1/11. Renal Behcet's disease: a cumulative analysis.OBJECTIVE: To analyze cumulated data about renal involvement in Behcet's disease (BD) and to report on 6 patients with BD and renal problems. methods: We found reports of 159 patients (including our patients) with BD and specific renal disease (amyloidosis 69, glomerulonephritis [GN] 51, renal vascular disease 35, and interstitial nephritis 4) in our survey. RESULTS: The frequency of renal problems among BD patients has been reported to vary between 0% to 55%. male gender is a risk factor for all types of renal BD. nephrotic syndrome was present in 83% of patients with amyloidosis, and renal failure was common at the time of diagnosis. The mean interval between the initial manifestation of BD and diagnosis of amyloidosis was shorter in men than in women (P =.02). AA-type amyloid fibrils were shown in all cases studied. Vascular involvement was common in the patients with amyloidosis (60%). The renal findings in GN show a wide spectrum, from asymptomatic hematuria and/or proteinuria to rapidly progressive GN. Several types of glomerular lesions ranging from minor glomerular changes to crescentic glomerulonephritis are observed in BD. The common types of glomerular lesions among the reported cases are crescentic GN, proliferative GN, and immunoglobulin a (IgA) nephritis. Aneurysms may be located throughout the renal artery, from the orifice of the main artery to intrarenal microaneurysms. Another type of renal disease (amyloidosis or GN) and other major vascular involvement were present in all cases with renal vein thrombosis. hypertension is common among patients with renal artery aneurysm or stenosis. Microscopic vascular disease was described in 4 patients. CONCLUSIONS: Based on data in the literature, we suggest that renal involvement in BD is more frequent than has been recognized, although it is most often mild in nature. amyloidosis is one of the prognostic factors affecting survival. patients with vascular involvement carry high risk for amyloidosis, and administration of colchicine to these patients may be beneficial. More evidence is needed to accept interstitial nephritis as a manifestation of BD. In spite of some difficulties, hemodialysis and renal transplantation are safe treatment options in BD-related uremia.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
2/11. nephrotic syndrome associated with fanconi syndrome. Immunopathogenic studies of tubulointerstitial nephritis with autologous immune-complex glomerulonephritis.The nature of renal lesions in a patient with simultaneous onset of the fanconi syndrome and nephrotic syndrome was investigated by immunologic studies of the patient's serum, cryoproteins, and renal tissue. Acute severe tubulointerstitial nephritis and generalized segmental glomerulonephritis were present. Renal tubular epithelial (RTE) antigen, IgG, and Clq were localized in the glomerull and proximal tubules. Cryoprecipitates containing RTE antigen and anti-RTE antigen were isolated from the patient's serum antibody to RTE antigen was detected in the serum of the patient. However, antibody to tubular basement membrane was not found in the cryoproteins or serum. The unusual simultaneous presentation of these two syndromes in our patient possibly represents a common etiology: tubular damage with release of RTE antigen and subsequent development of immune-complex glomerulonephritis mediated by renal tubular epithelial antigen and antibody to this antigen.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
3/11. C1q nephropathy: do C1q deposits have any prognostic significance in the nephrotic syndrome?C1q deposits are usually found in association with other complement components and immunoglobulins in proliferative glomerulonephritis and may predominate in systemic lupus erythematosus (SLE). We report the clinical outcome of four patients who developed a nephrotic syndrome associated with C1q nephropathy unrelated to SLE. On presentation the mean urinary protein loss was 6.8 g/24 h (range 4-10), and renal function impaired, mean serum creatinine 201 mumol/l (150-400). Over a mean follow up period of 6.5 years (1.7-19), all four patients improved, three spontaneously and one treated with steroids and cyclosporin, to a current urinary protein loss of 0.3 g/24 h (less than 0.2-0.9) and serum creatinine 98 mumol/l (68-115). C1q nephropathy was confirmed in each biopsy by conventional immunohistology. C1q deposits were demonstrated within the glomerular basement membrane of three biopsies and the mesangium in two samples. One patient had been categorized on light- and electron-microscopy as having mesangiocapillary glomerulonephritis, one membranous glomerulonephritis, one proliferative glomerulonephritis with focal segmental glomerulosclerosis, and one diffuse proliferative glomerulonephritis with both subendothelial and mesangial dense deposits. In view of the expected progressive nature of the underlying renal histopathological appearance, the presence of predominant C1q deposits would appear to be associated with a better clinical outcome.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
4/11. Acute renal failure in children with idiopathic nephrotic syndrome.Acute renal failure (ARF) is an uncommon but alarming complication of idiopathic nephrotic syndrome. The renal failure could be secondary to causes evident from the history and evaluation, such as severe intravascular volume depletion, acute tubular necrosis, allergic interstitial nephritis, bilateral renal vein thrombosis, acute pyelonephritis, or rapid progression of the original glomerular disease. It may be termed idiopathic if the underlying cause is undetermined. We present three children with idiopathic nephrotic syndrome who were admitted with acute renal failure. One case was due to drug-induced allergic interstitial nephritis. The other two were idiopathic in nature. Improvement in renal function occurred in the three patients over a variable period of 10 days to 4 weeks. After careful exclusion of well-known causes of acute renal failure, idiopathic acute renal failure (IARF) should be considered as a diagnostic possibility in these patients. The exact pathophysiology of IARF is not understood. Possible proposed explanations include interstitial edema, tubular obstruction, altered glomerular permeability, and unrecognized hypovolemia.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
5/11. Localised plasma-cell type Castleman's disease and AA-amyloidosis cured by resection. A case report and review of the literature.Castleman's disease (CD) is a rare atypical lymphoproliferative disorder with frequent, yet less well known renal involvement. We describe the case of a 58-year-old woman with localised abdominal plasma-cell type CD complicated by nephrotic syndrome due to renal amyloidosis. 18Fluorodeoxyglucose (FDG) positron emission tomography (PET) confirmed the unicentric nature. Resection of the mass resulted in regression of the signs of inflammation, a negative FDG-PET and complete remission of the nephrotic syndrome. A review of the literature of renal involvement (incidence, clinical and pathological manifestations, treatment and prognosis) in CD is provided.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
6/11. Pulmonary thrombo-embolism in the nephrotic syndrome.The case of a 10.5-year-old boy who had complete right main pulmonary artery thromboembolism complicating steroid-resistant nephrotic syndrome is presented. Despite the massive nature of the lesion clinical symptoms were relatively minor. The thromboembolism was successfully treated with intrapulmonary artery streptokinase. The case highlights the discrepancy that may be found between the clinical and pathological findings in pulmonary thromboembolism in the nephrotic syndrome of childhood.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
7/11. Immune deposit nephritis and single-component cryoglobulinemia associated with chronic lymphocytic leukemia. Evidence for a role of circulating IgG-anti-IgG immune complexes in the pathogenesis of the renal lesion.2 patients developed the nephrotic syndrome several years after diagnoses of chronic lymphocytic leukemia. In both cases light microscopy showed membranoproliferative glomerulonephritis. Electron microscopy and immunofluorescent staining revealed electron-dense deposits and deposition of immunoglobulins and C3. Both patients had single-component IgG cryoglobulinemia. The eluted glomerular-bound protein contained IgG only. IgG in patients' sera, cryoglobulins, and kidney eluate had kappa light chains only. Immune complexes were detected in the sera and in the cryoglobulins by the Clq binding test. Immunoadsorption studies revealed anti-IgG antibodies in the patients' sera, cryoglobulin, and kidney eluate. Direct immunofluorescent studies using the patients' sera, cryoglobulins, and kidney eluate on frozen sections of patients' kidneys were positive, providing additional evidence for the immune complex nature of the glomerulonephritis. The immunohistochemical studies of our patients are suggestive of the presence of circulating IgG-anti-IgG immune complexes and their possible involvement in the pathogenesis of the glomerulonephritis and the nephrotic syndrome in these 2 cases.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
8/11. nephrotic syndrome and immune complex glomerulonephritis associated with chlorpropamide therapy.Therapeutic drugs are well-recognized as a cause of the nephrotic syndrome in humans. However, documentation of the renal histopathologic features is lacking or incomplete in many cases. Even when accurate histopathologic information is available, there is little evidence to support a specific pathogenetic mechanism of renal injury in the vast majority of cases. We describe a patient with diabetes who had hepatitis and dermatitis in association with the use of chlorpropamide. In addition to these well-described toxic reactions to this drug, the nephrotic syndrome developed. Renal biopsy revealed the presence of a proliferative glomerulonephritis that was shown to be of an immune complex nature on immunofluorescence and electronmicroscopic study. Serial serum complement levels and circulating immune complex levels were consistent with an immunologically mediated reaction. Repeated renal biopsy documented resolution of the renal changes. Thus, in this patient, a drug-induced nephrotic syndrome was associated with a proliferative glomerulonephritis, probably due to the formation of immune complexes.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
9/11. Acquired protein s and antithrombin iii deficiency caused by nephrotic syndrome: an unusual cause of graft thrombosis.Thrombotic phenomena are well-recognized complications of nephrotic syndrome attributable to loss of intermediate-sized antithrombotic proteins in the urine, resulting in a hypercoaguable state. As such, nephrotic syndrome may be associated with a reduction in circulating antithrombin III and free protein s levels. Associated spontaneous thrombotic complications are generally venous in nature, with arterial thrombosis occurring less frequently. Hypercoagulability caused by acquired nephrotic syndrome has not generally been recognized as a cause of acute thrombosis of arterial bypass grafts. We report two patients who after having nephrotic syndrome sustained acute thrombosis of their arterial bypass grafts. Pathogenesis and management are discussed.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
10/11. Idiopathic retroperitoneal fibrosis presented as an abdominal mass and nephrotic syndrome.We present a 30-year-old male patient who was initially diagnosed as minimal change nephrotic syndrome, 5 years later, the patient developed a localized form of idiopathic retroperitoneal fibrosis (IRF). An elevated ESR and concomitant nephrotic syndrome in the patient suggested the immunologic nature of IRF, IRF has been reported in association with collagen diseases and rarely with proliferative and nonproliferative glomerulopathies. To our knowledge, the association between minimal change lesion (MC) and IRF has not been reported. Furthermore, the fact that IRF presented itself as an abdominal mass and lacked systemic symptoms was also unusual.- - - - - - - - - - ranking = 1keywords = nature (Clic here for more details about this article) |
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