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J Ordi-Ros

Publications and source records attributed to J Ordi-Ros.

33 records · Page 2Linked to original sources
PubMed1995

Adult onset of nemaline myopathy presenting as respiratory insufficiency.

A 49-year-old woman was admitted to the hospital for hypercapnia. Pulmonary function testing showed small lung volumes without parenchymal lung disease. Muscle enzyme levels were normal and the EMG was nonspecific. Finally, muscle biopsy revealed abundant nemaline bodies characteristic of nemaline myopathy. Nasal intermittent pressure ventilation was started with a preset pressure ventilator during sleeping hours with a good response.

Female↗
PubMed1994

Comparison of the primary and secondary antiphospholipid syndrome: a European Multicenter Study of 114 patients.

PURPOSE: To determine whether the features of the antiphospholipid syndrome (APS) are in any way influenced by the presence or absence of systemic lupus erythematosus (SLE). We followed up patients with 'primary' APS (PAPS) and APS secondary to SLE (APS plus SLE) with the objective of comparing laboratory and clinical events and of determining whether patients with PAPS would have evolution to SLE. PATIENTS AND METHODS: A total of 114 patients from 3 European referral centers were included in this study. Fifty-six had APS plus SLE and 58 had PAPS. Laboratory and clinical data were collected during an average 2-year period. RESULTS: Patients with PAPS and patients with APS plus SLE had similar clinical and laboratory profiles, with the exceptions of autoimmune hemolytic anemia, endocardial valve disease, neutropenia, and low C4 levels, all of which occurred more frequently in patients with APS plus SLE (p values: < 0.05, < 0.005, < 0.01, and < 0.001, respectively). On follow-up, 10 thrombotic episodes occurred in 10 patients, 8 of whom were receiving anticoagulant therapy. No patient with PAPS had either anti-DNA or anti-extractable nuclear antigen antibodies, and these patients had a significantly lower prevalence of antinuclear antibodies (41%) than patients with APS plus SLE (89%). CONCLUSIONS: Patients with APS plus SLE and PAPS have similar clinical profiles, although heart valve disease, hemolytic anemia, low C4 levels, and neutropenia seem to be more common in patients with APS plus SLE. Patients with APS may develop further thrombotic events despite anticoagulation therapy.

Adult↗
PubMed1994

Prevalence, significance, and specificity of antibodies to phospholipids in Q fever.

A cross-sectional study of the prevalence, significance, and specificity of antibodies to phospholipids (aPL) in patients with Q fever was undertaken in a university-based tertiary care medical center. The results of the lupus anticoagulant (LA) test, VDRL test, fluorescent treponemal antibody absorption test, and ELISA with different phospholipid antigens were determined for 26 patients with Q fever diagnosed by clinical and serological criteria. Plasma from four patients with Q fever and antibodies to cardiolipin (aCL) was purified by affinity chromatography in a cardiolipin column and tested against all phospholipids. For 17 patients with high levels of aCL, a modified ELISA without fetal calf serum was performed to determine if the serum cofactor was needed for a CL activity. Fisher's exact test was used for the statistical analysis. From the results of our study we conclude the following: patients with Q fever have a high incidence of aPL, with techniques with aCL or LA being the most sensitive to determine aPL (these antibodies can help diagnose Q fever presenting only as a fever); antibodies (phase II) to Coxiella burnetii and aCL are different antibodies; and the aCL activity in patients with Q fever is cofactor-independent.

Adolescent↗
PubMed1994

Antibodies against platelet-activating factor in patients with antiphospholipid antibodies.

We have studied the specificity of antiphospholipid antibodies in 148 patients with autoimmune diseases, 120 patients with systemic lupus erythematosus and 28 with the primary antiphospholipid syndrome. In addition, 20 patients suffering from syphilis were studied. As a control group, 64 healthy volunteers were investigated. Patient and control serum samples were tested for binding to seven different phospholipid antigens by ELISA. Interestingly, 90% of the sera from syphilis patients and 6% of the autoimmune patients exhibited a significant binding to platelet-activating factor (PAF), a molecule similar to the structure of phosphatidylcholine. In addition, the IgG fraction from one of the lupus patients, which showed a high binding activity to PAF, was further affinity-purified using both liposomes and an affinity chromatography column. Preincubation of these antibodies with PAF inhibited subsequent binding to immobilized PAF. These observations might suggest a putative interaction of antiphospholipid autoantibodies with PAF 'in vivo', which may have, in some patients, important pathophysiological consequences.

Antibodies, Antiphospholipid↗
PubMed1992

Transverse myelitis in systemic lupus erythematosus: two cases with magnetic resonance imaging.

Transverse myelitis is one of the most serious neurological complications occurring in the course of systemic lupus erythematosus. We describe two lupus patients, with transverse myelitis, one of whom had associated optic neuritis. In both, magnetic resonance imaging of the spinal cord showed an abnormal signal. In one case a good response to steroid and immunosuppressive therapy was observed; the other case failed to improve despite the therapy applied.

Adrenal Cortex Hormones↗
PubMed1990

Raynaud's phenomenon and positive antinuclear antibodies in a malignancy.

Both Raynaud's phenomenon and the presence of antinuclear antibodies are uncommon features of malignant disease and the association of both with a malignancy extremely rare. The case is reported of a 78 year old woman who presented with Raynaud's phenomenon and positive antinuclear antibodies related to adenocarcinoma of unknown primary site.

Adenocarcinoma↗
PubMed1989

The "primary" antiphospholipid syndrome: major clinical and serological features.

The antiphospholipid syndrome--the association of venous and/or arterial thromboses, often accompanied by thrombocytopenia in the presence of the antiphospholipid antibodies ("lupus anticoagulant" antibodies to cardiolipin)--is seen mainly in patients with systemic lupus erythematosus (SLE) and the closely related "lupus-like" disease, i.e., lupus patients not conforming to the 1982 revised American Rheumatism Association classification for SLE. It is also seen in a group of patients who do not manifest any of the major clinical or serologic features of SLE, the majority of whom do not appear to progress to classical lupus. A multicenter study of 70 of these patients is documented and their major clinical and serologic characteristics examined: They have been characterized as suffering from a "primary" antiphospholipid syndrome and present typically with a history of deep vein thromboses, often accompanied by pulmonary thromboembolism, which in a few is complicated by thromboembolic pulmonary hypertension, arterial occlusions (most commonly strokes), or fetal loss. The events are often recurrent and may be accompanied by hemocytopenias (thrombocytopenia and less frequently Coombs positivity and/or hemolytic anemia). They are often antinuclear antibody-negative and are always negative for antibodies to dsDNA and to ENA, typical serologic features of SLE. There may be a family history of SLE or a familial clotting tendency in a minority. The group of patients presented appears to be closely related, but distinctly separate from SLE.

Adult↗
PubMedDate not supplied

Clinical and therapeutic aspects associated to phospholipid binding antibodies (lupus anticoagulant and anticardiolipin antibodies).

Antiphospholipid antibodies (APA) comprise a family of immunoglobulins characterized by their pattern of reactivity in a number of laboratory tests. Included in this family are lupus anticoagulant (LA) anticardiolipin antibodies (ACA) and antibodies causing biologic false positive serologic tests for syphilis (BFP-STS). LA and ACA occur in a variety of conditions, including other autoimmunes disorders, infectious diseases, neoplasic disorders, in association with certain drugs and in otherwise healthy individuals. Clinical interest in LA and ACA is increasing. Antiphospholipid antibody syndrome is characterized by a triad of clinical features which include fetal loss, thromboembolic disease and thrombocytopenia. Other clinical manifestations related with APA are livedo reticularis, cutaneous necrosis, hemolytic anemia, heart valve disease, chorea, migraine and obstetric problems as fetal growth retardation, pre-eclampsia, post-partum serositis or neonatal thrombosis or catastrophic antiphospholipid syndrome. Therapy is mainly directed against the widespread and diverse manifestations associated with the obstruction of small and large vessels. Long-term treatment with oral anticoagulation therapy is advised, even if the venous or arterial occlusion occurred many years previously. In patients with primary antiphospholipid syndrome there is no evidence that the prophylactic administration of steroids or immunosuppression will prevent thromboembolic events. Although the administration of more energetic immunosuppression with cyclophosphamide in pulse form is effective in reducing elevated antibody levels, there is usually a rapid rebound to pretreatment levels shortly after discontinuation of the therapy. A history of recurrent fetal loss requires mandatory treatment during pregnancy. Although the actual prospective risk of pregnancy loss in women with antiphospholipid syndrome and prior pregnancy loss is unknown, it may exceed 60%. Because of this many investigators have treated women with antiphospholipid syndrome with either antiplatelet agents, immunosuppressive agents, or anticoagulants in an attempt to improve pregnancy outcome. Unfortunately, there is no unequivocal proof that any of these therapies are fully efficacious. Despite varying treatment protocols, the live birth rate with treatment was 70%, similar to that reported in the recent randomized clinical trial. Thrombocytopenia and autoimmune hemolytic anemia in patients with APA are treated similarly as patients without APA. Treatment of asymptomatic patients isn't indicated, because only approximately 10-15% of patients with APA developed complications.

Abortion, Habitual↗