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Biomedical subjects

J Font

Publications and source records attributed to J Font.

At least 91 records · Page 5Linked to original sources
PubMed2000

Splenectomy for refractory Evans' syndrome associated with antiphospholipid antibodies: report of two cases.

The main haematological manifestations seen in patients with antiphospholipid antibodies (aPL) are thrombocytopenia, usually mild, and haemolytic anaemia with a positive Coombs test. Owing to the shared characteristics with idiopathic thrombocytopenic purpura, similar rules are followed in the treatment of these cytopenias. Two patients with severe aPL associated cytopenias, who required splenectomy after being refractory to steroids, immunosuppressive agents, and other treatments (intravenous gammaglobulin, danazol), are described, and previously reported cases are reviewed.

Adult↗
PubMed2000

Circulating concentrations of soluble L-selectin (CD62L) in patients with primary Sjögren's syndrome.

OBJECTIVE: Serum concentrations of soluble (s) L-selectin (CD62L) were measured in patients with primary Sjögren's syndrome (SS) to relate these concentrations to clinical and immunological features of SS. METHODS: The study included 40 consecutive patients (38 women and two men) with a mean age of 61 years (range 24-78) who fulfilled four or more of the preliminary diagnostic criteria for SS proposed by the European Community Study Group in 1993, and 33 healthy blood donors from the hospital blood bank. A sandwich enzyme linked immunosorbent assay (ELISA) was used to detect the soluble form of human sL-selectin (CD62L). RESULTS: The mean (SEM) values of sL-selectin (CD62L) were 861 (66) microg/ml for patients with SS and 986 (180) microg/ml for healthy blood donors, but there was no significant difference. In patients with primary SS, serum sL-selectin (CD62L) concentrations were significantly higher in patients with Raynaud's phenomenon (1275 (112) microg/ml versus 789 (69) microg/ml, p=0.007), autoimmune thyroiditis (1162 (113) microg/ml versus 787 (69) microg/ml, p=0.02) and rheumatoid factor (993 (95) microg/ml versus 684 (70) microg/ml, p=0.01) when compared with patients without these features. CONCLUSION: The presence of Raynaud's phenomenon, autoimmune thyroiditis and rheumatoid factor is associated with higher concentrations of circulating sL-selectin (CD62L) in the sera of patients with primary SS.

Adult↗
PubMed2000

HLA class II alleles associations of anticardiolipin and anti-beta2GPI antibodies in a large series of European patients with systemic lupus erythematosus.

The objective of this study was to determine the HLA class II associations of the anticardiolipin (aCL) and anti-beta2GPI (abeta2GPI) antibodies in a large series of European patients with systemic lupus erythematosus (SLE). A cohort of 577 European SLE patients was enrolled. aCL and abeta2GPI were measured by ELISA methods. Molecular typing of HLA-DRB1, DRB3, DRB4, DRB5, DQA1 and DQB1 loci was performed by the polymerase chain reaction-sequence specific oligonucleotide probes (PCR-SSOP) method. aCL of IgG, IgM and IgA isotypes were detected in 22.8%, 14% and 13.9% of patients, respectively. IgG and IgM abeta2GPI were detected in 20% of patients. aCL showed positive association with HLA DRB1*04, DRB1*0402, DRB1*0403, DRB1*07, DRB3*0301, DQA1*0201, DQA1*0301, DQB1*0302, and negative association with DQA1*0501, DRB3*0202. abeta2GPI showed positive association with DRB1*0402, DRB1*0403, DQB1*0302. DRB1*0402 carried the highest relative risk for the presence of both aCL (RR=8. 1) and abeta2GPI (RR=4.6). Our results confirm the already described associations of aCL with HLA DR4 and DR7, but also demonstrate that, among the alleles at the DRB1*04 locus, the *0402 was most represented both in aCL and in abeta2GPI positive patients. In addition, HLA class II associations of abeta2GPI are for the first time extensively examined in a large cohort of European SLE patients.

Adolescent↗
PubMed2000

Primary Sjögren's syndrome in men: clinical and immunological characteristics.

OBJECTIVE: To determine the clinical and immunological characteristics of primary Sjögren's syndrome (SS) in men from a large series of unselected patients with this condition. METHODS: We studied 223 consecutive patients (204 women and 19 men; mean age at onset 53 y, range 15-87 y, mean disease duration 77 months) with primary SS visited in our units. All these patients fulfilled 4 or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. RESULTS: Nineteen (9%) patients were men and they represent the male group described in this paper. Extraglandular manifestations during the course of their disease were present in 10 (53%) of our male patients with primary SS: articular involvement in 4 (21%) patients, interstitial pneumopathy in 3 (16%) and peripheral neuropathy in 2 (11%). ANA were positive in 13 (68%) patients, RF in 5 (31%), anti-Ro/SS-A in 3 (16%) and cryoglobulins in 1/14 (7%). When compared with women, men with primary SS presented a lower prevalence of articular involvement (21 percent; vs 46%, P=0.03, OR 0.32, CI 0.07-0.97). CONCLUSION: Although primary SS is typically a disease of middle-aged women, clinicians should note that it may be diagnosed in male patients. Except for a lower prevalence of articular involvement, we could no find any notable differences in clinical and immunological characteristics between male and female patients with primary SS.

Adolescent↗
PubMed2000

Mixed cryoglobulinemia: new concepts.

The most documented extrahepatic manifestation of hepatitis C virus (HCV) infection is mixed cryoglobulinemia (MC). MC is characterised by the presence of temperature-sensitive protein complexes: in type II MC, cryoglobulins are composed of a monoclonal rheumatoid factor (usually, IgMkappa) against polyclonal IgG. In type III MC, all components are polyclonal. The presence of microheterogeneity and other new types of cryoglobulins is a novel and recent observation. The production of different autoantibodies and circulating immune complexes, including the cryoglobulins, are responsible for systemic vasculitis and various organ damage. In a limited number of MC patients, a malignancy, that is B-cell non-Hodgkin's lymphoma or hepatocellular carcinoma, may also develop. Finally, results of interferon and/or ribavirin treatments in MC patients represent an indirect proof for the pathogenetic link between MC and HVC infection. The discovery of the relation between HCV infection and MC shows the striking association between a viral infection and an autoimmune disease and, thus, a potential link between the systemic autoimmune and lymphoproliferative disorders.

Antiviral Agents↗
PubMed2000

Psychiatric and psychosocial disorders in patients with systemic lupus erythematosus: a longitudinal study of active and inactive stages of the disease.

The objective was to analyze psychiatric disorders and psychosocial dysfunction in patients with systemic lupus erythematosus (SLE), studied longitudinally during active and subsequent inactive stage of their disease. During a 6 month period of study, we selected 20 consecutive patients with SLE who presented with a SLE flare. All patients fulfilled the 1982 revised criteria of the American College of Rheumatology for the classification of SLE. When patients entered the study, we performed psychiatric (CIS, RDC, STAI, HD, BDI, GHQ and MMS) psychosocial (GAS and VAS-P) scores assessment. One year later, we repeated the psychiatric and psychosocial assessment when patients showed inactive disease. The 20 patients evaluated were women, with a mean age of 34 y (SE 14.4, range 20-57). According to CIS evaluation, we diagnosed 8 (40%) psychiatric cases in the acute episode of SLE. The RDC diagnosis showed generalized anxiety in 5 patients, panic disorders in 2 patients and generalized anxiety plus depressive symptoms in one patient. One year later, when patients did not show disease activity, we diagnosed 2 (10%) psychiatric cases (P<0.05). When SLE patients were clinically inactive, they showed lower levels of psychological distress (GHQ scale, 1.8 vs 5.6, P<0.001), with a lower grade of anxiety measured by both HA (3.2 vs 8.2, P<0.01) and STAI-S (7.95 vs 20.90, P<0.001) scales. We also found a lower score in pain perception (VAS-P) (2.80 vs 4.25, P<0. 01) and higher occupational activity (VAS-P) (83.9 vs 66.2, P<0.01) and general functioning (GAS) (93.75 vs 83.50, P<0.05) during the inactive stage. No significant differences were found when we compared cognitive impairment, grade of depression and physical disability between inactive and active stages. We conclude that in SLE patients, psychiatric and psychosocial disorders during acute episodes are usually mild and seem to be related to the psychological impact of disease activity on patients. This type of psychiatric pathology is similar to that which would be expected in other groups coping with a stressful event, indicating that our patients did not react in a way specifically determined by their systemic disease.

Adult↗
PubMed2000

Anti-ganglioside antibodies in a large cohort of European patients with systemic lupus erythematosus: clinical, serological, and HLA class II gene associations. European Concerted Action on the Immunogenetics of SLE.

OBJECTIVE: To assay anti-ganglioside antibodies (aGM1) in sera of a large cohort of European patients with systemic lupus erythematosus (SLE) to define the prevalence of these autoantibodies in SLE; to evaluate the association of aGM1 with clinical manifestations and other autoantibodies found in SLE; and to search for aGM1 association with HLA class II alleles. METHODS: Four hundred forty-eight patients with SLE were consecutively enrolled in 8 centers from 6 European countries. All sera were tested for antinuclear antibodies by immunofluorescence on HEp-2 cells as substrate, anti-dsDNA, aGM1, aCL, abeta2-glycoprotein I (abeta2-GPI) antibodies by ELISA, and antineutrophil cytoplasmic antibodies (ANCA) by immunofluorescence and by ELISA. Genomic typing for HLA class II loci was performed by polymerase chain reaction-sequence specific oligonucleotide probe method. Clinical assessment was done at the time of enrolment. RESULTS: We found 41.9% of patients with clinical signs of neuropsychiatric involvement; 15.5% of patients were positive for aGM1, 8% of the IgG isotype and 8.6% of the IgM isotype; aGM1-IgG were associated with neuropsychiatric manifestations (NPM) (RR = 3.7), with migraine (RR = 2.4), with OBS (RR = 7.3), and with peripheral neuropathy (RR = 8.5). aGM1-IgM were associated with NPM (RR = 4) and with depression (RR = 3.4). Furthermore, the genetic study showed that aGM1-IgG were associated with HLA-DQB1*0404 (RR = 7.2) while aGM1-IgM were associated with HLA-DQB1*0605 (RR = 33.3). No associations were found between aGM1 and anti-dsDNA, aCL, abeta2GP1, or ANCA. CONCLUSION: Our results show aGM1 can be found in patients with SLE. aGM1 may play a pathogenetic role for some NPM in this condition.

Adolescent↗
PubMed2000

Catastrophic antiphospholipid syndrome: clinical description and triggering factors in 8 patients.

In 1992, an attempt to single out a different and important group of patients was adopted by introducing the term "catastrophic" antiphospholipid syndrome (APS) to describe their potentially life threatening clinical course, characterized by acute multiorgan failure (3 or more organ systems damaged). Patients previously described in the literature showed certain differences that appear to distinguish this minority group of patients with catastrophic APS from the majority of APS patients. We describe 8 patients with catastrophic APS, focusing especially on the possible extrinsic factors (mainly infections) that might trigger this "catastrophic" situation.

Adolescent↗
PubMed2000

Prevalence and clinical significance of antiprothrombin antibodies in patients with systemic lupus erythematosus or with primary antiphospholipid syndrome.

BACKGROUND AND OBJECTIVE: Antibodies to prothrombin (aII) have been identified in patients with antiphospholipid antibodies, but their clinical significance is not well known. The aim of our study was to investigate their prevalence and association with clinical manifestations of the antiphospholipid syndrome (APS) in patients with primary APS or with systemic lupus erythematosus (SLE). DESIGN AND METHODS: A series of 177 patients with autoimmune diseases was studied: 70 with primary APS and 107 with systemic lupus erythematosus. A control group of 87 healthy volunteers were included in the study. aII were investigated in sera by an ELISA, using human prothrombin as antigen fixed in irradiated polystyrene plates. RESULTS: aII prevalence in patients with autoimmune disease was 47% (57% and 40% in patients with primary APS or with SLE, respectively) significantly higher than in controls (5%) (p<0.0001). In the whole series, thrombotic events were more prevalent in patients with aII (45% vs 28%; p=0.02). Moreover, aII was found to be an independent risk factor for arterial thrombosis (OR=2.4; p=0. 04). Similarly, in patients with SLE, aII were associated with both arterial and venous thrombosis (35% vs 14%; p=0.01), although only IgG-aII (OR=3.7; p=0.01) had an independent value as risk factor for thrombosis. However, a relationship between aII and thrombosis was not found in primary APS. aII were associated with thrombocytopenia only in patients with primary APS (OR=6.7; p=0.007). INTERPRETATION AND CONCLUSIONS: aII seem to be a serological marker of thrombosis in autoimmune diseases, mainly in SLE patients and/or in the arterial territory.

Abortion, Spontaneous↗
PubMed1999

Protein kinase C inhibits CD95 (Fas/APO-1)-mediated apoptosis by at least two different mechanisms in Jurkat T cells.

We have recently reported that activation of protein kinase C (PKC) plays a negative role in CD95-mediated apoptosis in human T cell lines. Here we present data indicating that although the PKC-induced mitogen-activated protein kinase pathway could be partially implicated in the abrogation of CD95-mediated apoptosis by phorbol esters in Jurkat T cells, the major inhibitory effect is exerted through a PKC-dependent, mitogen-activated protein kinase-independent signaling pathway. Furthermore, we demonstrate that activation of PKC diminishes CD95 receptor aggregation elicited by agonistic CD95 Abs. On the other hand, it has been reported that UV radiation-induced apoptosis is mediated at least in part by the induction of CD95 oligomerization at the cell surface. Here we show that activation of PKC also inhibits UVB light-induced CD95 aggregation and apoptosis in Jurkat T cells. These results reveal a novel mechanism by which T cells may restrain their sensitivity to CD95-induced cell death through PKC-mediated regulation of CD95 receptor oligomerization at the cell membrane.

Animals↗
PubMed1999

Bimetallic catalysts for continuous catalytic wet air oxidation of phenol.

Catalytic wet oxidation has proved to be effective at eliminating hazardous organic compounds, such as phenol, from waste waters. However, the lack of active long-life oxidation catalysts which can perform in aqueous phase is its main drawback. This study explores the ability of bimetallic supported catalysts to oxidize aqueous phenol solutions using air as oxidant. Combinations of 2% of CoO, Fe2O3, MnO or ZnO with 10% CuO were supported on gamma-alumina by pore filling, calcined and later tested. The oxidation was carried out in a packed bed reactor operating in trickle flow regime at 140 degrees C and 900 kPa of oxygen partial pressure. Lifetime tests were conducted for 8 days. The pH of the feed solution was also varied. The results show that all the catalysts tested undergo severe deactivation during the first 2 days of operation. Later, the catalysts present steady activity until the end of the test. The highest residual phenol conversion was obtained for the ZnO-CuO, which was significantly higher than that obtained with the 10% CuO catalyst used as reference. The catalyst deactivation is related to the dissolution of the metal oxides from the catalyst surface due to the acidic reaction conditions. Generally, the performance of the catalysts was better when the pH of the feed solution was increased.

Aluminum Oxide↗
PubMed1999

Sjögren's syndrome and hepatitis C virus.

Sjögren's Syndrome (SS) is an autoimmune disease that mainly affects exocrine glands and usually presents as a persistent dryness of the mouth and eyes. The spectrum of the disease extends from an organ-specific autoimmune disease to a systemic process. Viral infection has long been suspected as a potential cause of SS because several viruses have been incriminated in the aetiology of this disease, and a possible relationship between SS and hepatitis C virus (HCV) was postulated in 1992. In this paper, we review the literature concerning SS and HCV infection and summarise the current knowledge regarding their association and their pathogenic, clinical and immunological significances. The main conclusions of this review are that the prevalence of antibodies to HCV in patients with primary SS ranges between 14 and 19% using third-generation ELISA, chronic HCV infection may mimic the main clinical, histological and immunologic features of 'primary' SS and, finally, testing for HCV infection must be performed in patients with SS, especially in those patients with evidence of liver involvement or associated cryoglobulinaemia. HCV seems to be a rare cause of 'primary' SS in the absence of recognised liver disease or cryoglobulinaemia.

Enzyme-Linked Immunosorbent Assay↗
PubMed1999

Calcitriol-mediated hypercalcaemia and increased interleukins in a patient with sarcoid myopathy.

In this report we describe a patient with Sjögren's syndrome (SS) and calcitriol-mediated hypercalcaemia. Initially, there was no clinical evidence of sarcoidosis. The patient had hypercalcaemia associated with increased calcitriol serum levels; circulating interleukin-6 and tumour necrosis factor alpha levels were also elevated. At the beginning, therapy with clodronate was effective in decreasing the serum calcium levels. However, the serum calcitriol decreased only after chloroquine treatment was added. After 2 years of therapy, the patient developed progressive and extensive muscle weakness. A muscle biopsy revealed a very prominent non-caseating granulomatous myopathy. Corticosteroid therapy was then instituted. Although both chloroquine and corticosteroid therapy were associated with decreased serum interleukin and calcitriol levels, only corticosteroid therapy was effective in treating the sarcoid myopathy. The role of cytokines in calcitriol mediated hypercalcaemia is discussed.

Antirheumatic Agents↗
PubMed1999

Association of antiphospholipid antibodies with retinal vascular disease in systemic lupus erythematosus.

OBJECTIVES: To study the prevalence and characteristics of retinal vascular disease in patients with systemic lupus erythematosus (SLE) and to analyze their relationship with antiphospholipid antibodies (aPL) and other serological markers. PATIENTS AND METHODS: Eighty-two consecutive patients (77 women and 5 men; mean age, 36 years) were studied. All patients fulfilled the 1982 revised criteria of the American College of Rheumatology for the classification of SLE. Ophthalmologic examination included assessment of best corrected visual acuity, tonometry, slit-lamp biomicroscopy, and fundus examination. Serologic studies included determination of anticardiolipin antibodies (aCL) (ELISA), lupus anticoagulant (LA) (coagulation tests), antinuclear antibodies (indirect immunofluorescence), anti-DNA (Farr's test), and anti-ENA antibodies (counterimmunoelectrophoresis). RESULTS: Retinal vascular disease was detected in 13 (15%) of 82 SLE patients. The retinal lesions consisted of retinal vascular occlusions in six patients (five arterial and one venous), cotton-wool spots in three, optic disc edema in three, retinal hemorrhages in three, and ischemic optic neuropathy in one. Antiphospholipid antibodies were detected in 10 (77%) of these 13 patients: nine had aCL and two had the LA. When compared with patients without retinal vascular disease, patients with retinopathy had a higher prevalence of aPL (77% v. 29%, P = .005). CONCLUSIONS: Retinal vascular disease is frequent in patients with SLE. The presence of aPL is associated with a higher prevalence of retinal abnormalities in SLE patients.

Adolescent↗
PubMed1999

Clinical study and follow-up of 100 patients with the antiphospholipid syndrome.

OBJECTIVES: To study the clinical characteristics at diagnosis and during follow-up of patients with the antiphospholipid syndrome (APS) and to analyze the influence of treatment on their outcome. PATIENTS: One hundred patients with APS were included (86% female and 14% male; mean age, 36 years). Sixty-two percent had primary APS and 38% had APS associated with systemic lupus erythematosus (SLE). The median length of follow-up was 49 months. RESULTS: Fifty-three percent of the patients had thromboses, 52% had thrombocytopenia, and 60% of the women had pregnancy losses. Patients with APS associated with SLE had a higher prevalence of hemolytic anemia (P = .02), thrombocytopenia (platelet count lower than 100 x 10(9)/L) (P = .004), antinuclear antibodies (P = .0002), and low complement levels. Fifty-three percent of the patients with thrombosis had recurrent episodes (86% in the same site as the previous thrombotic event). Recurrences were observed in 19% of the episodes treated with long-term oral anticoagulation, in 42% treated prophylactically with aspirin, and in 91% in which anticoagulant/antiaggregant treatment was discontinued (P = .0007). Multivariate analysis showed that prophylactic treatment and older age had an independent predictive value for rethrombosis. Prophylactic treatment during pregnancy (usually with aspirin) increased the live birth rate from 38% to 72% (P = .0002). CONCLUSIONS: Patients with APS have a high risk of recurrent thromboses. Long-term oral anticoagulation seems to be the best prophylactic treatment to prevent recurrences. Prophylactic treatment with aspirin during pregnancy reduced the rate of miscarriages remarkably.

Adolescent↗