1/176. Primary perireticulin amyloidosis in a 14-year-old girl.A primary perireticulin amyloidosis is reported in a 14-year-old girl, which showed the symptoms of a steroid-resistant nephrotic syndrome. The diagnosis was established by biopsies of kidney and rectum. Occurrence of amyloidosis in childhood and the clinical picture are discussed and references to clinical trials carried out are given.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
2/176. fibrinogen A alpha chain mutation (Arg554 Leu) associated with hereditary renal amyloidosis in a French family.A French family with hereditary renal amyloidosis (HRA) was studied. The disease presented in 7 of the 8 affected individuals with proteinuria or the nephrotic syndrome. The age of onset was in the fifth decade of life. There is currently no sign of extrarenal involvement in any affected individual. However, the nephropathy in this family is progressive and led to terminal renal failure in 4 patients. immunohistochemistry studies of glomerular amyloid deposits suggested that the amyloid protein was the fibrinogen A alpha chain. Direct dna sequencing revealed a G 4993 T transversion and subsequently Arg 554 Leu mutation in the fibrinogen A alpha chain. This is the first description of this fibrinogen A alpha chain mutation in europe. This family is of French descent and cannot be related to the previously reported Peruvian/Mexican and African-American kindreds.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
3/176. Remission of the nephrotic syndrome in a patient with renal amyloidosis due to rheumatoid arthritis treated with prednisolone and methotrexate.A 46-year-old woman developed nephrotic syndrome secondary to rheumatoid arthritis (RA). A renal biopsy showed deposition of amyloid fibrils in the subendothelial space of the glomerular capillary walls. After treatment with prednisolone (PSL, 40 mg/day), the levels of c-reactive protein (CRP) and serum amyloid A decreased to within normal limits for 2 weeks. However, the nephrotic syndrome persisted for 6 months after the therapy. To maintain the suppression of disease activity and to reduce PSL, methotrexate (5 mg/week) was added. The nephrotic syndrome resolved gradually, and the level of serum albumin returned to normal. Although renal prognosis of patients with nephrotic syndrome due to amyloidosis caused by RA has been considered poor, adequate and long-term treatment of RA with antiinflammatory drugs, including PSL and methotrexate, is useful for patients with secondary amyloidosis complicated by RA.- - - - - - - - - - ranking = 8keywords = nephrotic syndrome (Clic here for more details about this article) |
4/176. Renal amyloidosis with nephrotic syndrome in two patients with schistosomiasis mansoni and chronic salmonellosis.This report describes two Egyptian patients who presented with the nephrotic syndrome and concurrent infections with schistosoma mansoni and salmonella paratyphi a. Unlike similar cases previously reported from this unit, these patients did not respond to antimicrobial and antischistosomal therapy, and their renal biopsies demonstrated amyloidosis. These two case reports and several experimental observations suggest that chronic schistosomiasis and salmonellosis may lead to secondary amyloidosis in susceptible individuals.- - - - - - - - - - ranking = 5keywords = nephrotic syndrome (Clic here for more details about this article) |
5/176. amyloidosis: recognition, confirmation, prognosis, and therapy.amyloidosis should be considered in any patient older than 40 years who has nephrotic syndrome, congestive heart failure (not on an ischemic basis), idiopathic peripheral neuropathy, or unexplained hepatomegaly. When a patient has one of these problems, immunoelectrophoresis and immunofixation of the serum and urine should be done for the detection of a monoclonal light chain. If a monoclonal light chain is found, a diagnosis usually can be established by amyloid stains performed on a bone marrow biopsy specimen or a subcutaneous fat aspirate. The presence or absence of cardiac involvement with amyloid is the most important prognostic factor. Treatment can range from observation to oral chemotherapy to hematopoietic stem cell transplantation. A practical understanding of the mechanisms underlying this disease can lead to prompt diagnosis and early therapeutic intervention.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
6/176. Rapid reversal of nephrotic syndrome due to primary systemic AL amyloidosis after VAD and subsequent high-dose chemotherapy with autologous stem cell support.In a patient with nephrotic syndrome, renal biopsy revealed AL amyloid deposits. Monoclonal lambda light chains were identified in serum and urine. A low percentage of monoclonal plasma cells was detected in the bone marrow. The patient received four cycles of VAD and subsequent high-dose chemotherapy (HDCT) with melphalan (200 mg/m2) followed by autologous peripheral blood stem cell transplantation. proteinuria rapidly diminished during chemotherapy. Three months after HDCT, the patient has no edema, and no signs of plasma cell dyscrasia are currently detectable. Using VAD before starting HDCT may improve the condition of patients with amyloidosis and reduce transplantation-related morbidity and mortality.- - - - - - - - - - ranking = 5keywords = nephrotic syndrome (Clic here for more details about this article) |
7/176. A patient with multiple myeloma, amyloidosis and light-chain deposition disease in kidneys with a long survival.We report a case of a 39-year-old man with lambda-light-chain multiple myeloma, nephrotic syndrome due to lambda-light-chain deposition disease in kidneys and amyloidosis in other tissues. The patient was treated with melphalan and prednisone for 1 year. After that, he was administered interferon-alpha2b (IFN-alpha2b; 3 MU, 3 times a week) as maintenance therapy for 2 years. At present, 5 years and 6 months after the initial diagnosis, the patient receives IFN-alpha2b (3 MU, twice a week) and remains in complete haematological remission.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
8/176. Reversal of nephrotic syndrome due to AA amyloidosis in psoriatic patients on long-term colchicine treatment. Case report and review of the literature.A case of nephrotic syndrome due to AA amyloidosis in a young woman suffering from erythrodermic psoriasis and psoriatic arthropathy is reported. The nephrotic syndrome regressed completely during long-term (57 months) colchicine treatment. There are 39 case reports in the literature of psoriasis associated with amyloidosis. More than 85% of these patients had concomitant arthropathy. This suggests that arthritis may be an important factor in the appearance of amyloidosis. 59% of psoriatics with amyloidosis had renal failure and 56% of them died shortly after diagnosis of amyloidosis. These observations support the view that amyloidosis associated with psoriasis is an aggressive disease that may be fatal. However, the clinical course of our patient suggests that renal amyloidosis associated with psoriasis may be successfully treated by colchicine.- - - - - - - - - - ranking = 6keywords = nephrotic syndrome (Clic here for more details about this article) |
9/176. hepatomegaly and cholestasis as primary clinical manifestations of an AL-kappa amyloidosis.A 53-year-old man, who presented with weight loss over a period of 10 months, hepatomegaly, markedly raised cholestatic enzymes and Ca 19-9, was initially suspected of suffering from metastatic cholangio-carcinoma. liver biopsy revealed depositions of AL-amyloid. Further investigations confirmed a generalized amyloidosis. Biopsies taken from the gastric, colonic, and bronchial mucosa all showed depositions of amyloid. A nephrotic syndrome was interpreted as being secondary to the renal involvement. echocardiography identified changes which were consistent with cardiac involvement. A plasmacytoma or lymphoma was excluded. At the time of diagnosis the patient was in a good physical condition with normal renal function. Within a few weeks the renal function deteriorated and after 2 months the patient developed ascites and became jaundiced. Four months after initial presentation the patient died from cardiac failure.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
10/176. Secondary (AA-type) amyloidosis in patients with polymyalgia rheumatica.Several cases of systemic amyloidosis associated with polymyalgia rheumatica (PMR) or giant-cell arteritis (GCA) have been described. Nevertheless, the type of amyloid deposit has not been characterized in most of them. Here we report on two patients with PMR (one with associated GCA) who developed nephrotic syndrome and end-stage renal failure caused by massive amyloid deposition. Immunohistochemical analysis showed that the amyloid deposits were of AA type (secondary amyloidosis) in both cases.- - - - - - - - - - ranking = 1keywords = nephrotic syndrome (Clic here for more details about this article) |
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