1/102. 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 = nephritis (Clic here for more details about this article) |
2/102. 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 = 0.5keywords = nephritis (Clic here for more details about this article) |
3/102. 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 = 0.5keywords = nephritis (Clic here for more details about this article) |
4/102. Development of focal segmental glomerulosclerosis in the renal allograft of a patient with lupus.Nephritis has been a recognized complication of systemic lupus erythematosus since the early 1900s. Almost all lupus patients have some degree of renal involvement related to their condition, but a considerably smaller proportion of these patients actually progress to end-stage renal disease (ESRD). However, lupus patients are also susceptible to other primary renal insults that may significantly contribute to the deterioration in their renal function. We present a case of a patient with clinical and pathological evidence of lupus nephritis that progressed to ESRD and subsequently developed "recurrent" focal segmental glomerulosclerosis in her transplant kidney. Retrospective clinicopathologic correlation suggested the possibility of more than 1 primary renal process that eventually led to her dialysis-dependent state. This case illustrates the importance of meticulously examining both clinical and renal biopsy data in patients with lupus nephritis and considering the presence of co-existing renal pathologies to resolve an otherwise discordant picture of disease progression. These considerations may have important therapeutic and prognostic implications.- - - - - - - - - - ranking = 1keywords = nephritis (Clic here for more details about this article) |
5/102. Nodular glomerulosclerosis secondary to mu heavy chain deposits.mu heavy chain deposition disease is very rare. We report the first case of glomerulonephritis in a woman without evidence of hematopoietic malignancy. Nodular glomerulosclerosis and monotypic mu heavy chain mesangial deposits were identified by immunofluorescence without kappa or lambda deposits. Electron microscopy showed fibrillar mesangial deposits of 16-18 nm in diameter. serum immunoglobulins, cryoglobulins, serum immunoelectrophoresis, and immunofixation, bone marrow biopsy, and Bence Jones proteins in urine were negative. The patient has stable renal disease and is free of malignancy 6 years after the initial occurrence of proteinuria.- - - - - - - - - - ranking = 0.5keywords = nephritis (Clic here for more details about this article) |
6/102. Renal transplantation in systemic amyloidosis.Renal transplantation was performed in a 24-yr-old woman with type AA systemic amyloidosis. Renal biopsy at 11 yr post-transplantation showed no amyloid recurrence, but the presence of mesangial proliferative glomerulonephritis and focal segmental glomerular sclerosis. Renal function was satisfactory with the exception of mild proteinuria. Symptoms related to systemic amyloidosis including goiter and cardiac deposition, improved post-transplantation. Renal transplantation is the recommended therapy for type AA systemic amyloidosis.- - - - - - - - - - ranking = 0.5keywords = nephritis (Clic here for more details about this article) |
7/102. Two cases of hypothyroidism complicated by renal dysfunction.We report two patients in whom hypothyroidism was considered to cause renal dysfunction. Case 1 was a 65-year-old woman who stopped taking levothyroxine sodium for hypothyroidism. After 6 months, she developed proteinuria, edema, weight gain, and renal dysfunction. Renal biopsy revealed focal segmental proliferative glomerulonephritis. After re-administration of levothyroxine sodium, thyroid function and renal function both recovered. Case 2 was a 51-year-old man who presented with edema, difficulty in swallowing, muscular weakness, and fatigue. We diagnosed hypothyroidism, and focal segmental proliferative glomerulonephritis was revealed by renal biopsy. After administration of levothyroxine sodium, his symptoms resolved and his thyroid function and renal function both improved. Our experience suggests that hypothyroidism should be taken into consideration as one of the causes of renal dysfunction.- - - - - - - - - - ranking = 1keywords = nephritis (Clic here for more details about this article) |
8/102. Chronic inflammatory demyelinating polyradiculoneuropathy and severe peripheral oedema: a renal explanation.Inflammatory demyelinating neuropathies have been associated with membranous and focal sclerosing glomerulonephritis. Here we describe a 58 year old man with a clinical history, physical examination and laboratory investigations consistent with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), who also had severe lower limb and sacral oedema resistant to medical therapy. Mild proteinuria was present and a renal biopsy showed features consistent with focal sclerosing glomerulonephritis (FSGN). The patient's weakness and oedema did not respond to i.v. immunoglobulin or plasmapheresis but responded to high dose oral prednisone. The oedema was not explained by immobility, hypoproteinaemia or local factors. The occurrence of the oedema in a person with CIDP and FSGN and its improvement with prednisone, together with improvement in CIDP and FSGN, suggests that it was immune mediated, possibly due to increased capillary permeability. The presence of renal disease in patients with inflammatory demyelinating neuropathies may be more common than currently realised.- - - - - - - - - - ranking = 1keywords = nephritis (Clic here for more details about this article) |
9/102. Synchronous herpes simplex virus and cytomegalovirus esophagitis.Infective esophagitis is a rare disease, affecting mostly immunocompromised patients. Very few cases of a multiple viral infection have been reported. We present a case of combined cytomegalovirus (CMV) and herpes simplex virus (HSV) esophagitis in an 81-year-old female with extracapillary sclerosing glomerulonephritis treated for five months with steroids and chemotherapy. She died of septic shock. At autopsy, erosive and ulcerative esophagitis was found in the distal half of the esophagus. Slides were stained by HE, and the immunohistochemical avidin-biotin method was used to detect HSV and CMV infection. On histological examination of the esophagus, epithelial giant cells with intranuclear viral inclusions showing HSV immunopositivity were found at the margin of the ulcerations. giant cells with intranuclear inclusions with CMV immunopositivity were also found in the mesenchymal cells obtained from the ulcer bed. Long-term immunosuppressive therapy provoked an immune deficiency, evidenced by grave leukopenia and depletion of all bone marrow elements. diagnosis of HSV and CMV esophagitis is important to evaluate the risk of hemorrhage and esophageal perforation in esophagitis.- - - - - - - - - - ranking = 0.5keywords = nephritis (Clic here for more details about this article) |
10/102. 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 = 0.5keywords = nephritis (Clic here for more details about this article) |
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