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Biomedical subjects

Gabriella Moroni

Publications and source records attributed to Gabriella Moroni.

17 recordsLinked to original sources

Long-term outcome of idiopathic retroperitoneal fibrosis treated with surgical and/or medical approaches.

BACKGROUND: Retroperitoneal fibrosis is a severe disease that affects the ureters, causing renal insufficiency in three-quarters of patients. The optimal treatment is far from being established. METHODS: Seventeen patients with idiopathic retroperitoneal fibrosis and ureteral entrapment followed in our unit for at least 1 year were selected for this study. At presentation 13 patients had renal insufficiency. All patients received steroids, associated with ureterolysis in five (group 1), with azathioprine in six (group 2) and with tamoxifen in six (group 3). Four patients of group 2 and five of group 3 received ureteral stenting or nephrostomy. There were no significant differences among the three groups or the clinical and biochemical characteristics at presentation. RESULTS: All patients of groups 1 and 2 entered remission after therapy. One patient from group 3 did not respond to therapy. During a mean follow-up of 56 +/- 41 months, three patients (two from group 1, one from group 2, 18%) had a recurrence of the disease, which fully responded to retreatment in all three cases. At the last observation, all patients were alive; three patients (18%) had renal insufficiency, of them one from group 1 had to start dialysis 6 years after ureterolysis, one patient from group 2 and one from group 3 had serum creatinine of 1.5 mg/dl. Renal survival was 100% at 5 years and 80% at 10 years. CONCLUSIONS: In most patients, each of the three different therapeutic approaches restored renal function and significantly reduced the fibrotic mass in the short-term and maintained stable serum creatinine in the long-term.

Adult↗

Chronic periaortitis and HLA-DRB1*03: another clue to an autoimmune origin.

OBJECTIVE: Patients with chronic periaortitis (CP) often show clinical and laboratory findings of a systemic autoimmune disorder. The aim of the present study was to investigate the role of the HLA system in CP. METHODS: Low-resolution genotyping for HLA-A, HLA-B, and HLA-DRB1 loci and genotyping of TNFA(-238)A/G and TNFA(-308)A/G single nucleotide polymorphisms were performed in 35 consecutive patients with CP and 350 healthy controls. RESULTS: The HLA-DRB1*03 allele frequency was strikingly higher in patients with CP than in controls (24.28% versus 9.14%; chi(2) = 15.50, P = 0.000084, corrected P [P(corr)] = 0.0012, odds ratio [OR] 3.187, 95% confidence interval [95% CI] 1.74-5.83); the HLA-B*08 allele frequency was also higher in patients than in controls (17.14% versus 6.28%; chi(2)=11.12, P = 0.0008, P(corr) = 0.0269, OR 3.085, 95% CI 1.54-6.16). The A*01 allele frequency was significantly different (P = 0.0463), but the statistical significance was lost after correction for multiple testing (P(corr) = 0.5088). TNFA(-238)A allele and TNFA(-308)A allele frequencies were not significantly different (P = 0.512 and P = 0.445, respectively). Comparison of the main clinical and laboratory findings suggestive of a systemic autoimmune disease (e.g., acute-phase reactants, constitutional symptoms, other autoimmune diseases associated with CP) between the HLA-DRB1*03-positive and the HLA-DRB1*03-negative patients showed that the former group had significantly higher levels of C-reactive protein (P = 0.045) at disease onset, although this difference was not statistically significant after correction for multiple tests (P(corr) = 0.369). CONCLUSION: The HLA system plays a role in susceptibility to CP. The strong association between CP and HLA-DRB1*03, an allele linked to a wide range of autoimmune conditions, further supports the view that CP may represent a clinical manifestation of an autoimmune disease.

Adolescent↗

Withdrawal of therapy in patients with proliferative lupus nephritis: long-term follow-up.

BACKGROUND: Whether corticosteroid and immunosuppressive therapy may be safely withdrawn in patients with proliferative lupus nephritis is still unclear. METHODS: In 32 patients with biopsy-proven proliferative lupus nephritis previously put into remission, therapy was gradually tapered off. RESULTS: When immunosuppressive therapy was stopped (median: 38 months; 25th-75th percentile: 24-81 months, after biopsy), 24 patients were in complete remission and eight had a median proteinuria of 1.05 g/24 h (25th-75th percentile: 0.91-1.1 g/24 h) with normal renal function. After stopping therapy, patients were followed for a median of 203 months (25th-75th percentile: 116-230 months). Fifteen patients (Group 1) never developed lupus activity. The other 17 patients (Group 2) developed lupus exacerbations in a median of 34 months (25th-75th percentile: 29-52 months) after stopping therapy and were re-treated. The only significant differences between the two groups were the longer median durations of treatment, 57 months (25th-75th percentile: 41.5-113.5 months) vs 30 months (25th-75th percentile: 18-41 months; P<0.009), and remission, 24 months (25th-75th percentile: 18-41) vs 12 months (25th-75th percentile: 7-20 months; P<0.02), before stopping therapy in Group 1 than in Group 2. At last follow-up, 12 patients of Group 1 were in complete remission, two had mild proteinuria and one had died. In Group 2, one patient died, 14 were in complete remission, one had mild proteinuria and in another patient serum creatinine doubled. CONCLUSIONS: Some patients with severe lupus nephritis who enter stable remission can be maintained without any specific treatment for many years. Those patients who have new flares can again go into remission with an appropriate treatment. The longer the treatment and remission before withdrawal, the lower the risk of relapse.

Adrenal Cortex Hormones↗

Efficacy prospective study of different frequencies of Epo administration by i.v. and s.c. routes in renal replacement therapy patients.

BACKGROUND: The problem of pure red cell aplasia (PRCA) prompted nephrologists to revert to a wider intravenous (i.v.) utilization of erythropoeitin (Epo). Once weekly i.v. Epo administration has been suggested to be as effective as the twice/thrice weekly i.v. dose. The aim of the present study was to test whether once weekly i.v. Epo administration is equally as cost-effective as once weekly subcutaneous (s.c.) and 2-3 times weekly i.v. administration. METHODS: We prospectively studied 41 patients (23 males, aged 28-82 years), on renal replacement therapy for 18-286 months, stabilized on twice or thrice weekly s.c. Epo-alpha (basal). The patients were treated for three consecutive 6 month periods with once weekly s.c. (OWSC), once weekly i.v. (OWIV) and twice/thrice weekly i.v. (TWIV) Epo-alpha. The initial dose for each period was equal to the final dose of the previous one; when necessary, the dose was adjusted according to DOQY guidelines. Iron, folic acid and vitamin B(12) supplementations were given throughout all the study periods. At the end of each of the four study periods, the following parameters were evaluated: haemoglobin, haematocrit, hypochromic red blood cells (RBCs), iron, serum ferritin, transferrin, folate, vitamin B(12), C-reactive protein (CRP), Kt/V, parathyroid hormone (PTH) and weekly dose of Epo-alpha. RESULTS: Thirty-three out of 41 enrolled patients completed the study (there were five deaths, two renal transplants and one transfer). No significant changes were observed as regards iron, serum ferritin, transferrin, folate, vitamin B(12), CRP, Kt/V or PTH level. Haemoglobin levels were not different at the end of the basal (11.7+/-1.21), OWSC (11.8+/-0.86) and TWIV (12.1+/-1.04) periods, while significantly lower levels were observed after the OWIV period (11.0+/-0.97, P<0.01). Weekly Epo consumption (Epo U/week/kg body weight/g haemoglobin) was: basal 11.57+/-5.96; OWSC 10.22+/-4.53; OWIV 15.99+/-7.7*(a); and TWIV 11.89+/-6.3*(a) (*P<0.01 vs basal; (a)P<0.01 vs OWSC). CONCLUSIONS: From our results, the OWIV schedule seems to have less efficacy in the control of anaemia of chronic renal failure patients on dialysis treatment than either OWSC or TWIV schedules.

Adult↗

The long-term prognosis of renal transplantation in patients with lupus nephritis.

BACKGROUND: Few data are available about the long-term outcome of renal transplantation in patients with systemic lupus erythematosus (SLE). METHODS: Between June 1982 and 2004, a total of 33 adults with lupus nephritis received 35 kidney allografts. Outcomes of these grafts and those of 70 controls matched for age, sex, and donor source who underwent transplantation during the same period were compared. RESULTS: Mean follow-up after renal transplantation was 91 +/- 59 months for patients with lupus and 90 +/- 64 months for controls. Actuarial 15-year patient (80% versus 83%) and death-censored graft survival rates (69% versus 67%) were not significantly different between patients with lupus and controls. Risks for acute and chronic rejection, arterial hypertension, and infection were not different between the 2 groups. Mean serum creatinine levels also were similar in the 2 groups at the last follow-up visit. Intravascular thrombotic events occurred in 9 patients with SLE (26%) and 6 controls (8.6%; P = 0.038). In the SLE group, 6 of 7 antiphospholipid (aPL) antibody-positive versus 3 of 17 aPL antibody-negative patients experienced thrombotic events ( P = 0.015). Recurrence of lupus nephritis was documented in 3 renal grafts (8.6%), but no graft was lost because of recurrent lupus nephritis. CONCLUSION: Long-term patient and graft survival probabilities were similar in patients with SLE and matched controls. The risk for thrombotic complications was greater in patients with SLE, particularly aPL-positive patients. Nephritis recurred in less than 10% of patients with SLE and did not influence graft survival.

Adult↗

Anti-DNA antibodies: a diagnostic and prognostic tool for systemic lupus erythematosus?

The clinical impact of anti-DNA antibodies lies on their diagnostic power for systemic lupus erythematosus (SLE), being a formal classification criterion. In spite of such a disease association, low-avidity anti-DNA antibodies might also be part of the natural autoantibody repertoire. Their switch to pathogenic high-avidity autoantibodies is the result of the autoimmune process leading to SLE.Anti-DNA antibodies were shown to play a role in SLE pathogenesis and particularly in kidney damage. Accordingly, antibody titres might fluctuate in relation to disease activity, but their prognostic value for flares is still debated.Several methods for anti-DNA detection were described and there is evidence that the assays identify different antibodies with different prognostic value. The results of a multicenter study on four different routine tests for anti-dsDNA antibody detection showed that: (i) Farr assay displays the best diagnostic specificity/sensitivity for SLE, followed by Crithidia luciliae method (CLIFT), (ii) the new generation of solid phase assay (EliA) shows an increased sensibility versus the classical enzyme linked immune assay (ELISA) but a decreased specificity. Antibody titre detected by EliA and Farr assay correlated with disease activity. These findings would suggest that more than one assay should be useful for SLE diagnosis and monitoring.

Antibodies, Antinuclear↗

Increased levels of circulating endothelial cells in chronic periaortitis as a marker of active disease.

BACKGROUND: The pathogenesis of chronic periaortitis (CP) has not been clarified. The histologic features and the association with autoimmune diseases suggest an immune-mediated disorder with marked inflammatory vascular and perivascular lesions. To clarify the role of vascular damage we looked for the presence and the surface phenotype of circulating endothelial cells (CECs) in the peripheral blood of patients with chronic periaortitis. METHODS: Eleven patients with CP were evaluated for the presence of CECs; 9 patients had active and 2 inactive disease. Three patients with active disease were also evaluated 3 months after therapy. Ten atherosclerotic patients, 10 patients with renal insufficiency of variable degree and etiology, and 40 healthy subjects were evaluated as controls. Five-parameter, 3-color flow cytometry was performed with a FACScan. CECs were defined as CD45 negative, CD31, P1H12, and CD36 positive, and activated CECs as CD45 negative and P1H12, CD62 positive. RESULTS: The median number of CECs in patients with CP (10(6) cells/mL) was significantly higher than in healthy controls (16 cells/mL, P= 0.0004) and atherosclerotic patients (25 cells/mL, P= 0.0005) Two patients with inactive disease had a CEC count comparable to that of normal subjects. In 2 of the 3 patients reevaluated, 3 months after therapy CEC numbers normalized. Almost all CECs were microvascular in origin and showed an activated phenotype. CONCLUSION: The presence of a high number of CECs in the active phase of chronic periaortitis and their normalization during inactive disease suggest that endothelial damage may play a role in the pathogenesis of the disease.

Aged↗

Differentiation of human melanoma cells induced by cyanidin-3-O-beta-glucopyranoside.

Great attention has been recently given to a flavonoid of the anthocyanin class, cyanidin-3-O-beta-glucopyranoside (C-3-G), which is widely spread throughout the plant kingdom, and is present in both fruits and vegetables of human diets. In this study, we investigated the effect of C-3-G on proliferation and differentiation of human melanoma cells. Both morphological and functional parameters were evaluated, using electron and confocal microscopy, cytofluorometric analysis, HPLC assay, Western blot analysis, and enzymatic assay, as appropriate. A treatment with a single dose of C-3-G decreased cell proliferation without affecting cell viability and without inducing apoptosis or necrosis. The mitotic index and cell percentage in S phase were significantly lower in C-3-G treated cells compared with untreated control. C-3-G treatment induced, in a dose- and time-dependent manner, melanoma cell differentiation characterized by a strong increase in dendrite outgrowth accompanied with a remodeling of the microtubular network, a dramatic increase of focal adhesion and an increased expression of "brain specific" cytoskeletal components such as NF-160 and NF-200 neurofilament proteins. C-3-G treatment also induced increase of cAMP levels and up-regulation of tyrosinase expression and activity resulting in an enhanced melanin synthesis and melanosome maturation. Up-regulation of the melanoma differentiation antigen Melan-A/MART-1 in treated cells respect to the untreated control was also recorded. Data obtained provide evidence that a single treatment with C-3-G is able to revert the human melanoma cells from the proliferating to the differentiated state. We conclude that C-3-G is a very promising molecule to include in the strategies for treatment of melanoma; also because of its nutritional relevance.

Anthocyanins↗

Acute post-bacterial glomerulonephritis in renal transplant patients: description of three cases and review of the literature.

Only a few cases of acute post-infectious glomerulonephritis have been described in renal transplant patients. We report here three cases of acute post-bacterial glomerulonephritis in renal transplants. In contrast to the classic cases of post-streptococcal glomerulonephritis the type of infection was heterogeneous: respectively, Escherichia coli bacteremia, a skin abscess, and cholangitis. The clinical presentation was characterized by a deterioration of graft function in two of our three patients. Acute renal dysfunction recovered in both patients, but in the long term the outcome was severe; two of the three patients lost their graft function. It is difficult to ascertain whether progression was due to chronic allograft nephropathy, to glomerulonephritis, or both. It may be concluded that acute post-infectious glomerulonephritis is a possible, although rare, complication in renal transplant recipients. It has an unusual presentation and may have a poor outcome in the long term. The role of therapy, if any, is still undefined.

Acute Disease↗

Antiphospholipid antibodies are associated with an increased risk for chronic renal insufficiency in patients with lupus nephritis.

BACKGROUND: Previous studies have documented the high frequency of thrombosis and fetal loss in patients with lupus nephritis and antiphospholipid (aPL) antibodies, but there is little information on the impact of aPL antibodies on the outcome of lupus nephritis. The aims of this study are to evaluate the prevalence of aPL antibodies in patients with lupus nephritis and assess their prognostic value for thrombosis and pregnancy morbidity and impact on long-term renal outcome. METHODS: One hundred eleven patients with lupus nephritis followed up for a mean of 173 +/- 100 months were tested regularly for immunoglobulin G (IgG) and IgM anticardiolipin antibodies and lupus anticoagulant. RESULTS: The overall prevalence of aPL antibodies was 26%. In follow-up, 79% of aPL antibody-positive patients experienced thrombotic events and/or fetal losses, and aPL antibodies were associated significantly with arterial or venous thrombosis (P = 0.00001), pregnancy morbidity (P = 0.045), thrombocytopenia (P = 0.0015), and persistent arterial hypertension (P = 0.028). aPL antibodies were significantly more frequent in patients with biopsy-proven membranous lupus nephritis (P = 0.01). A strong association between aPL antibodies and the development of chronic renal insufficiency in the long-term outcome also was found (P = 0.01). With multivariate analysis, aPL antibody positivity (P = 0.02), high plasma creatinine level at presentation (P = 0.01), and chronicity index (P = 0.00004) were independent predictors of chronic renal function deterioration. CONCLUSION: Detection of aPL antibodies in patients with lupus nephritis is useful not only to identify patients at risk for vascular and obstetric manifestations, but also for their potential deleterious impact on renal outcome.

Adolescent↗

[Acute post-infective glomerulonephritis].

In children post-infectious acute glomerulonephritis is usually caused by beta-hemolytic streptococcus and has a fair prognosis with spontaneous remission in most cases. Rarely, however, the disease may be complicated by oliguria and/or severe hypertension which can cause cardiac congestion or cerebral edema. In adults the acute glomerulonephritis may be caused by a number of different micro-organisms and it often develops in patients with other diseases. The prognosis may be severe and is worsening over the years. The risk of chronic renal failure is increased in patients with crescents and with large interstitial infiltrates. A renal biopsy is recommended in adults with acute glomerulonephritis to make possible a differential diagnosis with other diseases presenting with a nephritic syndrome, to assess the prognosis and to evaluate the pro's and the con's of a treatment with steroid and/or immunosuppressive agents.

Acute Disease↗

Pregnancy in lupus nephritis.

BACKGROUND: Fetal and maternal outcomes of 70 pregnancies in 48 women with lupus nephritis were retrospectively analyzed. METHODS: In 13 women, lupus nephritis developed during pregnancy (group A). In 38 patients with known lupus nephritis (including 3 patients in group A who had another pregnancy), 57 pregnancies occurred. In 6 patients, a therapeutic abortion was performed. The remaining 51 pregnancies were considered pregnancies in lupus nephritis (group B). RESULTS: Fetal loss was 36% (38%, group A; 35%, group B); it decreased from 46% in the 1970s to 30% in the last decade. Among 41 live births, there were 13 preterm deliveries and 28 full-term deliveries. At multivariate analysis, proteinuria (P = 0.025), arterial hypertension (P = 0.05), and antiphospholipid antibodies (P = 0.01) were independent predictors of fetal loss. In group A, 3 patients developed acute renal failure, irreversible in 1 patient (7.7%); all other patients recovered after steroid and immunosuppressive therapy. In group B, 12 renal flares and 1 extrarenal flare developed during pregnancy or the postpartum period. Two patients progressed to irreversible renal failure (3.9%), and 1 of the 2 patients died. All other patients recovered. The incidence of renal flares before or during pregnancy was not different (P = 0.51). Renal quiescence at the onset of pregnancy was the only predictor of favorable maternal outcome. CONCLUSION: Proteinuria, hypertension, and positivity of antiphospholipid antibodies are independent predictors of adverse fetal outcome. Quiescence of renal disease is the only predictor of favorable maternal outcome.

Abortion, Spontaneous↗

Long-term prognosis of diffuse proliferative glomerulonephritis associated with infection in adults.

BACKGROUND: Infection-associated glomerulonephritis is rare in adults and its long-term prognosis is undefined. METHODS: We retrospectively evaluated the clinical course of 50 adults (30 men, 20 women) with infection-associated glomerulonephritis diagnosed in our department from 1979 to 1999. The mean follow-up was 90+/-78 months. Patients were subdivided into two groups: group 1 included those without underlying disease and group 2 included those with severe underlying disease. RESULTS: At presentation, the median age was 54 years, and 33 patients were hypertensive, 31 had nephritic syndrome, eight had nephrotic syndrome and 11 had non-nephrotic proteinuria. Patients in group 2 were significantly older and had a significantly higher proteinuria than patients of group 1. Of the 21 patients in group 2, nine had liver cirrhosis, four cancer, five diabetes, three bronchiectasis, one thalassaemia intermedia, one polymyositis and one had anti-phospholipid antibodies syndrome. At the last follow-up, five patients had died, 21 patients were in complete remission, ten had partial remission, ten had renal insufficiency and three were on chronic dialysis. Multivariate analysis showed that an underlying disease (P=0.04) and interstitial infiltration at biopsy (P=0.036) were predictors of incomplete recovery. A correlation analysis between the year of diagnosis and the clinical/ histological characteristics at presentation showed that age (P=0.05), atypical infections (P=0.01), underlying disease (P=0.01) and interstitial infiltration at biopsy (P=0.02) increased over time, while the number of patients with complete remission significantly decreased (P=0.001). CONCLUSIONS: Infection-associated glomerulonephritis may progress to chronic renal failure in a consistent number of adult hospitalized patients, particularly in those with an underlying disease and when associated with interstitial infiltration at biopsy.

Adult↗

The risk of pregnancy in patients with lupus nephritis.

Since many women with systemic lupus erythematosus (SLE) are of child-bearing age and have normal fertility, the clinician must often face many problems relating to pregnancy in patients with lupus nephritis. These include the influence of lupus nephritis on fetal outcome, obstetric complications, and the influence of pregnancy on SLE. Studies published in the 1960s underlined the increased fetal and maternal risk and recommended against pregnancy in lupus patients. The prognosis for non-pregnant SLE active patients was also poor, making it difficult to know whether pregnancy altered the prognosis of the disease. Recent prospective studies indicate that the majority of lupus mothers can sustain pregnancy without detrimental effects, providing that the pregnancy is planned during the inactive phase of the disease. Nevertheless the fetal risk, although progressively reduced during the last 40 years, continues to be higher than in pregnancies of healthy women particularly in patients with antiphospholipid antibodies.

Embryonic and Fetal Development↗

Renal replacement therapy in lupus nephritis.

The indications and the choice of renal replacement therapy for lupus patients are similar to those for other uremic patients. However, lupus patients can pose some particular problems. First, 10-28% of patients needing dialysis can have a partial renal function recovery. Therefore, the clinician has to decide whether to administer a rescue treatment, risking side-effects, or to reduce immunosuppression precluding a potential recovery. Many patients on regular dialysis show subdued biological and clinical activity. Others can show a hectic disease activity, particularly in the 1st year. In these cases, treatment is difficult, as vigorous immunosuppression can expose uremic patients to severe side-effects. The presence of circulating antiphospholipid antibodies (aPL) can favor thrombosis or stenosis of vascular access (VA). Renal transplantation is the best therapy for most lupus patients with end-stage renal failure. Many, but not all, studies have reported similar patient and graft survival rates in lupus and in non-lupus transplant recipients. The results are much better with living donor transplantation. Patients with aPL, black patients and those on long-term dialysis have a higher graft failure risk. Candidates with active lupus and/or those with significant iatrogenic morbidity should be advised to wait 6-12 months before transplantation. The recurrence risk of lupus nephritis ranged between 2% and 30% in different studies. The histological picture does not usually show severe features. Antiplatelet agents or anticoagulation can be advised for aPL patients.

Humans↗

Urinary sediment features in proliferative and non-proliferative glomerular diseases.

BACKGROUND: The studies on urine sediment particles in patients with glomerular diseases (GD) are few and have focused only on single urine particles. In this study, we investigated the prevalence and number of 12 urine sediment particles in two groups of patients, one with proliferative GD, and the other with non-proliferative GD. METHODS: The urine sediment of 100 consecutive patients, with a renal biopsy-proven proliferative or non-proliferative GD and marked cylindruria, were examined a few hours before renal biopsy according to a standardized method. The urine particles investigated were erythrocytes, leukocytes, renal tubular cells, lipids and hyaline, hyaline-granular, granular, waxy, erythrocytic, leukocytic, epithelial and fatty casts. RESULTS: Patients with proliferative GD (n=52) had both a significantly higher prevalence of microscopic hematuria, leukocyturia, tubular epithelial cells, erythrocytic casts, epithelial casts, and significantly higher amounts of erythrocytes,leukocytes, tubular epithelial cells/20 high power field (HPF), erythrocytic and epithelial casts. On the other hand, patients with non-proliferative GD (n=48) had significantly higher numbers of fatty casts. In proliferative GD, leukocyturia was associated with intracapillary and extracapillary proliferation, crescents and fibrinoid necrosis at renal biopsy. At discriminant analysis, the two types of GD could be identified with 80.8% sensitivity and 79.2% specificity. By multiple logistic regression analysis, patients with erythrocytes, leukocytes and erythrocytic casts in the urine had an odds ratio (OR) of 9.91 (95% confidence interval (95% CI): 1.01-97.51), 7.85 (95% CI: 2.77-22.20), and 4.33 (95% CI: 1.41-13.31), respectively, of having proliferative GD. CONCLUSIONS: Our examination of the urine sediment shows that proliferative GD and non-proliferative GD differ in many respects.

Biopsy↗