The role of renal biopsy in the diagnosis of Fabry's disease.
Explore the source record for details and available documents.
Biomedical subjects
Publications and source records attributed to G M Frascà.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
Explore the source record for details and available documents.
This study reports on 9 patients suffering from Wegener's granulomatosis (WG) with crescentic GN and severe systemic manifestations. On admission the mean serum creatinine was 10.9 +/- 5.1 mg/dl (4-20 mg/dl); 8 patients were oliguric and required dialysis treatment. Renal biopsy showed crescents in all cases, involving 66 to 100% of glomeruli. Patients were treated with a protocol including: a plasma exchange (PE) course; methylprednisolone; cyclophosphamide; and an antithrombotic agent (defibrotide). Clinical picture and renal function progressively improved in all patients within the first 4 weeks of treatment. After 1 month serum creatinine was 2.7 +/- 0.8 mg/dl and dialysis was no longer needed in any patient. Five relapses occurred in 3 patients 12-26 months after the onset of the disease, while they were still receiving immunosuppressive treatment. At follow-up (22 to 112 months: mean 71) all patients were alive with no clinical signs of disease activity. One patient was on regular dialysis while the others had a serum creatinine of 1.2-2.8 mg/dl (mean 1.9). Our results confirm that crescentic GN associated with WG can be successfully treated even when associated with severe clinical picture and suggest that PE can contribute to control the disease without increasing immunosuppression.
This study reports on 12 patients with acute renal failure due to biopsy-proven rapidly progressive glomerulonephritis and signs of systemic disease in whom antineutrophil cytoplasmic autoantibodies (ANCA) were detected by indirect immunofluorescence (IIF) on alcohol-fixed neutrophils and assessed in serial determinations by ELISA. The diagnosis was: Wegener's granulomatosis in nine patients who showed a diffuse cytoplasmic pattern at IIF (c-ANCA), and microscopic polyarteritis in three where a perinuclear pattern (p-ANCA) was seen. All patients underwent a course of plasma exchange - PE - (3-10 sessions per patient) associated with steroids and cyclophosphamide. The ANCA titer dropped steeply during PE in all cases and was followed by disappearance of systemic symptoms and renal function improvement within four weeks. After a follow-up period of 50 +/- 31.2 months all patients were alive without signs of disease activity; ten had stable renal function, with serum creatinine 1.8 +/- 0.7 mg/dl; two had entered regular dialysis treatment after 44 and 82 months. Our results suggest that the rapid removal of ANCA by means of PE can help control disease activity and reduce the risk of death or end-stage renal disease.
Explore the source record for details and available documents.
Plasma exchange has been used in our renal transplantation programme for over ten years to treat 86 patients divided into four groups. Five patients had preformed cytotoxic antibodies before transplantation (group A); 13 sensitized patients (greater than 60% PRA) underwent prophylactic plasma exchange in the immediate post-operative period (group B); 62 patients were treated for acute vascular rejection (group C); six patients had chronic graft rejection (group D). Plasma exchange is a valid tool for the treatment of acute vascular rejection, provided that it is started before irreversible graft damage occurs: 75% rejection crises were reversed by plasma exchange and the actuarial graft survival from the rejection episode was 75% at one year, 66% at two and 50% at five years. Serum creatinine before treatment and glomerular thrombosis at graft biopsy correlated with the response to plasma exchange. In sensitized patients and in those with chronic rejection the results were disappointing and suggest that in these clinical conditions plasma exchange should be used only in selected cases.
Recurrent glomerulonephritis and de novo glomerulonephritis may develop in the graft after renal transplantation. Among 59 patients with a pathological diagnosis of glomerulonephritis as original renal disease, 12 (20.3%) showed recurrence of the original lesions in the graft. Two patients with hereditary nephritis developed anti-GBM disease (one patients in two grafts). The disease rapidly progressed to graft loss. A de novo membranous nephropathy was diagnosed in four patients whose original renal disease was not a glomerulonephritis. One patient had been treated with antilymphocyte globulin, another with captopril.
Explore the source record for details and available documents.
To investigate the possibility of slowing down disease progression 27 patients with primary glomerular diseases unresponsive to steroids and cytotoxic drugs were treated with Defibrotide. This drug is a single stranded DNA fraction which has profibrinolytic and deaggregating properties and can promote the generation and release of prostacyclin from vascular tissue. Before treatment all patients showed proteinuria in excess of 1 g/day and 16 had a nephrotic syndrome (59%); 10 patients had serum creatinine above 1.6 mg/dl (37%) and 6 were hypertensive. After therapy a significant decrease in daily proteinuria was observed, although the reduction exceeded 50% of pre-treatment values in only 16 patients (59%). A progressive decrease in serum creatinine occurred in patients with abnormal renal function; serial measurement of renal plasma flow showed a progressive improvement with an average increase of 6 and 12%, after 1 and 3 months of treatment, respectively. These observations confirm the view that drugs improving endothelial function and renal hemodynamics can be of value in the treatment of chronic glomerular diseases and can contribute to the maintenance of renal function.
Explore the source record for details and available documents.
This study analyses 75 membrane plasma exchanges carried out in 18 patients where various amounts of heparin were used to define the heparin kinetic during plasma exchange and the appropriate anticoagulation. A specific assay was employed to measure heparin concentration. Our results showed that: 1) the heparin distribution volume exceeded the expected value by 10 to 25%; 2) the drug is filtered with a sieving coefficient = 1; 3) the appropriate concentration range is within 0.2 and 0.5 Ul/ml.; 4) the heparin blood levels strictly correlate with a PTT (p less than 0.001); 5) the individual need for heparin is related to the patient Hct (p less than 0.001) and plasma flow (p less than 0.001). Simple guidelines are provided to determine the appropriate heparin dosage in single patients.
Explore the source record for details and available documents.
The present study reports on the effects of plasmapheresis on both cellular (E-rosette receptor- and Fc receptor-bearing lymphocytes) and humoral (antilymphocyte antibodies) immunity in patients with active SLE. Ten untreated patients were studied before and after two plasma exchanges. The E-rosette receptor-positive cells were studied by OKT11 monoclonal antibody and Fc receptor-bearing cells by Leu15 monoclonal antibody. Antilymphocyte antibodies were studied before and after plasma exchange by an indirect immunofluorescence technique on autologous and normal lymphocytes. A reduction in OKT11+ and Leu15+ lymphocytes was observed in all patients before plasmapheresis. Antilymphocyte antibodies were detected in five patients. After plasma exchange, an increase in E-rosette receptor- and Fc receptor-bearing lymphocytes was observed along with a reduction in the amount of antilymphocyte antibodies.
In transplanted patients graft rejection is the most frequent complication in the first year after surgery. Vascular lesions (necrosis, intimal proliferation, thrombosis) are signs of poor prognosis and lead to irreversible loss of renal function and graft removal in most cases. The problem of vascular rejection is still not solved and the results of therapy unsatisfactory, both because of inadequacy of diagnosis and/or inadequacy of available therapy. The role of prevention, very likely the best approach, is still sub judice. In an attempt to explore the validity of prevention, 22 transplanted patients (group A) were given a new antithrombotic agent (defibrotide) immediately after surgery, and the results were compared with those of a well-matched group of 30 patients on dipyridamole (group B). Follow-up lasted 6-19 months (mean 9.9) for group A; 5-21 months (mean 12) for group B. In group A, 8 patients (36%) had rejection episodes. Antirejection therapy was followed by recovery of renal function in all cases. In group B, 9 patients (29%) had rejection crises and graft removal was necessary in 7 instances due to severe vascular lesions. At the end of follow-up, all patients treated with defibrotide had normally functioning grafts: among the 30 patients on dipyridamole, 22 (73%) had satisfactory graft function.