1/187. nephrotic syndrome as a clinical manifestation of graft-versus-host disease (GVHD) in a marrow transplant recipient after cyclosporine withdrawal.GVHD is one of the most frequent complications of BMT and recently nephrotic syndrome (NS) has been described as a manifestation of chronic GVHD. Here, we present an AA patient who developed NS 1 year after BMT when cyclosporine was stopped. Renal biopsy showed focal sclerosis associated with membranous deposits. He also had other clinical manifestations of chronic GVHD: sicca-like syndrome and colestasis. After 15 days of CsA therapy, he experienced a remarkable improvement in the NS and GVHD as a whole. We comment on immunological mechanisms that could be involved in the pathogenesis of this manifestation.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
2/187. 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 = 6keywords = sclerosis (Clic here for more details about this article) |
3/187. Cyclosporin A mono-therapy in nephrotic syndrome with contra-indication of steroid therapy.We describe three cases of nephrotic syndrome with a contra-indication for steroid therapy successfully treated with cyclosporin A (CsA). A 21-year-old man with focal segmental glomerulosclerosis (FSGS) complicated by necrosis of the femoral head, and a 34-year-old woman and a 48-year-old man with minimal change disease (MCD) complicated by psychogenic reaction and diabetes mellitus, respectively, were given CsA at initial dosages of 3.8-5.0 mg/kg/day and immediately remitted completely. However, two of these patients suffered relapses when CsA was tapered. They are currently maintained in complete or partial remission on CsA at dosages of 3.2-4.7 mg/kg/day. These findings suggest that CsA mono-therapy may be useful in nephrotic syndrome patients contra-indicated for steroid therapy.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
4/187. 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 = 3keywords = sclerosis (Clic here for more details about this article) |
5/187. Congenital nephrotic syndrome responsive to captopril and indometacin.Two children with congenital nephrotic syndrome are described (one with Finnish-type nephrosis, the other with diffuse mesangial sclerosis). Both children have had a prolonged and sustained clinical response with good physical health and normal growth patterns using captopril and indometacin as their sole treatment. No adverse effects have been noted. We recommend a trial of indometacin and captopril treatment in cases of congenital nephrotic syndrome.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
6/187. nephrotic syndrome and end-stage renal disease with WT1 mutation detected at 3 years.We report a boy who presented at 3 years with nephrotic syndrome and end-stage renal failure. Although histopathological findings showed end-stage kidney, isolated diffuse mesangial sclerosis (IDMS) was suspected because of his clinical course, and was confirmed by the presence of WT1 (wilms tumor suppressor gene) mutation. He did not have ambiguous genitalia or wilms tumor. The karyotype was 46:XY. A constitutional mutation in exon 7 (953G-->A, 312Arg-->Gin) was detected. A few cases of male IDMS, associated with WT1 mutations, have been reported. We believe that investigation for the WT1 mutation should be performed not only in denys-drash syndrome and IDMS, but also in end-stage renal disease with unexplained nephrotic syndrome of early onset. WT1 mutation-associated nephrotic syndrome has an increased risk of wilms tumor. Careful ultrasound evaluations or bilateral nephrectomies are indicated.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
7/187. 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 = 1keywords = sclerosis (Clic here for more details about this article) |
8/187. Acute lymphoblastic leukaemia after treatment of nephrotic syndrome with immunosuppressive drugs.The authors present a 4-year-old girl with nephrotic syndrome who developed actue lymphoblastic leukaemia 5 months after the start of a combined treatment of alternate day prednisone and daily cyclophosphamide during 3 months. The nephrotic syndrome was due to focal segmental glomerulosclerosis. The occurrence of leukaemia might be related to the preceeding treatment with cyclophosphamide.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
9/187. Extensive intraglomerular thrombi of monoclonal IgM-kappa in a patient with malignant lymphoma.We describe an 80-year-old man who developed malignant lymphoma (ML) complicated by extensive intraglomerular thrombi of immunoglobulin m (IgM)-kappa monoclonal immunoglobulin. The clinical picture was characterized by nephrotic syndrome and systemic lymphadenopathy. Laboratory examination showed mild anemia and a small amount of monoclonal IgM-kappa in the blood. The histopathologic findings and surface immunoglobulin analysis of the lymph node biopsy specimen were consistent with CD5-positive diffuse large B-cell (type, IgM-kappa) lymphoma. The subsequent renal biopsy showed a massive deposition of amorphous material in the glomerular capillary lumens, subendothelial areas, and mesangium. Nodular glomerulosclerosis was not found. An immunofluorescent study showed that the deposits consisted of IgM-kappa monoclonal immunoglobulin. Ultrastructurally, the deposits were composed of granular electron-dense material. Chemotherapy was effective for both the ML and nephrotic syndrome, and the patient's urine analysis results returned to normal. The histopathologic manifestations of this case are rare, and the pathogenesis of these glomerular lesions was obviously associated with ML.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
10/187. Thin basement membrane disease with heavy proteinuria or nephrotic syndrome at presentation.Thin basement membrane disease (TBMD) is a condition originally defined as diffuse thinning of the glomerular basement membrane (GBM) associated with hematuria in all patients. Although proteinuria has been described in up to 60% of patients with TBMD, it is almost always mild, with a 24-hour excretion mostly of less than 500 mg. We describe eight patients (four men and four women between 32 and 66 years of age) with TBMD who presented with heavy proteinuria or nephrotic syndrome. Among the seven cases with family history, hematuria was noted in five. All patients had a long history of microscopic hematuria, with episodic gross hematuria in two. Renal biopsies showed diffuse thinning of the GBM in each patient (mean between 185.3 x 29.8 nm and 232.6 x 34.5 nm versus control between 325 x 35 nm and 350 x 15 nm). Three cases showed thinning of GBM only (group I); the remaining five cases showed thinning of GBM associated with focal segmental glomerulosclerosis. All three patients of group I presented with nephrotic syndrome and normal renal function. Treatment with steroids resulted in remission of nephrotic syndrome in two, whereas nephrotic syndrome persisted in the untreated patient. Among the five patients in group II, nephrotic syndrome and normal renal function at presentation were noted in two, whereas the other three had heavy proteinuria (2.2, 2. 5, and 2.6 g/d, respectively) associated with mildly decreased renal function (serum creatinine 1.8, 1.3, and 1.5 mg/dL, respectively). At last follow-up, although the renal function was stable in all five, only the three who received steroid treatment had remission or marked improvement of proteinuria. hematuria, however, persisted in all eight patients of both groups. Whether specific gene mutations are translated into structural changes responsible for both excessive GBM thinning and increased transcapillary permeability remains to be elucidated. Alternatively, the heavy proteinuria/nephrotic syndrome may not be related to TBMD, but rather is the manifestation of associated glomerular diseases. Follow-up, including a response to steroids, supports the latter hypothesis.- - - - - - - - - - ranking = 1keywords = sclerosis (Clic here for more details about this article) |
| | Next -> |