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J Font

Publications and source records attributed to J Font.

At least 109 records · Page 6Linked to original sources

Prevalence of hepatitis C virus infection in patients with antiphospholipid syndrome.

BACKGROUND/AIMS: The aim of this study was to determine the prevalence and clinical significance of hepatitis C virus (HCV) infection in patients with the antiphospholipid syndrome (APS). METHODS: A series of 88 consecutive patients (78 female and 10 male), with a mean age of 39 years (range 15-79), was prospectively studied. All patients had been diagnosed with APS: 54 (61%) primary APS and 34 (39%) APS associated with systemic lupus erythematosus. A group of 200 apparently healthly blood donors was included in the study. Anti-HCV antibodies were investigated in the serum of all patients using a third-generation ELISA and confirmed by recombinant immunoblot assay. RNA-HCV was investigated in anti-HCV positive samples by polymerase chain reaction. Anticardiolipin, anti-beta2-glycoprotein I and antiprothrombin antibodies were evaluated by ELISA. Lupus anticoagulant was studied by coagulometric assays. RESULTS: Only 2 (2.2%) patients showed positivity for anti-HCV antibodies, but none of them had clinical or biochemical signs of liver disease. Furthermore, RNA-HCV was not detected in serum of any of these patients. Lupus anticoagulant was positive in 57% of patients. Anticardiolipin antibodies were positive in 60% of patients, anti-beta2-glycoprotein I antibodies in 43% of patients, and antiprothrombin antibodies in 56% of patients. The prevalence of anti-HCV in blood donors was 1%. CONCLUSIONS: The prevalence of anti-HCV in patients with APS is low and similar to that in healthy people in our area. HCV infection does not seem to be involved in the etiopathogenesis of this syndrome.

Adolescent↗

Obstetrical outcome of pregnancy in patients with systemic Lupus erythematosus. A study of 60 cases.

OBJECTIVE: To analyze the course of maternal diseases and the outcome of pregnancy in patients with systemic Lupus Erythematosus (SLE). STUDY DESIGN: During a period of 11 years we prospectively followed 60 pregnancies in 46 SLE patients in a tertiary care center in Barcelona (Spain). The management protocol included: (1) planning of conception when disease was inactive; (2) frequent follow-up visits by an internist-obstetrician team; (3) use of sequential ultrasonographic, Doppler and fetal echocardiographic examinations; (4) serial evaluations of maternal immunological condition; and (5) low dose aspirin from 1 month before attempting conception and throughout pregnancy was added in women with antiphospholipid antibodies. From 1985 until 1994 prednisone prophylaxis was used in all lupus patients during the last month of pregnancy and during the first month of the puerperium; from 1995 onwards this regime was abandoned. RESULTS: The mean (S.D.) age of patients was 28.6 (4.8) years (range 20 to 42) and the mean (S.D.) previous duration of SLE was 6.25 (4.8) years (range 0 to 17). SLE was diagnosed during the pregnancy in two cases (3.3%) and the disease was active at conception in four cases (6.7%); at that time nine patients (15%) were taking prednisone. Antiphospholipid antibodies were positive in 16 patients (30.4%) and there were 10 (16.7%) pregnancies in patients having lupus nephropathy. There were three first-trimester miscarriages (5%) and four (6.7%) voluntary abortions. Obstetric complications in the remaining 53 pregnancies included: preterm delivery, 11 cases (20.8%); intrauterine growth retardation, five cases (9.4%); hypertension, 10 patients (18.9%), five of them fulfilling the criteria of preeclampsia; premature rupture of membranes, four patients (7.5%); finally, 13 neonates had a birthweight lower than 2500 g. There were 15 lupus flares (28.3%), giving a flare rate of 0.044 per patient/month. There were five neonatal deaths (perinatal mortality rate, 94 per thousand): one because of complete heart block, three due to severe hyaline membrane disease resulting from extreme prematurity and one intrauterine death in a patient having the Leiden mutation. CONCLUSION: Pregnancy in patients with SLE should not be regarded as an unacceptable high-risk condition for the mother or her baby provided that conception is accurately planned and patients are managed according to a careful multidisciplinary treatment schedule.

Female↗

Identification of a natural soluble form of human CD5.

CD5 is a 67 kDa type I glycoprotein which belongs to the Scavenger Receptor Cysteine-Rich (SRCR) family of receptors. This family includes either cell-surface (e.g. CD6) or secreted (e.g. Spalpha) proteins implicated in the development of the immune system and the regulation of immune responses. In this study, we purified and characterised a circulating natural soluble CD5 form (nsCD5) which is indistinguishable (in apparent molecular mass, glycosylation pattern, and antibody reactivity) from a recombinant soluble CD5 form (rsCD5) composed of the three extracellular SCRC domains. The nsCD5 is a N-glycosylated 52 kDa molecule present in normal human serum and in supernatants of in vitro phorbol ester- and CD3-stimulated peripheral blood mononuclear cells. The nsCD5 concentration in sera from healthy donors is relatively low (median 1.75 ng/ml, rn=166) and is similar to that found in sera from patients suffering of various autoimmune (systemic lupus erythematosus, primary Sjogren syndrome, rheumatoid arthritis) and non-autoimmune (chronic renal failure, B-cell chronic lymphocytic leukemia) disorders. In vitro experiments indicate that nsCD5 is released by proteolytic cleavage of the membrane form. These results represent the first evidence of proteolytic release of a transmembrane SRCR family member following cell activation.

Animals↗

Antibodies to yeast Sm motif 1 cross-react with human Sm core polypeptides.

Two regions common to all UsnRNP core polypeptides have been described: Sm motif 1 and Sm motif 2. Rabbits were immunized with a 22 amino-acid peptide containing one segment of Sm motif 1 (YRGTLVSTDNYFNLQLNEAEEF, corresponding to residues 11-32) from yeast F protein. After immunization, the rabbit sera contained antibodies that not only reacted specifically with the peptide from yeast F protein but also cross-reacted with Sm polypeptides from mammals; that is, with purified human U1snRNPs. The results suggest that the peptide used and human Sm polypeptides contain a common feature recognized by the polyclonal antibodies. A large collection of human systemic lupus erythematosus sera was assayed using the yeast peptide as an antigen source. Seventy per cent of systemic lupus erythematosus sera contain an antibody specificity that cross-reacts with the yeast peptide.

Amino Acid Sequence↗

Human reproductive failure is not a clinical feature associated with beta(2) glycoprotein-I antibodies in anticardiolipin and lupus anticoagulant seronegative patients (the antiphospholipid/cofactor syndrome)

It has been suggested that patients with clinical features suggestive of antiphospholipid syndrome but being lupus anticoagulant (LA) and anticardiolipin (aCL) negative, should be tested for antibodies to beta(2) glycoprotein-I (abeta(2)GP-I), a protein involved in the binding of antiphospholipid antibodies (aPL) to phospholipid surfaces. This was investigated in the present study where a total of 385 women aged </=40 years were included. Of these, 175 were experimental subjects and 210 were controls. The former comprised the following two study groups: 100 spontaneous recurrent aborters (group one), and 75 patients with repeated failure of embryo transfer (group two). Controls included three groups of women: 100 normal healthy parous women with no previous abortion (group three), 60 infertile patients achieving a live birth with their first in-vitro fertilization (IVF)/embryo transfer attempt (group four), and 50 patients with recurrent abortion who tested positive for aPL (LA and/or aCL) (positive controls, group five). Only one patient among recurrent aborters (group one) tested positive for abeta(2)GP-I. All women in groups two, three and four were negative for abeta(2)GP-I screening. As expected, prevalence of patients testing positive for abeta(2)GP-I was significantly higher in group five than among the other groups of patients (P < 0.001). No differences were observed regarding the prevalence of abeta(2)GP-I positive sera in the subgroup of patients having aCL and those having the LA in group five. It is concluded that abeta(2)GP-I screening in first-trimester recurrent abortion or in failure of implantation after IVF is not warranted in patients without aPL as detected by standard antiphospholipid assays.

Abortion, Habitual↗

Contribution of cosmic ray heavy ions to the radiation hazard in manned space flights.

Primary cosmic radiation arriving near the Earth may be classified into two general categories: the gamma component and the hadronic component. The hadronic component contains mainly protons, a small amount of alpha particles and a smaller amount of heavier charged nuclei (ions). Although the fluxes of these heavier ions are very small in comparison to those of protons, they are able to originate a huge linear energy transfer (LET). This work studies the contribution of heavy ions from cosmic rays to the radiation hazard to which the crew of a manned long duration space flight might be exposed. The geometry of the energy deposition by a heavy ion is studied, and it is found that energies of the order of up to 10(23) J kg-1 are deposited.

Cosmic Radiation↗

Morbidity and mortality in systemic lupus erythematosus during a 5-year period. A multicenter prospective study of 1,000 patients. European Working Party on Systemic Lupus Erythematosus.

In the present study we assessed the frequency and characteristics of the main causes of morbidity and mortality in SLE during a 5-year period and analyzed the prognostic significance for morbidity and mortality of the main immunologic parameters used in clinical practice. We started in 1990 a multicenter study of 1,000 patients from 7 European countries. All had medical histories documented and underwent medical interview and routine general physical examination when entered in the study, and all were followed prospectively by the same physicians during the ensuing 5 years (1990-1995). Four hundred thirteen patients (41.3%) presented 1 or more episodes of arthritis, 264 (26.4%) had malar rash, 222 (22.2%) active nephropathy, 139 (13.9%) fever, 136 (13.6%) neurologic involvement, 132 (13.2%) Raynaud phenomenon, 129 (12.9%) serositis (pleuritis and/or pericarditis), 95 (9.5%) thrombocytopenia, and 72 (7.2%) thrombosis. Two hundred seventy patients (27%) presented infections, 113 (11.3%) hypertension, 75 (7.5%) osteoporosis, and 59 (5.9%) cytopenia due to immunosuppressive agents. Sixteen patients (1.6%) developed malignancies, with the most frequent primary localizations the uterus and the breast. Several immunologic parameters (anti-dsDNA or antiphospholipid antibodies) were found to have a predictive value for the development of SLE manifestations during the period of the study. Forty-five patients (4.5%) died; the most frequent causes of death were divided similarly among active SLE (28.9%), infections (28.9%), and thromboses (26.7%). A survival probability of 95% at 5 years was found. A lower survival probability (92%) was detected in those patients who presented at the beginning of the study with nephropathy.

Adolescent↗

Intestinal volvulus in dogs: a study of four clinical cases.

Four cases of intestinal volvulus in German shepherd dogs are described. A definitive diagnosis was achieved by exploratory laparotomy in three cases and after necropsy in the remaining animal. Clinical signs, laboratory investigations and radiological changes are reported for three of the dogs. These dogs were all euthanased. Treatment of complete intestinal volvulus is difficult. By the time the condition is diagnosed, the pathological changes are irreversible, with consequent poor prognosis.

Animals↗

Paraneoplastic pemphigus: a case of long-term survival associated with systemic lupus erythematosus and polymyositis.

A 35-year-old man presented with a generalized bullous eruption and oral ulceration. He had been diagnosed as having systemic lupus erythematosus and pelvic Castleman disease (hyaline-vascular type) in the past. Histologic, direct and indirect immunofluorescence and immunoprecipitation studies confirmed a diagnosis of paraneoplastic pemphigus (PNP). Initially several medical treatments were tried unsuccessfully. The pelvic tumor was surgically removed and the mucocutaneous lesions slowly regressed. Four years after presentation, he developed polymyositis which was completely controlled with short courses of corticosteroids. There was no evidence of relapse of PNP or lupus erythematosus at that time. At the 6-year follow-up he showed no clinical evidence of PNP, lupus erythematosus or polymyositis without requiring immunosuppressive therapy. This case emphasizes the fact that patients with benign-neoplasm-associated PNP may undergo complete remission of the autoimmune disease upon complete excision of the tumor. This case also stresses the possible association of PNP with other autoimmune diseases such as lupus erythematosus and polymyositis.

Adult↗

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

The objective of the study was to determine the clinical and immunological characteristics of primary Sjogren's syndrome (SS) in patients with an older onset of the disease. We included 223 consecutive patients (204 female and 19 male; mean age at onset 53 years; range 15-87 years) visited in our Units. All patients were white and fulfilled four or more of the diagnostic criteria for SS proposed by the European Community Study Group in 1993. Disease onset was determined on the basis of the appearance of symptoms strongly suggestive of SS. In 31 patients the onset of disease occurred after the age of 70 years, and they represent the elderly-onset group described in this report. The remaining 192 patients presented disease onset before the age of 70 years, and they represent the younger-onset group. Of the 31 (14%) patients with elderly onset of primary SS, 26 were female and 5 male, and the disease onset occurred between 70 and 87 years (mean 74 years). The most common extraglandular manifestations were articular involvement (29%), hepatic involvement (20%), peripheral neuropathy (16%) and interstitial pneumopathy (13%). When compared with patients with a younger onset, the prevalences of glandular and extraglandular manifestations and immunological features (cryoglobulinemia, hypocomplementemia and positivity for RF, anti-Ro/SS-A or anti-La/SS-B) were similar in both groups. In conclusion, although primary SS is typically a disease of middle-aged adults, clinicians should note that it may be diagnosed frequently among elderly patients. However, we could not find any notable differences in clinical and immunological characteristics of patients with elderly onset of primary SS.

Adolescent↗

Nodular regenerative hyperplasia of the liver and antiphospholipid antibodies: report of two cases and review of the literature.

Nodular regenerative hyperplasia of the liver (NRHL) is a rare disorder characterized by diffuse micronodular transformation of the hepatic parenchyma without fibrous septa between the nodules. This condition appears to be associated in many occasions with systemic autoimmune diseases. We describe two new patients with NRHL in whom antiphospholipid antibodies (aPL) were detected in their sera and review the few similar cases reported previously. We also discuss the possible relationship between aPL and NRHL and suggest that these antibodies may play a role in the pathogenesis of some cases of NRHL, specially those with an associated antiphospholipid syndrome.

Adolescent↗

The pulmonary-renal syndrome: a poorly understood clinicopathologic condition.

The co-existence of pulmonary hemorrhage and glomerulonephritis delineates a severe syndrome, often underestimated, resulting from several diseases and frequently associated with serum positivity for antineutrophil cytoplasmic antibodies (ANCA) or antiglomerular basement membrane (GBM) antibodies. The most common illness presenting as pulmonary-renal syndrome is systemic vasculitis. Moreover, the idiopathic pulmonary-renal syndrome is a distinctive clinicopathologic entity with different pathogenetic mechanisms. Tissue immunofluorescence studies are fundamental in distinguishing anti-GBM antibody-mediated forms from immune-complex-mediated and ANCA-associated forms. The type of glomerular or alveolar immunologic injury is the main factor determining the outcome and thus the prognosis of the pulmonary-renal syndrome. Development and improvement of appropriate serologic detection techniques have given reliable and early guidance for diagnosing these cases.

Anti-Glomerular Basement Membrane Disease↗

[Primary antiphospholipid syndrome evolving into systemic lupus erythematosus: a report of 3 new cases and a review of the literature].

The antiphospholipid syndrome (APS) is characterized by the presence of venous and arterial thrombosis, fetal losses, and thrombocytopenia, associated with the presence of antiphospholipid antibodies (aPL). This syndrome may be "primary" or associated with other diseases, particularly systemic lupus erythematosus (SLE). There is considerable debate on the interrelations between primary APS and SLE as well as on the value of the American College of Rheumatology (ACR) criteria to distinguish between both entities. Several authors have suggested that some patients with primary APS may ultimately develop SLE. Likewise, some authors have proposed exclusion criteria for primary APS. The presence of any of these criteria in a patient with the initial diagnosis of APS might indicate progression of SLE. Three new cases are here reported in whom the diagnosis evolved into SLE some years after the initial diagnosis of primary APS was made. Similar cases previously reported in the literature are reviewed.

Adult↗

Anticardiolipin and anti-beta2GPI antibodies in a large series of European patients with systemic lupus erythematosus. Prevalence and clinical associations. European Concerted Action on the Immunogenetics of SLE.

OBJECTIVE: To test the prevalences and the clinical associations of anticardiolipin (aCL) and anti-beta2GPI (abeta2GPI) antibodies in a large series of European patients with systemic lupus erythematosus (SLE). METHODS: 574 SLE patients from 7 European countries were tested for aCL and abeta2GPI by ELISA methods. RESULTS: aCL of IgG, IgM, and IgA isotypes were detected in 22.8%, 14%, and 13.9% of the patients, respectively. IgG and IgM abeta2GPI were detected in 20% of the patients. The presence of aCL was highly associated with the presence of abeta2GPI. Medium-high titer IgG aCL and abeta2GPI were associated with thrombosis, with similar sensitivity, specificity, and positive predictive value. When present at medium-high titer, IgG aCL were associated with thrombocytopenia, IgM aCL with hemolytic anemia, and cerebrovascular accidents. IgA aCL with livedo reticularis and Raynaud's phenomenon. CONCLUSIONS: aCL, when present at medium-high titer, are as important as abeta2GPI, as a risk factor for thrombosis. Medium-high titer aCL, but not abeta2GPI, are associated with other clinical features of the antiphospholipid syndrome.

Adolescent↗

Determination of organochlorine pesticides in skins and leather by gas chromatography.

The simultaneous determination of residues of lindane (gamma-HCH) and 10 other organochlorine pesticides (OCPs) in skins and leather was carried out by gas chromatography (GC) with electron-capture detection (ECD). GC with mass spectrometric detection was used to identity confirmation. Samples were extracted with hexane. The extracts were concentrated, and cleaned up on a Florisil column. Dibromooctafluorobiphenyl was added as internal standard. Hide fortifications of 0.5 an d5.0 ppm yielded average lindane recoveries of 98% and 96%, respectively. OCPs was determined in 57 samples of skins purchased from American, European and African countries in 1996-1997. OCPs were not detected in any of the American and European samples. Residues of lindane were found in 56% of African samples.

Animals↗