Recurrence Of Nephrotic Syndrome Research Articles

Early recurrent nephrotic syndrome after renal transplantation in children with focal segmental glomerulosclerosis

We analysed risk factors to predict the recurrence of nephrotic syndrome and the therapeutic efficacy of plasmapheresis combined with oral cyclophosphamide (PE+CPM) in early recurrent nephrotic syndrome after transplantation in children with focal segmental glomerulosclerosis (FSGS). Medical records after 1990 of 16 children with biopsy-proven idiopathic FSGS and renal transplantation before the age of 18 years were reviewed. Early recurrence of nephrotic syndrome developed in six cases (37. 5%). While early kidney graft biopsies, performed within the first week after the onset of recurrence, revealed diffuse effacement of foot process only, late biopsies contained segmentally sclerosed glomeruli as well. Among several possible risk factors, the mean duration from onset of original nephrotic syndrome to development of end-stage renal disease was shorter in the recurrent group (P=0.045) and the percentage of globally sclerosed glomeruli was higher in the non-recurrent group (P=0.001). PE+CPM therapy resulted in complete remission of nephrotic syndrome if it was started early and if there was no evidence of accompanying acute rejection. These results support more liberal use of living-related donors for renal transplantation of children with FSGS and ESRD, considering the shortage of cadaveric donors in our society and relatively good efficacy of the early and intensive PE+CPM therapy for early recurrent nephrotic syndrome.

Low density lipoprotein apheresis therapy for steroid-resistant nephrotic syndrome

The pathogenic role of hyperlipidemia in long-standing nephrotic syndrome (NS) is known to be responsible for both the progression of glomerulosclerosis and tubulointerstitial injury, especially in focal segmental glomerulosclerosis (FGS). Aggressive lipid lowering treatment by low density lipoprotein (LDL) apheresis (LDL-A) using a dextran sulfate cellulose column to treat patients with steroid-resistant or frequently recurrent severe NS was performed first without fixing the protocol in eight patients with FGS and one with minimal change nephrotic syndrome (MCNS). The period of NS before LDL-A, number and average intervals of LDL-A until the end of the therapy, and the prognosis were investigated. Next, a multicenter study with a fixed protocol of LDL-A treatment was designed in combination with steroid therapy for treatment twice a week for three weeks and weekly for six weeks, and was performed in 17 patients with FGS. The effects on the state of NS in addition to the change of urinary eicosanoid metabolites and remission rates were evaluated. In the preliminary study, along with a rapid improvement of hyperlipidemia, a high incidence of remission was achieved by LDL-A performed at relatively short intervals. In the multicenter study with a fixed protocol, there was a significant decrease of urinary protein (P < 0.001) and increase of serum albumin (P < 0.02) as well as a decrease of thromboxane B2 (TXB2) excretion (P < 0.05) after the treatment. Urinary excretion of TXB2 was significantly reduced after LDL-A (P < 0.05). The rate of entering into complete or incomplete remission was 71% with a relatively short duration of nephrotic-range proteinuria using the LDL-A therapy in comparison with steroid therapy alone. The rapid improvement of hypercholesterolemia with LDL-A treatment may provide a new approach for a high rate of improvement in the degree of NS in steroid-resistant NS of FGS and MCNS.

Immunoglobulins are directly involved in the pathogenesis of idiopathic nephrotic syndrome recurrence after renal transplantation

Immunoglobulins are directly involved in the pathogenesis of idiopathic nephrotic syndrome recurrence after renal transplantation

Immunoglobulins are directly involved in the pathogenesis of idiopathic nephrotic syndrome recurrence after renal transplantation.

Immunoglobulins are directly involved in the pathogenesis of idiopathic nephrotic syndrome recurrence after renal transplantation.

Podocytes undergo phenotypic changes and express macrophagic-associated markers in idiopathic collapsing glomerulopathy

Podocytes undergo phenotypic changes and express macrophagic-associated markers in idiopathic collapsing glomerulopathy

Case report of a recurrent nephrotic syndrome patient with sudden onset of blindness during treatment with cyclosporin A

Case report of a recurrent nephrotic syndrome patient with sudden onset of blindness during treatment with cyclosporin A

Outcome of children and adolescents with recurrent nephrotic syndrome and focal segmental glomerulosclerosis after renal transplantation

Indications for prophylaxis and treatment of recurrent FSGS after renal transplantation (Tx) are controversial. We have reviewed our experience with 40 pediatric patients (37 cadaver grafts) grafted from 1970 – 1995 because of primary NS associated with FSGS. Mean age at 1st Tx was 10.9 yrs and mean follow-up 4.9 yrs. Five pts received a 2nd graft. 12 pts developped recurrent NS or severe proteinuria after the 1st Tx. Two of the three recipients of living-related grafts developed recurrent NS. Mean age at onset of NS was higher in pts with recurent NS than in the non-recurrent cases (6.6 vs 4.9 yrs) and duration from onset of NS to ESRD was shorter (3.3 vs 7.2 yrs). 5/12 pts with recurrent and 11/28 pts with non-recurrent disease lost their 1st graft. Mean 1st graft survival was similar in recurrent and in non-recurrent disease. Proteinuria disappeared in 1 pt following introduction of Cyclosporine A (3 mg/kg/d) after 7 yrs of severe NS; serum creatinine is 204 μM/I 18 yrs post Tx. In 2 other pts proteinuria decreased after plasma exchange supplemented by oral cyclophosphamide.

Recurrent nephrotic syndrome in a renal allograft recipient with focal and segmental glomerulosclerosis: Efficacy of plasmapheresis

Summary: We report a 29‐year‐old man with renal failure due to focal and segmental glomerulosclerosis (FSGS) who received a well‐matched cadaveric renal allograft and had the early appearance of the nephrotic syndrome and renal insufficiency. His condition responded dramatically to plasmapheresis. the pathogenesis of FSGS recurring in a renal allograft may involve a recently identified circulating factor. the possible efficacy of plasmapheresis in the treatment of recurrent FSGS may be attributable to the removal of this factor.

Development of nephrotic syndrome in a patient with acute myeloblastic leukemia after treatment with macrophage—Colony-stimulating factor

We describe a patient with acute myeloblastic leukemia (AML) who developed nephrotic syndrome after receiving several courses of chemotherapy, including macrophage—colony-stimulating factor (M-CSF). At the onset of nephrotic syndrome, the patient remained in a hematological remission. A renal biopsy showed diffuse mesangial proliferation with marked glomerular infiltration of macrophages and massive subendothelial and mesangial deposits. After the institution of the combined therapy with corticosteroid, anticoagulant, and dipyridamole, urinary protein excretion was attenuated to less than 1.0 g/day. It should be emphasized that the recurrence of nephrotic syndrome was observed after the following chemotherapy, including M-CSF, whereas the bone marrow still remained completely remitted. In contrast, after the last course of chemotherapy, which did not include M-CSF, urinary protein excretion was not enhanced. Of note is that the renal histology at autopsy showed a remarkable improvement of mesangial hypercellularity with concomitant reduction in the number of glomerular macrophages. These evolutional changes in both proteinuria and glomerular histology suggest a close linkage between the MCSF treatment and macrophage-related glomerular injury. The possibility can be raised that M-CSF accelerated the underlying renal disease in this case through enhancing macrophage accumulation into the glomerulus, leading to the development of nephrotic syndrome.

Usefulness of Plasma Exchange in Recurrent Nephrotic Syndrome Following Renal Transplant

Nephrotic syndrome due to focal segmental glomerulosclerosis (FSGS) frequently recurs even after transplantation and may cause failure of the renal allograft. We report a case in which plasma exchange (PE) was used to treat a 32-year-old patient with biopsy-proven recurrence of FSGS in a second renal transplant after the first allograft had failed. One year after the second renal graft, the patient presented with proteinuria of 5 g/day and a creatinine level of 1.46 mg/dl. A course of 9 PE was performed over a 15-day period. Proteinuria improved rapidly, and 20 days after the last PE, it was already down to 0.8 g/day. This level continued to decrease progressively (0.5 g/day after 3 months) and reached zero after approximately 6 months. Eleven months after PE treatment, the patient had no proteinuria and his plasma creatinine level was normal. In our opinion PE should be instituted early in recurrent nephrotic syndrome after renal transplantation. The optimum frequency of such treatment still has to be established, especially with regard to its use as long-term maintenance therapy.

Effect of plasma protein adsorption on protein excretion in kidney-transplant recipients with recurrent nephrotic syndrome

Background Among patients with the idiopathic nephrotic syndrome who have focal and segmental glomerulosclerosis and undergo renal transplantation, 15 to 55 percent have recurrent nephrotic syndrome. The recurrence may be caused by a plasma factor or factors that increase glomerular permeability, because plasma exchange transiently decreases or abolishes proteinuria in some patients. We studied the effect on proteinuria of the removal of protein (mostly immunoglobulins) by adsorption onto protein A from the plasma of patients with recurrent nephrotic syndrome. Methods Eight patients were treated with one to three cycles of two to seven 1-day sessions of protein adsorption, and the patients' urinary protein excretion was measured repeatedly. Their immunosuppressive regimens were not changed during the treatment. The adsorbed proteins were eluted from the protein A and injected into rats, and the urinary albumin excretion of the rats was measured. Results The protein-adsorption treatment consistently decrea...

The Successful Treatment of Recurrent Nephrotic Syndrome With the MOPP Regimen in a Patient With a Remote History of Hodgkin’s Disease

The association of Hodgkin’s disease and minimal-change nephrotic syndrome is well established. We describe a gentleman who developed Hodgkin’s disease that was treated successfully. Two years later, he developed minimalchange nephrotic syndrome that responded to steroids. Over the next 9 years, the patient experienced two episodes of nephrotic syndrome due to focal segmental glomerulonephritis that were successfully treated with the mustard/vincristine/prednisone/procarbazine (MOPP) regimen. There was no evidence of recurrence of Hodgkin’s lymphoma during any episode of nephrotic syndrome.

Effect of Plasma Protein Adsorption on Protein Excretion in Kidney-Transplant Recipients with Recurrent Nephrotic Syndrome

Among patients with the idiopathic nephrotic syndrome who have focal and segmental glomerulosclerosis and undergo renal transplantation, 15 to 55 percent have recurrent nephrotic syndrome. The recurrence may be caused by a plasma factor or factors that increase glomerular permeability, because plasma exchange transiently decreases or abolishes proteinuria in some patients. We studied the effect on proteinuria of the removal of protein (mostly immunoglobulins) by adsorption onto protein A from the plasma of patients with recurrent nephrotic syndrome. Eight patients were treated with one to three cycles of two to seven 1-day sessions of protein adsorption, and the patients' urinary protein excretion was measured repeatedly. Their immunosuppressive regimens were not changed during the treatment. The adsorbed proteins were eluted from the protein A and injected into rats, and the urinary albumin excretion of the rats was measured. The protein-adsorption treatment consistently decreased urinary protein excretion by an average of 82 percent at the end of a cycle (P < 0.001). In one patient proteinuria disappeared, and in another urinary protein excretion remained below 2.5 g per day with repeated cycles of protein adsorption. In all but one patient the effect of adsorption was limited in time, with a return to the preadsorption level of protein excretion within a maximum of two months. The administration to rats of material eluted from the protein A increased urinary albumin excretion 2.9- to 4.6-fold (P < 0.001 and P = 0.005, respectively). Although protein A primarily binds immunoglobulins, the active fraction of the eluted proteins had a molecular weight below 100,000, indicating that immunoglobulin was not directly involved. Adsorption of plasma protein decreases urinary protein excretion in patients with recurrence of the nephrotic syndrome after renal transplantation. Studies of the adsorbed proteins should provide information about the mechanism of this disease.

Recurrent Nephrotic Syndrome Associated with Kimura’s Disease in a Young Non-Oriental Male

Kimura's disease (KD) is an angiolymphoid proliferative disorder of soft tissue with eosinophilia, with a predilection for head and neck regions in young Oriental men. Renal disease is rarely associated with it. We describe a young non-Oriental male with KD and relapsing steroid-responsive minimal-change nephrotic syndrome. This case of KD associated nephrotic syndrome is unique in the relapsing nature of the nephropathy and the non-Oriental origin of the patient. We comment on KD and nephrotic syndrome and on treatment strategy.

Nephrotic syndrome in children.

Proteinuria in children with idiopathic nephrotic syndrome is secondary to a loss of charge selectivity of the glomerular basement membrane. Loss of anionic charges may be secondary to a defect of heparan sulfate proteoglycans, which is also found in the congenital nephrotic syndrome, or to cationic proteins, which neutralize the anionic charges of the membrane. Reports on recurrence of nephrotic syndrome in patients with steroid-resistant idiopathic nephrotic syndrome following renal transplantation suggest that a humoral factor, possibly produced by T lymphocytes, may enhance glomerular permeability. Corticosteroids remain the basic treatment of idiopathic nephrotic syndrome. Most patients respond to steroid therapy and a high proportion of them relapse but continue to respond throughout the subsequent course of the disease. Levamisole may be effective in preventing relapses. Cyclosporine may be useful in steroid-dependent patients with signs of steroid toxicity and after a failure of a course of alkylating agent. Almost 85% of patients respond to cyclosporine, but they relapse after tapering or stopping the drug. In steroid-resistant patients, there is no study showing a clear-cut beneficial effect of alkylating agents, as the remission rate after treatment is close to the rate of spontaneous remission. Cyclosporine in association with prednisone may be effective, but the risk of nephrotoxicity seems to be higher than in steroid-dependent patients.

Steroid-dependent nephrotic syndrome following renal transplantation for congenital nephrotic syndrome

A boy developed recurrent steroid-responsive nephrotic syndrome following renal transplantation for congenital nephrotic syndrome. The first episode was associated with mild tubulointerstitial rejection on kidney biopsy. Subsequent episodes showed normal histology by light microscopy and epithelial foot process fusion on electron microscopy, consistent with minimal change nephrotic syndrome. Serum analysis for soluble immune response suppressor was negative pre-nephrectomy, positive during each bout of nephrotic syndrome, and negative during each remission. This case represents de novo occurrence of steroid-sensitive minimal change nephrotic syndrome following renal transplantation for congenital nephrotic syndrome. We stress the need for histological examination of the renal allograft to diagnose rejection, recurrent disease, or de novo disease.

Glomerular basement membrane thickness in recurrent and persistent hematuria and nephrotic syndrome: correlation with sex and age.

The glomerular basement membranes (GBM) were measured in 264 patients with recurrent and persistent hematuria (152 males, 112 females), 47 patients with minimal change nephrotic syndrome (26 males, 21 females), and 91 patients with focal segmental glomerular sclerosis and nephrotic syndrome (55 males, 36 females). The average number of glomeruli measured was 2.5 per case. Analysis of the data showed GBM thickness to be significantly greater in males (n = 233) than in females (n = 169) (Student's t-test, P less than 0.01). There was no difference in GBM thickness between the two groups of nephrotics; the data were subsequently combined and the group referred to as idiopathic nephrotic syndrome. There was also no difference in GBM thickness among the immunofluorescence-defined subcategories of recurrent hematuria, and these groups were combined. GBM thickness was significantly greater in the idiopathic nephrotic syndrome than in recurrent hematuria (Kruskal-Wallis H test, Po = less than 0.001). Analysis of the data showed, in both conditions, a gradual increase with age throughout the span of the study (1-69 years). Regression analysis of GBM thickness and age in recurrent hematuria showed Ro = 0.43 and Po = less than 0.001, in idiopathic nephrotic syndrome Ro = 0.61 and Po = less than 0.001. The differences between nephrotic syndrome and recurrent hematuria may be based on the inclusion in the recurrent hematuria group of patients with genetically thin GBM; they may also be based on the inclusion in the idiopathic nephrotic syndrome group of patients with subendothelial thickening, a relatively frequent occurrence.(ABSTRACT TRUNCATED AT 250 WORDS)

The Congenital Nephrotic Syndrome: A Review and Case Report

The congenital nephrotic syndrome (CNS) is a rare form of neo-natal nephropathy that generally occurs within the first three months of life, and is characterized by proteinuria, placentamegaly, and retarded intrauterine growth. Proteinuria in infants can result from a variety of other causes such as congenital infections (syphilis, CMV, etc.), mercury intoxication, and minimal change lesion, but CNS is the only nephrotic syndrome of neonates that is unresponsive to immunosuppressive drugs.This syndrome was first defined and extensively studied in Finland where this condition was estimated to occur in 1 per 10,000 births, and was found to be an expression of an autosomal recessive inheritence. It has also occured in children without a history of Finnish ancestry.The prognosis for these children is poor, most often resulting in death by the fourth year of life unless a renal transplant is received. A study of 17 transplants in children with CNS reported a 71% graft survival rate over a two year period with no recurrence of nephrotic syndrome in any transplants.

Spontaneous remission of nephrotic syndrome in IgA glomerular disease.

We report two cases of adult onset nephrotic syndrome with pathologic features of minimal change disease but a predominance of IgA in mesangial regions. Both cases underwent spontaneous remission despite an episode of recurrent nephrotic syndrome in one patient. The relationship of the glomerular disease to minimal change and IgA nephritis is discussed.

Meclofenamate Treatment of Recurrent Idiopathic Nephrotic Syndrome With Focal Segmental Glomerulosclerosis After Renal Transplantation

Recurrent corticosteroid-resistant nephrotic syndrome with focal segmental glomerulosclerosis (FSGS) caused the failure of a first renal allograft in a 41-year-old man. Recurrence of the nephrotic syndrome in the second renal allograft was successfully controlled by the administration of meclofenamate, and the renal function has remained stable for 2 1/2 years. No accepted treatment is available for corticosteroid-resistant nephrotic syndrome with FSGS. This report suggests that administration of meclofenamate might be beneficial in some patients with corticosteroid-resistant nephrotic syndrome and FSGS. Because of the potential side effects, however, careful supervision of this therapy is of the utmost importance.