Cases reported "Nephrotic Syndrome"

Filter by keywords:



Filtering documents. Please wait...

1/13. Long-term azathioprine therapy in two children with steroid-dependent minimal-change nephrotic syndrome.

    Long-term azathioprine therapy as an alternative treatment to cyclophosphamide was done in 2 children with steroid-dependent minimal-change nephrotic syndrome (MCNS). They had already been treated with prednisolone, intravenous methyl-prednisolone pulse therapy, cyclophosphamide and mizoribine. Although cyclophosphamide had been proved to be effective in maintaining their remission, the cumulative dose of the agent limited another course of cyclophosphamide therapy. Since ciclosporine therapy is much expensive, a trial of azathioprine (2 mg/kg per day) was started, and the therapy resulted in inducing sustained remission and reducing prednisolone. The patients were well tolerated the long-term azathioprine therapy over a year. Although the efficacy of azathioprine in the management of childhood MCNS might be restricted, we therefore suggest that this agent should be reconsidered as an alternative treatment to cyclophosphamide.
- - - - - - - - - -
ranking = 1
keywords = alternative
(Clic here for more details about this article)

2/13. frasier syndrome, part of the Denys Drash continuum or simply a WT1 gene associated disorder of intersex and nephropathy?

    Dysfunction of the Wilms' Tumour gene (WT1), a transcription factor critical for normal development and function of the urogenital tract, can result in both tumourigenesis [corrected] and urogenital abnormalities. The association of WT1 gene mutations with most cases of denys-drash syndrome is well described. More recently WT1 mutations have also been described in a related condition, frasier syndrome. We report a case where genetic analysis showed a WT1 mutation typically associated with frasier syndrome: a 1228 5 guanine to adenine substitution at the 3' alternative splice donor site in intron 9. The case provides a focus for the discussion of recent evidence that Denys Drash and frasier syndrome form two ends of a spectrum of disorders. In addition, it illustrates the increasing significance of genetic investigation within clinical practice for diagnostic, prognostic and therapeutic purposes and the importance of karyotype analysis in phenotypically normal girls with renal disease.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)

3/13. nephrotic syndrome in Hodgkin's disease. Evidence for pathogenesis alternative to immune complex deposition.

    The nephrotic syndrome has been reported to occur in patients with Hodgkin's disease even in the absence of amyloidosis, tumor infiltration of renal vein thrombosis. Three patients are presented with Hodgkin's disease and the nephrotic syndrome whose renal biopsy specimens studied with light, immunofluorescence and electron microscopy were compatible with "lipoid nephrosis" (minimal change disease). A review of the literature reveals 35 patients with Hodgkin's disease and the nephrotic syndrome. Renal tissue was available for examination in only 27 patients. The majority of patients apparently had glomerular alterations consistent with lipoid nephrosis. The nephrotic syndrome in most of these patients remitted with a variety of methods of therapy (including excision, irradiation, prednisone and cyclophosphamide) and tended to relapse with a recurrence of Hodgkin's disease. In three-fourths of the patients with Hodgkin's disease and the nephrotic syndrome, the Hodgkin's disease was of a mixed cellularity type. The etiology of lipoid nephrosis, although unclear, may be a consequence of altered lymphocyte function. Hodgkin's disease is a malignancy involving T lymphocytes, and the nephrotic syndrome occurring in the course of Hodgkin's disease may be a result of an adverse effect of glomeruli by products of tumor lymphocytes rather than of glomerular deposition of immune complexes.
- - - - - - - - - -
ranking = 2
keywords = alternative
(Clic here for more details about this article)

4/13. Therapy-resistant focal and segmental glomerulosclerosis.

    The clinical course of a young female patient with focal segmental glomerulosclerosis (FSGS) who failed to respond to any of the recommended therapeutic protocols will represent the background of a discussion of currently available alternative treatments for FSGS. Traditionally, FSGS has been believed to have a poor prognosis, with a low response rate to treatment and a progressive course terminating with end-stage renal disease (ESRD). Some 40% of patients respond to prolonged corticosteroid treatment. Steroid resistance in adults should perhaps be assumed only after failure to respond to a 6-month course of daily steroid therapy. Regarding recent recommendations, the use of cytotoxic therapy (cyclophosphamide, chlorambucil or azathioprine) may be considered as second-line therapy (evidence D). Treatment with cyclosporin A at doses of 4-6 mg/kg/day has been successful in reducing proteinuria. There is little information available on the effects of such treatment on the progression of FSGS. Even fewer data are available on the success rate of the use of tacrolimus in resistant forms of FSGS in adults. Mycophenolate mofetil has been used with impressive success in a few high-risk patients who failed on previous therapeutic regimens. There is preliminary evidence in an uncontrolled series of patients with resistant primary FSGS that the addition of plasmapheresis may provide effective long-term benefits in some patients. The accurate assessment of the role of plasmapheresis and possibly immunoadsorption in the management of patients with FSGS requires further evaluation. Non-immunosuppressive therapy (i.e. angiotensin-converting enzyme inhibitors, angiotensin ii receptor blockers, lipid-lowering drugs, non-steroidal anti-inflammatory drugs) should be applied to almost all patients with primary FSGS.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)

5/13. cyclosporine A in the treatment of a patient with immune thrombocytopenia accompanied by myelodysplastic syndrome and nephrotic syndrome.

    We describe a complete remission with cyclosporine A in a myelodysplastic syndrome (MDS) patient who had a 9-year history of nephrotic syndrome (NS) due to autoimmune nephritis. A 72-year-old woman with MDS and NS rapidly developed thrombocytopenia with multiple spontaneous bleeding episodes and profound proteinuria. She showed persistent platelet refractoriness to platelet transfusions. A flow cytometry examination strongly detected antiplatelet autoantibodies on the surface of her platelets. The treatment with high-dose corticosteroids and intravenous immunoglobulin did not lead to complete improvement in the platelet count, bleedings and proteinuria. However, a low dose of cyclosporine A resulted in a sustained normal range of blood platelet count and negative proteinuria. This finding suggests that, in selected cases, cyclosporine A can be an attractive alternative for MDS patients who also have immune-mediated diseases.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)

6/13. Mesenteric thrombosis causing short bowel syndrome in nephrotic syndrome.

    Nephrotic patients are at risk of developing venous and arterial thrombotic complications. pulmonary embolism due to affected deep leg veins is by far the most common event. Renal or cerebral vein thromboses have been described. thrombosis of arterial vessels is less frequent. Mesenteric infarction is a rare but severe complication in patients with nephrotic syndrome (NS). We report a 7-year-old boy with a steroid-dependent (SD) NS and a homozygous mutation of methylenetetrahydrofolate reductase, increasing the risk of thromboembolic events. He developed a thrombosis of his superior mesenteric artery during his ninth relapse, which was responsible for a necrosis of 240 cm of his small bowel, necessitating resection of necrotic parts and double external ostomy diversion. Remission was achieved with pulse prednisolone therapy. Corticoids were reduced over 4 months progressively. Oral cyclosporin A (CyA) was initiated for long-term treatment. Due to a short bowel syndrome with severe malabsorption, even oral administration of 22.5 mg/kg per day CyA did not lead to sufficient plasma levels. Intravenous cyclophosphamide pulse therapy over 6 months led to a complete remission. No relapse occurred over a period of more than 5 months after the last cyclophosphamide pulse. Anticoagulation and screening for increased susceptibility for thrombotic events are necessary in every nephrotic patient. Intravenous cyclophosphamide pulse therapy is a useful alternative in SDNS with impaired intestinal absorption of applied immunosuppressive drugs.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)

7/13. empyema and mesangiocapillary glomerulonephritis with nephrotic syndrome.

    Two cases of empyema with nephrotic syndrome are described, a previously unreported association. In one case the nephrotic syndrome resolved with treatment of the empyema. Renal biopsy in both patients showed mesangiocapillary glomerulonephritis and in one case there was evidence of activation of the alternative complement pathway, which is often associated with this histological type of glomerulonephritis. The association between glomerulonephritis and empyema is important because it represents a potentially reversible form of renal disease.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)

8/13. nephrotic syndrome associated with varicella infection.

    A 4-year-old boy developed nephrotic syndrome following varicella infection. Serologic studies during the early phase of the disease demonstrated a decrease in serum C3, C4, and properdin factor B. Renal biopsy revealed an acute proliferative glomerulonephritis with deposition of immunoglobulins A (IgA) and M, C3, C1q, and varicella virus antigen in the glomerulus, suggesting an immune complex deposition. Ultrastructurally, this suggested a postinfectious immune complex glomerulonephritis. These phenomena suggested that varicella virus antigen antibody complexes were deposited in the glomerulus and activated the classic and alternative pathway of complements, leading to an immune complex glomerulonephritis. During the nephrotic phase, an increase in OKT8 cells and decrease of the OKT4 cells were demonstrated. Two months later, this alteration returned to normal as the renal disease was in remission. This change of lymphocyte subsets during varicella infection may play a role in the pathogenesis of nephrotic syndrome.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)

9/13. Nephrotic range pseudoproteinuria in a tolmetin-treated patient.

    False-positive proteinuria by acid precipitation testing in tolmetin-treated patients has been noted on routine urinalysis screening. However, the magnitude of such false positivity has not been previously reported. We present a patient with systemic lupus erythematosus who presented with nephrotic range pseudoproteinuria using an acid precipitation method of testing. This misleading laboratory result and alternative methods of testing for proteinuria in tolmetin-treated patients are discussed.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)

10/13. Therapeutic strategy of nephrotic syndrome in patients over eighty.

    In the present paper, we report four very elderly patients (80 years of age or older) with primary nephrotic syndrome. In all patients, oral prednisolone (PSL) alternative day therapy was attempted and three patients responded very well (complete remission 2, partial remission 1). However, one patient exhibited no effect of PSL and died from acute oliguric renal failure in spite of aggressive measures including hemodialysis. Fracture of the femoral neck occurred in one patient during PSL therapy, although the relationship between the fracture and PSL therapy was uncertain. In addition to our reported cases, 5 cases of nephrotic syndrome in similar patients reported elsewhere are analyzed together with our cohort. PSL was commonly effective in inducing remission in very elderly patients (7 of 9 patients; 78%). Very elderly patients with nephrotic syndrome frequently suffer from oliguric renal failure, which has a potentially high mortality. Based on these data, we conclude that PSL therapy can ameliorate primary nephrotic syndrome in patients over the age of eighty years. However, the complications both of the disease and its treatment, (e.g., acute oliguric renal failure, hip fracture) must be carefully monitored.
- - - - - - - - - -
ranking = 0.5
keywords = alternative
(Clic here for more details about this article)
| Next ->


Leave a message about 'Nephrotic Syndrome'


We do not evaluate or guarantee the accuracy of any content in this site. Click here for the full disclaimer.