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E Imbasciati

Publications and source records attributed to E Imbasciati.

At least 19 recordsLinked to original sources

Collagen type III glomerulopathy: a new idiopathic glomerular disease.

A new type of idiopathic glomerular disease is reported in a 49-year-old Italian woman who presented with uncharacteristic renal symptoms, i.e., hypertension and slight proteinuria. Clinical investigation excluded a familial renal disease and more specifically nail-patella syndrome. Diagnostic renal biopsy by light microscopy showed a picture similar to membranoproliferative glomerulonephritis. The enlarged glomeruli were lobulated, the peripheral basement membranes were thickened by the deposition of light-microscopically undefined material, cell proliferation was lacking. By electron microscopy, the material was nonhomogenous, partly granular partly fibrillar, containing typical collagen fibers. The latter were identified as collagen type III, to a lesser extent collagen type I. Review of the literature resulted in 12 similar or identical cases reported from Japan and one additional case reported in a white American female. Evidence of systemic disease is lacking. Etiology and pathogenesis are elusive. A progressive deterioration of renal function must be expected. Collagen type III glomerulopathy is suggested as term of this new type of idiopathic glomerular disease.

Biopsy

Short-term effects of low-calcium dialysis solutions on calcium mass transfer, ionized calcium, and parathyroid hormone in CAPD patients.

Patients on CAPD using calcium carbonate (CaCO3) as phosphate binder might benefit from low-calcium (Ca) concentration dialysis solutions; however, no data are available for the effects of this regimen on Ca metabolism. We studied 10 patients on stable CAPD regimens with standard dialysis solutions (Ca 7 mg/dL) who were taking CaCO3 to control hyperphosphatemia (mean daily doses 4.5 +/- 2.4 g). Hypercalcemic episodes had been recorded in 6 patients. Standard dialysis solutions were replaced with solutions containing 5 mg/dL of Ca. Calcium and phosphate peritoneal mass transfer (MT), serum concentrations of total Ca, ionized Ca (Ca++), phosphate, intact PTH, and mid-molecular PTH, were evaluated before and 48 hours after change of dialysate. The switch to low-Ca solutions was accompanied by significant changes in calcium mass transfer (Ca MT) (+9.84 +/- 48.22 versus -96.74 +/- 48.32 mg/day, p less than .001). Ca MT was significantly (p less than .05) correlated with the serum/dialysate Ca gradient. There was no difference in phosphate MT. Serum Ca++ significantly (p less than .05) decreased from 5.20 +/- 0.32 to 4.88 +/- 0.36 mg/dL, and intact PTH significantly increased (81.5 +/- 139 versus 112.4 +/- 168 pg/mL, p less than .05). It is concluded that dialysis solutions with Ca 5 mg/dL result in a negative peritoneal Ca MT and can be useful to prevent and treat hypercalcemia in CAPD patients taking CaCO3 as phosphate binder. A careful monitoring of ionized calcium, PTH, and phosphate is suggested when an extensive and long-term use of this solution is considered.

Adult

A randomized trial of methylprednisolone and chlorambucil in idiopathic membranous nephropathy.

We conducted a controlled trial to investigate the long-term effects of treatment with methylprednisolone and chlorambucil in patients with idiopathic membranous nephropathy. We have previously reported that after a mean of 31 months, treated patients did better. We now report the results of a longer follow-up. Eighty-one patients with proteinuria (greater than or equal to 3.5 g per day) and biopsy-proved membranous nephropathy were randomly assigned to receive either supportive therapy alone or a six-month course of corticosteroids alternated with chlorambucil (0.2 mg per kilogram of body weight per day) every other month. Methylprednisolone was first given intravenously in three pulses (1 g per day) and was then given orally (0.4 mg per kilogram per day) for 27 days. The patients were followed for 2 to 11 years (median, 5). Two patients in the control group and one in the treatment group died. At the last follow-up visit, 9 of 39 patients assigned to the control group (23 percent) and 28 of 42 patients assigned to the treatment group (67 percent) did not have the nephrotic syndrome. At five years there were more remissions of the nephrotic syndrome in treated patients than in controls (22 of 30 vs. 10 of 25; P = 0.026). Compared with base-line values, the mean reciprocal of the plasma creatinine level declined significantly in the control group (33 percent; P = 0.0002) but not in the treatment group (6 percent; P not significant). Plasma creatinine increased by 50 percent or more in 19 controls (49 percent) and in 4 treated patients (10 percent). We conclude that a six-month course of methylprednisolone and chlorambucil can bring about sustained remission of the nephrotic syndrome and help to preserve renal function in patients with idiopathic membranous nephropathy.

Administration, Oral

Beta-2 microglobulin in patients on peritoneal dialysis and hemodialysis.

Serum beta 2 microglobulin (beta 2 mu) levels were determined in 62 patients on chronic dialysis, divided according to the type of dialysis--cuprophane hemodialysis, chronic ambulatory peritoneal dialysis (CAPD), or CAPD started after 76 +/- 47 months on cuprophane hemodialysis--and to residual urine output greater than 400 mL/day or less than 10 mL/day. In addition, for patients on CAPD, peritoneal excretion, peritoneal clearance, and urinary excretion of the protein were determined. In anuric patients serum beta 2 mu levels were significantly higher in HD than in CAPD. In patients with residual urine output, serum concentrations of the microprotein were similar in HD and in CAPD. Significant differences were observed in beta 2 mu serum levels and peritoneal clearances in patients switched to CAPD from hemodialysis as compared to those starting with CAPD. Peritoneal clearances of the microprotein was slightly and non-significantly greater in patients with urine output than in anuric patients.

Amyloidosis

Long term follow up of IgA mesangial deposits glomerulonephritis.

The clinical course of IgA Mesangial Deposits Glomerulonephritis (MDGN) has been investigated in 178 patients for 1 to 32 years (mean 6 years) from the onset of symptoms. Impairment of renal function occurred in 28 patients, 13 of whom required RDT or died in uraemia. Hypertension was observed in 67 patients. The actuarial survival rate at ten years was 91%. A significant correlation was observed between the occurrence of renal failure and the following features: absence of episodes of gross haematuria, early appearance of hypertension, marked proteinuria and sclerosing glomerular lesions. These data suggest that IgA MDGN has generally a very prolonged course, but in a few cases may evolve, sometimes early, to chronic renal failure.

Adolescent

Late recovery of renal function in a woman with the hemolytic uremic syndrome.

A case is reported of the hemolytic uremic syndrome (HUS) in a woman taking oral contraceptives. She was treated with heparin, dipyridamole and hemodialysis; and after more than three months, her urinary output rose above 500 ml; and six months after the onset of anuria, dialysis treatment was stopped. This case emphasizes the possibility that HUS in adults is not invariably irreversible and that, despite prolonged oliguria, recovery of renal function can be obtained. Therefore, in adult patients affected by HUS, dialysis should not be discontinued prematurely; moreover, bilateral nephrectomy, for treatment of severe hypertension and microangiopathic hemolytic anemia, should be performed with caution.

Adult

Human platelet aggregation by mixed cryoglobulins.

Glomerulonephritis in idiopatic mixed cryoglobulinemia represents perhaps a glomerular damage by immune complexes. In this study, a sigmoidal-like curve was obtained after addition of 13 different mixed cryoglobulins to both autologous and isologous platelet-rich plasma, tested in platelet aggregometer. The lag phase of the curve corresponds to platelet phagocytosis of cryoglobulin-binding ferritin, as shown in electron microscopy and the optical density decrease phase corresponds to the aggregation of platelets that shows the same ultrastructural characteristics of ADP-induced platelet aggregation. This platelet aggregation is inhibited by different drugs. Intraglomerular platelet aggregation by cryoglobulins might play a key role in determining the glomerular damage in cryoglobulinemia by the release of nucleotides and vasoactive amines.

Blood Platelets

Reversible acute anuric lupus nephritis.

Six cases of acute anuric lupus nephritis are described; renal biopsy showed intracapillary proliferation, epithelial crescents in some glomeruli, wire loops, and intracapillary thrombi. In two patients there were thrombotic occlusions and fibrinoid necrosis of arterioles. The patients were treated by heparin at anticoagulant doses, corticosteroids and dialysis (four). All patients showed improvement of renal function. One died following a gastric haemorrhage - the others were alive and well 64, 61, 40, 40 and 22 months from the onset of renal failure. One patient had a second episode of acute renal failure after 39 months and improved after resumption of heparin and high doses of steroids. It is suggested that heparin and high doses of corticosteroids may be a successful treatment in acute anuric lupus nephritis.

Acute Disease

[Alport's syndrome].

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Adolescent