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Biomedical subjects

S Feriozzi

Publications and source records attributed to S Feriozzi.

At least 19 recordsLinked to original sources
PubMed2006

Enzyme replacement therapy and renal function in 201 patients with Fabry disease.

AIM: Fabry disease is a rare lysosomal storage disorder caused by deficient activity of alpha-galactosidase A, resulting in progressive cellular accumulation of glycolipids, which may ultimately result in endstage renal disease. We examined the effects of enzyme replacement therapy (ERT) with Agalsidase-alpha on renal function using data from a large international database, the Fabry Outcome Survey (FOS). METHODS: This analysis was based on 1,040 serum creatinine measurements in 201 patients with Fabry disease, aged 20 - 60 years, with serum creatinine concentrations of less than 2 mg/dl and duration of ERT of up to 4.7 years. Both pretreatment and treatment data were used to examine independent predictors of changes in serum creatinine. In a second approach longitudinal serum creatinine measurements from 1 year before treatment, at baseline and 1 and 2 years after the start of treatment were analyzed in 20 patients with chronic kidney disease (CKD) Stage 2 and 3. RESULTS: We found an independent negative association between serum creatinine and time on Agalsidase-alpha treatment (p < 0.05). Renal function declined significantly (p < 0.05) in the year before treatment. After 1 year of treatment, however, the decline in estimated glomerular filtration rate had been halted, and renal function was preserved for up to 2 years. CONCLUSIONS: In conclusion, ERT with Agalsidase-alpha is associated with decrease of serum creatinine and may prevent the deterioration of renal function in patients with Fabry disease.

Adult↗
PubMed2000

Cephotaxime-associated allergic interstitial nephritis and MPO-ANCA positive vasculitis.

We report a case of reversible acute renal failure after cefotaxime treatment in a patient affected by non-Hodgkin lymphoma. Renal biopsy showed necrotizing vasculitis associated with eosinophil-rich interstitial inflammatory infiltrates and patchy infiltrates of CD20+ lymphoid cells. High serum p-ANCA titers were also detected. Drug withdrawal was closely related with recovery of renal function and disappearance of ANCA. Acute renal failure therefore represented a consequence of ANCA-mediated renal vasculitis and acute interstitial nephritis related to cefotaxime treatment.

Aged↗
PubMed1999

Spatial arrangement of subepithelial deposits in lupus and nonlupus membranous nephropathy.

Subepithelial deposits are a common feature of idiopathic membranous glomerulonephritis (MGN) and lupus membranous glomerulopathy (LMGN). We investigated the spatial arrangement of immunoglobulin G (IgG) and C3c fraction of complement (C3c) in the immune deposits of MGN and LMGN with confocal laser scanning microscopy to correlate specific patterns of IgG-C3 interactions with different diseases. Ten patients with MGN and 8 patients with LMGN (World Health Organization class VB) were selected. A determination of the spatial arrangement of the two fluorochromes and the glomerular area occupied by each fluorochrome was performed for each case. Our results showed MGN specimens have an orderly distribution of IgG and C3c, with each deposit showing an outer ring of sole IgG. IgG was always more abundant than C3c (1,619 +/- 271 v 790 +/- 105 micrometer(2), P = 0.002). In LMGN, IgG and C3c were haphazardly arranged, with deposits made of C3c only and an outer ring of IgG only rarely present. Also, the relative amounts of the two antigens were variable, and two groups could be identified (group 1: IgG, 5,515 +/- 1,179 micrometer(2) v C3c, 4,810 +/- 1,174 micrometer(2); P = 0.02; group 2: IgG, 3,358 +/- 658 micrometer(2) v C3c, 4,047 +/- 740 micrometer(2); P = 0.03). Our data show that diffuse IgG capping of the subepithelial immune deposits is diagnostic of MGN. The absence of an orderly three-dimensional arrangement in LMGN deposits (ie, outer ring of IgG) is likely to render active complement components more readily available to inflammatory activities.

Adult↗
PubMed1998

Expression of TGF-beta receptors type I and II in human glomerulonephritis.

BACKGROUND: Transforming growth factor-beta (TGF-beta) is a multipotent cytokine involved in the turnover of the extracellular matrix. Signal transduction of TGF-beta is regulated via at least five different surface receptors; most of the effects, however, are mediated through the interaction of receptors type I and II (RI and RII). We investigated the glomerular expression of TGF-beta and its receptors RI and RII in human glomerulonephritis. METHODS: Indirect immunofluorescence using monoclonal and polyclonal antibodies against TGF-beta isoforms (1,2,3 and 2,3), TGF beta-RI and TGF beta-RII has been carried out on 30 consecutive renal biopsies (5 FSGS, 11 IgAN, 4 MCGN, 6 SLE, 2 RPGN) and on normal kidney tissue. RESULTS: Glomerular immunoreactivity for TGF-beta isoforms correlated with the severity of proliferative lesions: immunofluorescent signal was absent or trace-like in normal kidneys, FSGS, and IgAN with mild mesangial cellularity, and was highest in IgAN with severe mesangial proliferation, MPGN, RPGN, and SLE. Glomerular positivity for TGF-beta-RI and -RII was detectable in SLE and RPGN; a low signal was present also in MPGN. Other types of glomerulonephritis, including focal extracapillary proliferations, and glomeruli of normal kidneys were always negative. CONCLUSIONS: Our data show that TGF-beta expression is a good indicator of the severity of proliferative glomerular lesions in most, if not all cases and that RI-RII expression occurs at levels detectable with indirect immunofluorescence in limited number of glomerulonephritides, mostly associated with systemic diseases. A complex interaction between TGF-beta and its ligand may represent a critical factor conditioning the tissue response to immunological injury.

Fluorescent Antibody Technique, Indirect↗
PubMed1998

Impact of recombinant human erythropoietin treatment on left ventricular hypertrophy and cardiac function in dialysis patients.

The results of anemia correction by recombinant human erythropoietin (rHuEPO) therapy with regard to cardiac function and left ventricular hypertrophy in dialysis patients are controversially discussed. The aim of the study was to assess the effects of therapy rHuEPO on cardiac morphology and function in dialysis patients. We studied 11 clinically stable hemodialysis patients with severe renal anemia (hematocrit <27%) and increased left ventricular mass index (LVMi) with no history of coronary or valvular heart disease, systemic disease, severe hyperparathyroidism, hypertension stage 2 or higher, transfusion-dependent anemia, and concurrent rHuEPO treatment. The patients were treated with rHuEPO administered subcutaneously once or twice weekly at a mean dose of 80 +/- 31 IU/kg week until the hematocrit was >30% and underwent a complete Doppler echocardiographic study at baseline and at follow-up (after 12.2 +/- 2.9 months). At follow-up, ejection fraction and fractional shortening significantly increased from 62.7 +/- 13.8 to 67.8 +/- 9. 7% (p < 0.05) and from 35.5 +/- 9.8 to 39.4 +/- 7.1% (p < 0.05), respectively, whereas mean velocity of circumferential fiber shortening demonstrated a trend towards amelioration from 1.18 +/- 0. 23 to 1.27 +/- 0.27 circ/s (n.s.). LVMi and morphological data remained unchanged throughout the study. Nevertheless, LVMi changes showed two different behaviors with respect to baseline values: in 6 patients with higher baseline values, LVMi decreased from 229 +/- 36 to 191 +/- 45 g/m2 (p < 0.05), while it worsened in 5 patients with less marked LVMi, increasing from 141 +/- 32 to 186 +/- 40 g/m2 (p < 0.05). Our data demonstrate that partial correction of renal anemia with rHuEPO therapy seems to improve cardiac performance and to induce a regression of left ventricular hypertrophy, particularly in patients with greater baseline hypertrophy, ultimately confirming the multifactorial pathogenesis of left ventricular hypertrophy.

Adult↗
PubMed1997

Systemic lupus erythematosus with membranous glomerulonephritis and uterine vasculitis.

We report a case of lupus vasculitis with uterine localization and concurrent membranous nephropathy. Immunofluorescence study suggested the occurrence of an immune complex nephropathy and a pauci-immune pathogenesis of vasculitis. Our case points out the event of tissue damage in two organs mediated by different pathogenetic mechanisms. In addition, uterine vasculitis without pregnancy may be observed in patients with systemic lupus erythematosus nephritis.

Cervix Uteri↗
PubMed1995

Spatial arrangement of IgA and C3 as a prognostic indicator of IgA nephropathy.

The histological picture of primary glomerulonephritis with glomerular IgA deposition (IgA nephropathy and Henoch-Schönlein disease) can vary from minimal mesangial involvement to severe endocapillary and/or extracapillary proliferation. Local activation of the complement cascade by glomerular IgA deposits and release of anaphylactoid factor are considered to be major triggers of inflammation, but clear-cut correlations between the severity of the histological findings and the intensity of glomerular deposition of immunoglobulin and complement fractions are still lacking. The purpose of this study was to investigate the spatial distribution of IgA and complement in mesangial deposits with confocal laser scanning microscopy (CLSM) and to correlate specific patterns of IgA-complement interaction with glomerular damage. Two groups of patients have been studied, one with mild to moderate diffuse mesangial proliferation and the other with diffuse proliferative endocapillary and/or extracapillary patterns. In milder forms of the disease, the majority of the immune deposits are composed of both IgA and C3, coated by an outer layer of IgA alone. Large C3 deposits, or deposits composed of IgA and C3 without an outer coat of IgA, were associated with more severe histological lesions. The results suggest that free access of active complement components to cell and/or mesangial matrix receptors could trigger a cytolytic reaction and that immunoglobulins seem to act as a protective layer on C3 components.

Adolescent↗
PubMed1995

Role of endothelial cells in the development of glomerular lesions of mesangiocapillary glomerulonephritis.

The histological and ultrastructural changes of mesangiocapillary glomerulonephritis (MCGN) are not unique to this entity: splitting of basement membrane (BM) is seen in a number of conditions with an altered coagulation pattern. The distribution of endothelial cells in the glomerular capillaries in five cases of MCGN was studied by light and electron microscopy and immunocytochemistry; endothelial cells were stained with peroxidase or FITC-conjugated antibodies against Factor VIII-related antigen or CD34 antigen and observed with conventional light/immunofluorescence microscopy and confocal laser scanning microscopy for three-dimensional reconstruction. Electron microscopy was performed with colloidal gold labelling. Endothelial cells were shown to be present within the duplicated basement membranes of capillaries and continuity was demonstrated with endothelial cells lining the capillary lumina. These results suggest that endothelial cells as well as mesangial cells can participate in the formation of the characteristic double contour of capillary walls in MCGN, especially in the early stages. There are some similarities to the changes seen in larger arteries as a consequence of thrombotic disease.

Adult↗
PubMed1994

Glomerular hypertrophy and chronic renal failure in focal segmental glomerulosclerosis.

Focal segmental glomerulosclerosis evolves toward chronic renal failure (CRF) with a highly variable rate; in particular, a group of patients with a much more rapid decline of renal function has been described. The purpose of this study was to evaluate the usefulness of morphometry in identifying those cases with a faster decline in renal function. Two groups of patients have been studied: six with rapid evolution toward CRF (group 1) and six without reduction in renal function during a follow-up of up to 10 years (group 2). The results of the morphometric analysis of glomeruli were as follows: mean glomerular area: 30,550 +/- 5,259 microns2 (group 1) versus 22,667 +/- 5,078 microns2 (group 2) (P = 0.01); maximum glomerular area: 40,827 +/- 9,508 microns2 (group 1) versus 30,445 +/- 7,224 microns2 (group 2) (P = 0.02); mean glomerular diameter: 193.9 +/- 15.8 microns (group 1) versus 161.8 +/- 16.8 microns (group 2) (P = 0.003); and caliper diameter: 328.6 +/- 20.6 microns (group 1) versus 260.6 +/- 36 microns (group 2) (P = 0.001). Values of body surface area were not different between the two groups (1.85 +/- 0.34 m2 v 1.6 +/- 0.13 m2) (P = NS). Our results suggest that glomerular hypertrophy in the course of focal segmental glomerulosclerosis is correlated with a faster decline toward CRF. It might represent a compensatory hypertrophy that would immediately precede a rapid decline in renal function or it might be the expression of the preexisting condition (meganephronia), which predisposes to the development of CRF.

Adult↗
PubMed1989

[Nicardipine, alone or in combination with enalapril, in the therapy of arterial hypertension secondary to chronic nephropathy].

The aim of the study was to evaluate the long term antihypertensive effect of nicardipine in hypertensive patients with chronic renal disease. Eight patients (creatinine clearance ranging from 51 to 78 ml/min/1.73 m2) received nicardipine (20 mg t.i.d.). Four weeks later, patients with diastolic blood pressure greater than or equal to 90 mmHg in recumbent position, were given enalapril (10 mg/day) as well. Blood pressure control was achieved in 3 patients treated with nicardipine alone and in 5 patients on a combined nicardipine-enalapril regimen, and it was maintained throughout the whole trial period (52 weeks). In two cases serum creatinine rose from 2.3 to 3.3 and from 1.4 to 2.2 respectively. However, the slope of the creatinine ratio, plotted against time, showed a significant reduction in renal function loss as compared to expected values. In conclusion, nicardipine, alone or in combination with enalapril, is an effective and well tolerated drug for use in treatment of hypertension secondary to chronic renal disease.

Adult↗
PubMed1989

Improvement of renal function with selective thromboxane antagonism in lupus nephritis.

To test the hypothesis that the vasoconstrictor thromboxane A2 may affect renal hemodynamics in lupus nephritis, we examined the short-term effects of a selective thromboxane-receptor antagonist, BM 13,177, and of low-dose aspirin. In a randomized, double-blind, crossover study, 10 patients with biopsy-proved lupus nephritis were given a 48-hour continuous infusion of BM 13,177 or placebo. At base line, seven patients had markedly elevated urinary levels of thromboxane B2, the breakdown product of thromboxane A2. During the infusion of BM 13,177, the inulin clearance rate, which was 68 ml per minute per 1.73 m2 of body-surface area at base line, increased by an average of 24 percent (range, 12 to 47 percent; P less than 0.01). Para-aminohippurate clearance was increased to the same extent, with no change in the filtration fraction. The bleeding time doubled, indicating an occupancy of platelet thromboxane receptors of more than 95 percent. The hemodynamic changes were associated with a significant increase in sodium excretion from 76 to 118 mmol per day (P less than 0.01) but with no change in arterial blood pressure. In another study, 10 additional patients with lupus nephritis were randomly assigned to receive either placebo or 20 mg of aspirin twice daily for four weeks. The aspirin regimen produced a selective, cumulative inhibition of platelet cyclooxygenase activity and a doubling of bleeding time. However, there was no change in the inulin clearance rate and no change in urinary levels of thromboxane B2 or 6-keto-prostaglandin F1 alpha, which are indicators of renal synthesis of thromboxane A2 and prostacyclin, respectively. We conclude that in lupus nephritis, impairment of renal function is at least in part mediated hemodynamically and is reversible with a thromboxane antagonist. Platelets, however, are not a major source of thromboxane A2 synthesis and action within the kidney.

Adolescent↗
PubMed1989

Angiotensin converting enzyme inhibition delays the progression of chronic renal failure in hypertensive patients with immunoglobulin A nephropathy.

Ten hypertensive, non-nephrotic patients with immunoglobulin (IgA) nephropathy treated with conventional therapy (vasodilators, beta-blockers, diuretics) were observed for 21.4 +/- 9 months. Treatment was then changed to an angiotensin converting enzyme (ACE) inhibitor alone for an additional 22.6 +/- 9 months. The ACE inhibitor therapy was associated with a lower diastolic blood pressure and a slowing in the rate of decline in renal function, but no change in proteinuria.

Adult↗
PubMed1983

Plasma renin activity: effect of temperature during blood processing.

Plasma renin activity may be affected by temperature during the processing of blood samples (loss of activity or cryoactivation). We measured plasma renin activity on samples maintained at room temperature up to 240 min. No significant change was found in comparison with samples maintained at 0 degrees C; similarly, no cryoactivation was observed when routine freezing procedures were employed instead of instant freezing.

Blood Specimen Collection↗