-transplantation period should prompt a work-up for acute rejection even in the absence of the common findings of this complication.- - - - - - - - - - ranking = 2.6666666666667keywords = nephrotic syndrome (Clic here for more details about this article) |
9/143. carbon disulfide nephropathy.A 45-year-old nondiabetic man presented with features resembling diabetic triopathy. He worked in a rayon manufacturing plant and was exposed to toxic levels of carbon disulfide (CS(2)). Clinical abnormalities included peripheral and central nervous system abnormalities as well as retinopathy, dyslipidemia, cardiovascular disease, and nephrotic syndrome. He later developed focal sclerosing glomerulonephritis. The latter has not previously been described in cases of CS(2) exposure. Terminally, he developed end-stage renal disease and progressive dementia, both of which were thought to be consequences of CS(2) exposure earlier in life.- - - - - - - - - - ranking = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
10/143. 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 = 0.33333333333333keywords = nephrotic syndrome (Clic here for more details about this article) |
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