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Biomedical subjects

J Font

Publications and source records attributed to J Font.

At least 145 records · Page 8Linked to original sources

Chorea in the antiphospholipid syndrome. Clinical, radiologic, and immunologic characteristics of 50 patients from our clinics and the recent literature.

We analyzed the clinical, radiologic, and immunologic characteristics of 50 patients with chorea and the antiphospholipid syndrome (APS) (6 from our clinics and 44 from a MEDLINE computer-assisted review of the literature from 1985 through 1995). Forty-eight (96%) patients were female and 2 (4%) were male. Twenty-nine (58%) patients had defined systemic lupus erythematosus (SLE), 6 (12%) had "lupus-like" syndrome, and 15 (30%) patients had "primary" APS. Mean age of patients in this series was 23 +/- 12 years (range, 6-77 yr); mean age at presentation of chorea was 21 +/- 12 years (range, 6-77 yr). In 11 (22%) patients, the onset of chorea was in childhood (6-14 yr), and in 2 (4%) patients it presented at 60 years or more. Six (12%) patients developed chorea soon after they started taking estrogen-containing oral contraceptives, 3 (6%) developed chorea gravidarum, and 1 (2%) patient developed chorea shortly after delivery. Most patients (66%) presented only 1 episode of chorea. Chorea was bilateral in 55% of patients. Computed tomography and magnetic resonance imaging scans reported cerebral infarcts in 35% of patients. The following antibodies were detected: lupus anticoagulant (92%), anticardiolipin antibodies (91%), antinuclear antibodies (82%), anti-DNA (59%), anti-Ro (10%), anti-RNP (8%), anti-La (2%), and anti-Sm (2%). The chorea in these patients responded to a variety of medications, for example, steroids, haloperidol, antiaggregants, anticoagulants, or a combination of therapy, usually prescribed in the presence of other manifestations of APS or SLE. However, many patients responded well to haloperidol and to the discontinuation of oral contraceptives if this was the precipitating factor.

Adolescent↗

Hepatitis C virus infection in 'primary' Sjögren's syndrome: prevalence and clinical significance in a series of 90 patients.

OBJECTIVES: To determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in a large cohort of patients with "primary' Sjögren's syndrome (SS). METHODS: 90 consecutive patients (83 female and seven male) were included, with a mean age of 62 years (range 31-80) who prospectively visited our unit. All patients fulfilled the European Community criteria for SS and underwent a complete history, physical examination, as well as biochemical and immunological evaluation for liver disease. Serum from all patients was tested for antibodies to HCV by third generation enzyme linked immunoassay and positivity was confirmed by polymerase chain reaction. RESULTS: Antibodies to HCV were present in 13 (14%) patients with 'primary' SS. When compared with patients without HCV infection, patients with HCV infection presented a higher prevalence of hepatic involvement, (100% v 8%, p < 0.05). Transcutaneous liver biopsy was performed in five patients with HCV infection, and specimens obtained showed in all cases a chronic active hepatitis with varying degrees of portal inflammation. CONCLUSION: HCV infection is frequent in patients with "primary' SS and liver involvement is presented in all these patients. The possible pathogenic role of HCV infection in these patients is still unclear.

Adult↗

Shrinking lungs syndrome in systemic lupus erythematosus: improvement with inhaled beta-agonist therapy.

The pathogenesis and therapy of shrinking lungs syndrome in patients with systemic lupus erythematosus remains controversial. It has been previously reported that corticosteroid treatment can be effective. We report a patient with shrinking lungs syndrome who presented a good response to inhaled beta-agonist therapy. Therapeutic approach to this syndrome is discussed.

Administration, Inhalation↗

Anti-endothelial cell antibodies in systemic autoimmune diseases: prevalence and clinical significance.

OBJECTIVE: To investigate the prevalence and characteristics of anti-endothelial cell antibodies (AECA) in a large cohort of patients with several well defined systemic autoimmune diseases, in order to determine their relationship with the clinical and laboratory features of these diseases. METHODS: Clinical and laboratory features of 216 consecutive Caucasian patients were prospectively studied. One hundred and seven patients had been diagnosed as having a primary systemic vasculitis-specifically, 39 had temporal arteritis (TA), 25 polyarteritis nodosa (PAN), 9 Wegener's granulomatosis (WG), and 34 Behcet's disease (BD)-, 90 patients had systemic lupus erythematosus (SLE), and 19 had a primary Sjogren's syndrome (SS). The AECA were determined by ELISA. RESULTS: One hundred and four (48%) patients with systemic autoimmune diseases were found to have a positive titre of AECA. Specifically, AECA were detected in 41 (38%) patients with a primary systemic vasculitis (13 (33%) with TA, 14 (56%) with PAN, 5 (56%) with WG and 9 (26%) with BD), in 58 (63%) patients with SLE, and in 5 (26%) patients with a primary SS. In patients with a primary systemic vasculitis, those with AECA were found to have an increased prevalence of disease activity (P < 0.05). In SLE patients, those with AECA were found to have an increased prevalence of vascular lesions (P < 0.05), lupus nephropathy (P < 0.05), and anticardiolipin antibodies (aCL) (P < 0.001). CONCLUSIONS: Patients with systemic autoimmune diseases have a high prevalence of AECA and they are associated with the presence of vascular lesions, nephropathy, and aCL in SLE, as well as with disease activity in several primary systemic vasculitis (TA, PAN, WG and BD).

Adult↗

Elevated soluble CD27 levels in serum of patients with systemic lupus erythematosus.

The CD27 belongs to the NGF-R, a family of type I transmembrane receptors present in most T cells. The soluble form of CD27 can be found in body fluids, and elevation of serum levels is observed in T cell activation. Preliminary studies indicate that measurement of sCD27 levels might be of use in the evaluation of immune responses in vivo. Systemic lupus erythematosus (SLE) is a disease of unknown etiology. T cells from SLE patients have impaired responsiveness to various kinds of stimuli. However, the precise localization of the defect in the activation of SLE remains unclear. In this study we have analyzed the sCD27 serum levels in a SLE population and in a healthy group control. Seventy patients with SLE were prospectively studied. As controls, 20 healthy volunteer blood donors, matched by sex and age, were studied. We developed an enzyme-linked immunosorbent assay (ELISA) to measure sCD27. The mean level (+/-SD) of sCD27 was higher in the SLE patients (48.29 +/- 23.86 u) than that in the control group (36.13 +/- 7.48 u) (P = 0.02). In addition, patients with active SLE revealed higher serum concentration of sCD27 (58.20 +/- 31.06 u) than that of patients in remission (42.77 +/- 16.71 u) (P < 0.01). We found a positive correlation among sCD27 and sCD25 serum levels in SLE patients (r = 0.30, P = 0.01). No significant relation was found for other clinical symptoms or immunological parameters. In conclusion, sCD27 serum levels were increased in SLE patients and this increase was associated with the activity of the disease. Our data are consistent with the hypothesis that the CD27 antigen may constitute a marker of disease activity.

Adolescent↗

Intravenous immunoglobulin preceding in vitro fertilization-embryo transfer for patients with repeated failure of embryo transfer.

OBJECTIVE: To determine the effectiveness of immunotherapy with high-dose IV immunoglobulin preceding IVF-ET for patients with repeated failure of ET. DESIGN: Prospective, observational. SETTING: Assisted Reproduction Unit of the Hospital Clínic i Provincial in Barcelona, a tertiary care setting. PATIENTS: Twelve consecutive tubal infertility patients experiencing repeated unexplained IVF-ET failure including at least three ETs replacing three to four fresh embryos each. Two women shared three or more human leukocyte antigens (HLA) with the husband. INTERVENTION: During the subsequent new IVF-ET cycle, each patient received 400 mg/kg IV immunoglobulin daily for 5 days during ovarian stimulation, that is, 5 to 7 days before ET. MAIN OUTCOME MEASURES: Clinical pregnancies. RESULTS: No implantation occurred. There were no side effects. CONCLUSIONS: High-dose IV immunoglobulin is not a useful tool for IVF-ET failure.

Adult↗

Source abundances of ultra heavy elements derived from UHCRE measurements.

A total of 205 tracks have been located, measured, and positively identified as originating from Ultra Heavy (Z > or = 65) cosmic ray ions with energies over 2 GeV/amu in the 10 UHCRE plastic track detector (mainly Lexan polycarbonate) stacks studied by our Group. About 40 values of reduced etch rate S have been obtained along each of these tracks. A method based on determining the gradient of S, together with calibration in accelerators, is used to determine the charge of each ion resulting in one of such tracks to obtain the charge spectrum of the recorded Ultra Heavy ions. The abundance ratio of ions with 87 < or = Z < or = 100 to those with 74 < or = Z < or = 86 as well as that of ions with 81 < or = Z < or = 86 to those with 74 < or = Z < or = 80 are calculated at 0.016 and 0.32, respectively, which agree with the values obtained from measurements in the HEAO-3 and Ariel-6 experiments. The abundance ratio of ions with 70 < or = Z < or = 73 to those with 74 < or = Z < or = 86 is also calculated, but its value (0.074) did not seem to be significant because of our detectors' low registration efficiency in the charge range 70 < or = Z < or = 73. A computer program developed by our Group, based on the Leaky Box cosmic ray propagation model, has been used to determine the source abundances of cosmic ray nuclei with Z > or = 65 inferred from the abundances measured in the UHCRE. It appeared that r-process synthesized elements were overabundant compared to the Solar System abundances, as predicted by other authors.

Actinoid Series Elements↗

Antiphospholipid antibodies and human reproductive failure.

Routine screening for circulating antiphospholipid antibodies (aPL), namely the lupus anticoagulant (LA) and anticardiolipin antibodies (aCL), was carried out in a total of 1273 women aged < 45 years. Of them, 822 were experimental subjects and 451 were controls. The former comprised the following three study groups: 498 infertile patients (group 1), 284 spontaneous recurrent aborters (group 2), and 40 patients with repeated failure of embryo transfer (group 3). Controls included five groups of women: 125 normal healthy women who had never been pregnant (group 4), 125 normal healthy parous women with no previous abortion (group 5), 52 women in labour after normal pregnancies at term (group 6), 49 infertile patients achieving a livebirth with their first in-vitro fertilization (IVF) and embryo transfer (group 7), and 100 female patients with systemic lupus erythematosus (positive controls, group 8). aPL positivity in the eight groups studied was as follows: 24, 9.2, 10, 0.8, 0, 0, 0 and 42% respectively for groups 1 to 8. There were no differences within groups 1 and 3 regarding incidence of aPL when patients were grouped according to infertility aetiological factors and indications of IVF respectively. Twenty-six out of 284 recurrent aborters (9.2%) tested positive for aPL, and the LA and/or a CL were identified as the aetiological factor in 12% of patients (24/199) with supposedly unexplained recurrent abortion. Incidence of positive sera for aPL in group 1 was similar to that observed in control groups 4, 5 and 6. On the contrary, incidence of aPL positivity in groups 2 and 3 was significantly higher than in control groups 4, 5 and 6 and among infertile women (group 1). The difference between groups 3 and 7 almost reached statistical significance. Interestingly, there was no difference between groups 2 and 3, but groups 2 and 7 resulted probably different regarding incidence of aPL positive sera. As expected, the highest incidence of patients testing positive for aPL was found in group 8. Seven infertile patients having circulating aPL and becoming pregnant spontaneously or after specific infertility treatment, successfully carried to term in spite of the fact that they did not receive immunotherapy. Among recurrent aborters, the live-born baby rate was significantly higher after treatment with low-dose aspirin than prior therapy. It is concluded that the presence of circulating aPL may be associated with recurrent abortion but with infertility. In addition, our results favour a possible role of aPL in failure of implantation after IVF embryo transfer.

Abortion, Habitual↗

Antibodies to endothelial cells and to beta 2-glycoprotein I in the antiphospholipid syndrome: prevalence and isotype distribution.

The aim of this study was to analyse the prevalence and isotype distribution of antibodies to endothelial cells (aEC) and to beta 2-glycoprotein I (a beta 2GPI) in the antiphospholipid syndrome (APS). Fifteen patients with an APS [nine associated with systemic lupus erythematosus (SLE) and six "primary'] and 15 with SLE without an APS were prospectively studied. The aEC were determined by an enzyme-linked immunosorbent assay (ELISA) using endothelial cells derived from human umbilical vein and the a beta 2GPI by ELISA using highly purified beta 2GPI. A positive titre of aEC was detected in 20 out of 30 patients (67%), but in none of the control group. Ten patients had both IgG and IgM isotypes, five had IgG only and five had only IgM. Thirteen patients with the APS (87%) were found to have a positive titre of aEC, while only seven with SLE but without a history of APS (47%) had aEC (P < 0.05). Nine patients with the APS (60%) had a positive titre of a beta 2GPI (four had both IgG and IgM isotypes, one had IgG only and four had only IgM), while none of the patients without an APS (0%) had these antibodies (P < 0.001). A significant association was also found between the presence of aPL and aEC (P < 0.05), as well as between aPL and a beta 2GPI (P < 0.001). Both aEC and a beta 2GPI can be found in the APS. This reinforces the theory that APS represents a complex autoimmune disorder in which several autoantibodies co-exist with aPL.

Adult↗

Von Willebrand factor in the outcome of temporal arteritis.

OBJECTIVE: To determine fluctuation in circulating von Willebrand factor (vWF) in the outcome of patients with temporal arteritis. METHODS: Plasma vWF antigen concentrations were measured in 65 patients with biopsy proven temporal arteritis at different disease activity stages, in 12 with isolated polymyalgia rheumatica, and in 16 controls. Fourteen temporal arteritis patients underwent serial determinations during the course of their disease. RESULTS: vWF concentrations were significantly raised in temporal arteritis (mean 220 [arbitrary units], range 96 to 720) and in polymyalgia rheumatica (mean 196, range 103 to 266) compared with healthy controls (mean 98, range 75 to 137) (P < 0.05). Although vWF values tended to be higher in temporal arteritis, no significant differences were found between temporal arteritis and polymyalgia rheumatica patients nor between temporal arteritis patients with ischaemic complications (mean 269, range 130 to 720) and those who presented with polymyalgia rheumatica or constitutional symptoms only (mean 179, range 140 to 220). The highest levels were obtained in patients with associated, mainly infectious, diseases (mean 631, range 240 to 1680). Raised vWF values found in active temporal arteritis patients (mean 220, range 96 to 720) persisted within the first two years after the beginning of treatment (mean 244, range 102 to 510) but tended to normalise in patients in long term remission (mean 143, range 50 to 260). CONCLUSIONS: Persistent elevation of vWF during early remission of temporal arteritis might represent an endothelial activation status induced by a remaining inflammatory microenvironment rather than a marker of endothelial cell injury. In long term remission, decreasing vWF concentrations might reflect progression of inflammatory lesions to a healing stage.

Acute Disease↗

Hypercoagulable state in patients with antiphospholipid syndrome is related to high induced tissue factor expression on monocytes and to low free protein s.

Antiphospholipid antibodies (aPLs) are associated with thrombosis, but the mechanisms of this thrombotic tendency are unknown. We studied 56 patients 612 with systemic lupus erythematosus [SLE] and aPLs and previous thrombosis, 12 with SLE and aPLs but no thrombosis, 15 with SLE without aPLs or thrombosis, 11 with primary antiphospholipid syndrome with thrombosis, and 6 asymptomatic subjects with aPLs) to investigate the ability of aPLs to induce tissue factor (TF) expression on human normal monocytes. A double direct immunofluorescence technique (anti-CD14 and anti-TF) was used, and procoagulant activity in viable and disrupted cells was measured after plasma incubation for 6 hours at 37 degrees C with normal mononuclear cells. Hemostasis regulatory proteins, prothrombin fragment 1 + 2, and thrombin-antithrombin III complex levels were determined. Increased TF expression and procoagulant activity were observed using plasma samples from SLE patients with aPLs and thrombosis (P < .01) and from primary antiphospholipid syndrome patients (P < .01) but not from patients with SLE and aPLs but no thrombosis, patients with SLE without aPLs, or asymptomatic patients with aPLs. Purified aPL immunoglobulins from one primary antiphospholipid syndrome and two SLE patients added to normal plasma showed a significant increase in both TF expression and procoagulant activity (P < .05) compared with purified aPL from two SLE patients without thrombosis. The addition of nonspecific IgG from three SLE patients without aPLs and from three control subjects did not increase TF expression. Low free protein S was seen in eight patients. Increased TF expression and low free protein S correlated with thrombosis (P < .01) and with higher prothrombin fragment 1 + 2 and thrombin-antithrombin III values (P < .01). These observations may contribute to a further understanding of the thrombotic risk in aPL patients.

Antiphospholipid Syndrome↗

Reduced serum creatine kinase activity in inflammatory rheumatic diseases.

OBJECTIVE: To determine if serum CK activity is reduced in inflammatory rheumatic diseases and to evaluate whether this phenomenon is linked to disease activity or steroid therapy. METHODS: Serum CK activity was measured in patients with systemic lupus erythematosus (SLE, n = 52), rheumatoid arthritis (RA, n = 80), ankylosing spondylitis (AS, n = 82), spondyloarthropathies other than AS (SpA, n = 22), and a miscellaneous group (MI, n = 27), and in 103 control patients with noninflammatory arthropathies (NIA). Laboratory variables of inflammatory activity such as ESR, CRP, platelet count, and hemoglobin (and anti-DNA antibodies and complement levels in SLE) were measured at the same time. Daily dose of steroids was also evaluated. RESULTS: Serum CK activity was significantly reduced in SLE (mean +/- SD: 49 +/- 41 IU/l), RA (68 +/- 41 IU/l), SpA (88 +/- 53 IU/l), and MI (75 +/- 32 IU/l) compared to controls (111 +/- 38 IU/l) (p = 0.002 for SpA and p < 0.001 for the other groups). No differences in CK values were observed between AS and controls, although AS patients with peripheral arthritis had lower serum CK activity than those without (80 +/- 32 vs 121 +/- 62 IU/l, respectively, p < 0.05. ESR, CRP, and platelets correlated inversely with CK values in RA, AS, and MI. In the SpA group only ESR correlated inversely with CK. In SLE, a positive correlation was found between CK values and CH50 and a negative one with anti-DNA levels. Patients taking steroids had significantly lower CK activity than those without corticotherapy. However, multivariate analysis showed that only inflammatory activity and no steroids had an effect in reducing CK activity. CONCLUSION: Serum CK activity is significantly reduced in several inflammatory rheumatic diseases. Inflammatory activity seems to play the major role in this phenomenon.

Adult↗

Existence of a soluble form of CD50 (intercellular adhesion molecule-3) produced upon human lymphocyte activation. Present in normal human serum and levels are increased in the serum of systemic lupus erythematosus patients.

CD50 (ICAM-3) is a leukocyte differentiation Ag expressed almost exclusively on hemopoietic cells, with a key role in the first steps of immune response. To develop a specific sandwich ELISA to detect a soluble CD50 form (sCD50), two different mAbs (140-11 and 101-1D2) recognizing non-overlapping epitopes were used. sCD50 was detected in the supernatant of stimulated PBMCs, with the highest levels after CD3 triggering. Simultaneously, the CD50 surface expression diminished during the first 24 h. sCD50 isolated from culture supernatant and analyzed by immunoblotting showed an apparent m.w. of 95 kDa, slightly smaller than the membrane form. These data, together with Northern blot kinetics analysis, suggest that sCD50 is cleaved from cell membrane. Furthermore, we detect sCD50 in normal human sera and higher levels in sera of systemic lupus erythematosus (SLE) patients, especially in those in active phase. The sCD50 levels showed a positive correlation with sCD27 levels (r = 0.4213; p = 0.0026). Detection of sCD50, both after in vitro CD3 triggering of PBMCs and increased in SLE sera, suggests that sCD50 could be used as a marker of lymphocyte stimulation.

Adolescent↗

A non-isotopic, highly sensitive, fluorimetric, cell-cell adhesion microplate assay using calcein AM-labeled lymphocytes.

A simple and sensitive cell-cell adhesion microplate assay was established using the cytoplasmic fluorescent dye, calcein AM. The procedure involves three steps: the labeling of lymphocytes with an adequate concentration of calcein AM (20 microM) during a short incubation period (30 min); the adhesion of 2 x 10(5) labeled lymphocytes per well to confluent keratinocyte or fibroblast monolayers grown in microtiter plates for 90 min; and, finally, measurement of the fluorescent signal utilizing a new system of cold-light microfluorimetry (Rat, 1993). During the adhesion assay, the release of calcein from labeled lymphocytes is low and the method permits the detection of as few as 1000 adherent cells. This non-radioactive procedure takes less than 4 h to perform and has proven to be as accurate and reliable as the common method using radioactive isotopes. In addition to its simplicity, the use of a fluorescent molecular probe in conjunction with cold-light microfluorimetry (CLF) offers many advantages of safety and economy, and can readily be adapted to the different cell types that participate in cell-cell adhesion.

Cell Adhesion↗

Prevalence and clinical significance of anticardiolipin antibodies in pregnancies complicated by parvovirus B19 infection.

Anticardiolipin antibodies were measured in 60 pregnant women with acute parvovirus B19 infection. Test results for eight (13.3 per cent) women were positive for anticardiolipin antibody. Six of these eight women became negative later, yielding a prevalence of anticardiolipin antibodies of 3.3 per cent (2/60) 6 months after acute parvovirus B19 infection. Anticardiolipin antibody positivity was not associated with an increased risk of abortion, fetal death, or maternal complications. This study suggests that there is an elevated frequency of anticardiolipin antibodies in pregnant women with acute parvovirus B19, probably representing an epiphenomenon. However, this is not associated with an adverse maternal or perinatal outcome.

Antibodies, Anticardiolipin↗