Cases reported "Nephrotic Syndrome"

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11/1179. 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. ( info)

12/1179. Effect of camostat mesilate on urinary protein excretion in three patients with advanced diabetic nephropathy.

    Effective treatment has not yet been established for patients with persistent proteinuria and hypoproteinemia related to advanced diabetic nephropathy. We report three patients with diabetic nephropathy presented with the nephrotic syndrome who showed a marked decrease in proteinuria following the administration of camostat mesilate, a protease inhibitor. Each patient was resistant to treatment with an angiotensin-converting enzyme (ACE) inhibitor and a platelet-aggregation inhibitor. Camostat mesilate, 600 mg/day, orally, caused a marked decrease in urinary protein excretion after the 7th consecutive day of drug administration. There were no serious adverse effects. Its mechanism of action in this respect is not known. Camostat mesilate thus merits clinical trials in the treatment of nephrotic syndrome related to diabetic nephropathy. ( info)

13/1179. mannitol and frusemide in the treatment of diuretic resistant oedema in nephrotic syndrome.

    Three children (two girls aged 7 and 9 years, and one boy aged 4 years) with diuretic resistant oedema in steroid resistant nephrotic syndrome were treated with a combination of intravenous mannitol and frusemide. All three responded with loss of oedema of 10% to 30% of body weight over one week. There were no complications of hypertension or hypovolaemia. mannitol-frusemide combination is a safe, inexpensive, and effective treatment for diuretic resistant oedema. Its use in other conditions and in developing countries (where the availability and purity of 20% albumin is limited) needs to be explored. ( info)

14/1179. 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. ( info)

15/1179. 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. ( info)

16/1179. Cerebral venous thrombosis in patients with nephrotic syndrome--case reports.

    The authors describe two cases of cerebral venous thrombosis (CVT) in patients with nephrotic syndrome. The main clinical features of CVT were persistent headache, hemiparesis, and seizure, and the diagnosis was based on magnetic resonance imaging and magnetic resonance angiography. Both showed acquired deficiency of free protein s. The neurologic symptoms remained stationary in the first patient, who received no anticoagulation therapy, but resolved rapidly in the second, treated with intravenous heparin and supplemented with fresh frozen plasma. CVT should be suspected in patients with nephrotic syndrome who present with symptoms of intracranial hypertension or any focal neurologic deficit. ( info)

17/1179. strongyloidiasis as a possible cause of nephrotic syndrome.

    Chronic strongyloidiasis is a mild disease and has never been reported to be associated with nephrotic syndrome. Disseminated strongyloidiasis is known to have high mortality, but it frequently is not diagnosed until autopsy. We report a patient with nephrotic syndrome developing disseminated strongyloidiasis after steroid therapy. The findings in renal biopsy, the time course of the development, and resolution of nephrotic syndrome after thiabendazole treatment suggested a possible causal relationship between chronic strongyloidiasis and nephrotic syndrome. The case also demonstrated the importance of early diagnosis in disseminated strongyloidiasis and the good clinical outcome of early treatment before the development of organ failure. ( info)

18/1179. purpura of the ears: a distinctive vasculopathy with circulating autoantibodies complicating long-term treatment with levamisole in children.

    The cutaneous side-effects of levamisole include non-specific and lichenoid eruptions, fixed drug eruption and, very rarely, cutaneous vasculitis. We describe a distinctive clinical and histological vasculopathy with immunological abnormalities in children with paediatric nephrotic syndrome receiving long-term levamisole treatment. Four boys and one girl were identified. Their average age was 10 years. levamisole had been used for an average of 24 months. purpura of the ears was the most common finding corresponding histologically to a vasculopathic reaction pattern ranging from a leucocytoclastic and thrombotic vasculitis to a vascular occlusive disease without true vasculitis but with associated antinuclear, antiphospholipid and anticytoplasmic antibodies. The eruption resolved in all patients 2-3 weeks after the discontinuation of levamisole, but serum autoantibodies persisted for 2-14 months. ( info)

19/1179. foscarnet-induced crystalline glomerulonephritis with nephrotic syndrome and acute renal failure after kidney transplantation.

    foscarnet nephrotoxicity has been reported to be associated with acute tubulointerstitial nephritis. Crystals in glomerular capillary lumens have also been observed in patients with acquired immunodeficiency syndrome who were treated with foscarnet for cytomegalovirus disease. We describe a kidney transplant recipient who developed a nephrotic syndrome with microscopic hematuria and nonoliguric acute renal failure within 15 days after starting foscarnet therapy for cytomegalovirus infection. A kidney biopsy specimen showed the presence of crystals in all glomeruli and in proximal tubules. Fourier transform infrared microscopy analysis demonstrated that crystals were made from several forms of foscarnet salts: mixed calcium and sodium salts, and unchanged trisodium foscarnet salts. Renal function and proteinuria spontaneously improved, and a second transplant biopsy performed 8 months after the first one revealed fibrotic organization of half of the glomeruli and of interstitial tissue, and crystal vanishing. We were thus able to provide proof of the possible precipitation of foscarnet in a transplanted kidney. ( info)

20/1179. Successful treatment of adult-onset Henoch-Schonlein purpura nephritis with high-dose immunoglobulins.

    A 26-year-old woman was admitted for the evaluation of edema and massive proteinuria. She had a history of purpura of the lower extremities, abdominal pain and melena. Laboratory investigations showed hypoalbuminemia, hypercholesterolemia and proteinuria of over 10 g/day. Renal biopsy showed moderate proliferative glomerulonephritis with mesangial immunoglobulin a (IgA) deposition. She was diagnosed as having Henoch-Schonlein purpura nephritis. Oral prednisolone, dipyridamole and intravenous heparin treatment were not effective. Steroid pulse therapy induced a partial improvement of proteinuria to 2-3 g/day. High-dose intravenous immunoglobulin (i.v.-IG) treatment was introduced and a dramatic improvement of proteinuria was noted. I.v.-IG should be fully considered in patients with steroid-resistant Henoch-Schonlein purpura nephritis. ( info)
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