[Ocular manifestations in the antiphospholipid syndrome].
Explore the source record for details and available documents.
Antiphospholipid Syndrome↗
Biomedical subjects
Y Levy
Publications and source records attributed to Y Levy.
Environmental factors may modulate antiphospholipid antibody production in family members of patients with systemic lupus erythematosus.
Explore the source record for details and available documents.
Adolescent↗
Survival without surgical repair of acute rupture of the right ventricular free wall.
Rupture of the myocardial free wall is an infrequent complication of acute myocardial infarction. Unless it occurs in a space confined by pericardial adhesions, only surgical emergency repair of ruptured myocardium can prevent death. In this paper we report the case of an 81-year-old woman who was admitted to the emergency room with cardiac tamponade, resulting from inferolateral acute myocardial infarction and a subsequent rupture of the right ventricular free wall, with the formation of pericardial thrombus and effusion. The patient refused to undergo any surgical or invasive intervention, and therefore she was only treated conservatively. Nevertheless, her condition improved dramatically, as her blood pressure increased and echocardiography abnormalities almost disappeared. Follow-up echocardiography 7 months post discharge was unremarkable. We believe that this rare case emphasizes that in special circumstances, such as creation of a thrombus that prevents more blood from extravasating, free-wall rupture without surgical repair is compatible with long-term survival.
Aged↗
Reactivity of peripheral blood lymphocytes to oxidized low-density lipoprotein: a novel system to estimate atherosclerosis employing the Cellscan.
BACKGROUND: The assumption that atherosclerosis involves an autoimmune response to oxidized LDL (oxLDL) is based on the presence of immunocompetent cells and immunoglobulin deposition in the atherosclerotic lesions by successful immunomodulation of the atherosclerotic process and by inhibition of experimental atherosclerosis by antioxidants. The Cellscan system is a multiparameter laser-based static cytometer that enables repeated monitoring of the fluorescence intensity (FI) and polarization (FP) of individual living cells. Analysis of intracellular fluorescein fluorescence polarization (IFFP) has previously been used to define activated lymphocyte population. HYPOTHESIS: In this study, the Cellscan apparatus has been used to monitor cellular response to oxLDL in patients with atherosclerosis and in controls. METHODS: The FI and FP of fluorescein diacetate (FDA)-labeled peripheral lymphocytes were measured following exposure to oxLDL in vitro. Using cluster analysis we were able to identify subpopulations of cells that were characterized by their FI and FP. Forty-two subjects were studied: 22 patients with severe coronary heart disease and 22 control individuals, either healthy or with other diseases. RESULTS: Fluorescence intensity of fluorescein-labeled peripheral blood lymphocytes (PBL) was markedly decreased upon exposure to high doses (> 25 micrograms/ml) of oxLDL concurrently with an increase in FP. A specific and dose-dependent reduction in FP of the high-intensity cell subpopulations, accompanied by higher FI, was evident in patients with ischemic heart disease upon exposure to low doses of oxLDL (up to 25 micrograms/ml). Maximal depolarization was shown upon triggering with 2 micrograms/ml oxLDL. The polarization ratio (the mean polarization value of the specific cell population with and without activation) obtained for patients' lymphocytes was significantly lower (p < 0.01) than that of the control group (0.936 +/- 0.05 and 1.028 +/- 0.055, respectively). CONCLUSION: These data suggest that PBL from patients with active ischemic heart disease show an increased reactivity to oxLDL. A 73% positivity rate was found for ischemic heart disease patients compared with 5% in the control subjects. One of the future prospects of this study might be the advent of a simple and rapid noninvasive test that could assess the extent of atherosclerosis, and possibly even the response to therapy, by monitoring the reactivity of PBL to oxLDL.
Aged↗
Anaphylactoid reaction to imiglucerase, but not to alglucerase, in a type I Gaucher patient.
Imiglucerase, the recombinantly produced enzyme, is gradually replacing the human placental derived alglucerase in the treatment of gaucher patients. We describe the first case, to the best of our knowledge, of an anaphylactoid reaction to imiglucerase in a patient who tolerated alglucerase. The patient was diagnosed at the age of 2 4/12 years with anemia and hepatosplenomegaly. Over the years he had suffered from marked splenomegaly, thrombocytopenia and recurrent bleeding episodes. At the age of 24 he started treatment with imiglucerase. After 3 months of treatment, immediately after starting an infusion, he experienced flushing, cough, tachycardia, palpitation, chest pain and excessive sweating, which reoccurred on a consecutive administration. Substitution with alglucerase was tolerated well, with only mild rash when he was premedicated with benadryl. Immediate skin tests to alglucerase, imiglucerase and gelatin were negative. IgG against alglucerase was undetectable. The in vitro mast cell degranulation test was positive for alglucerase, imiglucerase heamaccel (a gelatin based plasma substitute, which is a component of imiglucerase). This sensitivity to imiglucerase but not to alglucerase, raises the question of future treatment for this patient, since the production of alglucerase may cease, once imiglucerase production will cover the need for replacement enzyme.
Adult↗
In vivo administration of intravenous immunoglobulin (IVIg) can lead to enhanced erythrocyte sequestration.
Enhanced erythrocyte sequestration is one of the very few major adverse effects of intravenous immunoglobulin (IVIg). IVIg contains high molecular weight IgG complexes ( approximately 300 kDa) which, in the presence of serum, mimic immune complexes by activating complement, binding to CR1 of red blood cells (RBC) (CD35) and mediating erythrophagocytosis. Four of seven patients undergoing IVIg therapy showed significant drops in haematocrit and haemoglobin that were not due to isoantibodies in the IVIg. Prior to treatment, patients' RBC carried IgG and complement (C') 3d that were not bound as immune complexes via CR1 (CD35). The patients whose RBC bound immune complex-like moieties and showed drops in haematocrit and haemoglobin subsequent to IVIg were young adults (22-35 years); older patients (50-69 years) showed no ill effects. In the presence of complement, RBC of young patients bound IVIg complexes in vitro while those of older patients did not. It is not the absolute levels of erythrocyte-associated IgG or C'3 fragments, neither pre- nor post-therapy, which are predictive of IVIg associated decreases in haematocrit and haemoglobin levels. Patient age and RBC inability to bind the IVIg immune complex-like moieties in vitro both appear to be predictors of resistance to sequestration after in vivo treatment with IVIg.
Adult↗
Successful treatment of systemic lupus erythematosus cerebritis with intravenous immunoglobulin.
Neuropsychiatric lupus includes extremely diverse clinical manifestations, ranging from mild cognitive dysfunction to a severe, life-threatening presentation. We report a 28-year-old patient with systemic lupus erythematosus who had persistent fever for 3 months, and developed within a few hours motor and sensory aphasia, rotator nystagmus with deviation of the eyes, and severe nuchal rigidity. An extensive series of imaging and laboratory tests were interpreted as normal, except for an elevated opening pressure at lumbar puncture, cerebrospinal fluid inflammatory findings, and asymmetrical cortical perfusion on single-photon emission computed tomography. The patient received one course of high-dose intravenous immunoglobulin (IVIg) and within 5 days her condition returned to that of 3 months before admission. The mechanisms of injury, along with the management of cerebral lupus and the mechanisms of action of IVIg, are discussed.
Adult↗
Marked improvement of severe cardiac dysfunction after one course of intravenous immunoglobulin in a patient with systemic lupus erythematosus.
Intravenous immunoglobulin (IVIg) is currently used with much enthusiasm for the treatment of many autoimmune diseases, including systemic lupus erythematosus (SLE). Among its various indications, IVIg has also been found to be beneficial in myocarditis, whether or not it is associated with an autoimmune disease (e.g. Kawasaki's disease). We report a 59-year-old SLE patient who, while being treated with steroids, developed severe cardiac dysfunction with a left ventricular ejection fraction of 20%. Coronary angiography demonstrating normal coronary arteries supported the diagnosis of myocarditis. High-dose IVIg treatment was started, followed by improved cardiac function a few days later and normalisation of the ejection fraction (50%) 1 month later. This is the second report of a beneficial effect of IVIg in myocarditis secondary to SLE.
Female↗
Myelodysplastic syndrome and associated skin lesions: a review of the literature.
The skin involvement of the myelodysplastic syndrome (MDS) can take the form of either a neoplastic infiltration or various non specific lesions. The occurrence of these lesions may be the presenting feature of the disease (MDS) or may herald its progression to acute leukemia. Recognition and early diagnosis have therapeutic and prognostic significance.
Adolescent↗
Tono-Pen versus Goldmann tonometry after excimer laser photorefractive keratectomy.
PURPOSE: To compare intraocular pressure (IOP) measurements by the Tono-Pen 2 tonometer and Goldmann applanation tonometer (GAT) in post-photorefractive keratectomy (PRK) patients. SETTING: Refractive Surgery Center, Assaf Harofeh Medical Center, Zerifin, Israel. METHODS: In 18 patients, IOP was measured by GAT and then by Tono-Pen 2 tonometer 2 to 18 months following PRK (mean 6.6 months +/- 5.1 [SD]). Photorefractive keratectomy had been performed in 1 eye of each patient; the fellow eyes served as controls. Corneal curvature and thickness were evaluated. Ten of the 18 patients were treated with topical steroids. RESULTS: In the post-PRK eyes, mean GAT IOP was 1.8 +/- 3.1 mm Hg lower than mean Tono-Pen IOP (P = .012); there was no significant IOP difference in the control (fellow) eyes. In steroid-treated post-PRK eyes, mean GAT IOP (12.2 mm Hg) was 2.2 +/- 1.3 mm Hg lower than mean Tono-Pen IOP (14.4 mm Hg) (P = .0007). Mean Tono-Pen IOP in steroid-treated post-PRK eyes was 4.3 +/- 3 mm Hg higher than in the fellow eyes (P = .0014); mean GAT IOP was only 2.3 +/- 3.5 mm Hg higher (P = .04). In post-PRK eyes without topical steroid treatment, mean GAT IOP was 2.0 +/- 1.18 mm Hg lower than in the fellow eyes (P = .001); there was no significant difference in Tono-Pen IOP. There was a negative correlation between the difference in IOP values (Tono-Pen minus GAT) and corneal curvature in post-PRK eyes (r = 0.76, P = .0108, n = 15). CONCLUSIONS: The Tono-Pen tonometer appeared to be less affected than the GAT by the relative flattening, thinning, and anterior stromal decreased rigidity of the central cornea that occur following PRK. Post-PRK steroid-induced IOP elevation may be masked by the artifactual decrease in GAT IOP.
Adult↗
Early metalinguistic competence: speech monitoring and repair behavior.
This article examines 2-3-year-olds' responses to specific (e.g., "Where did he go?") and neutral (e.g., "What?") requests for clarification. The focus is on children's ability to locate the linguistic errors that provoked neutral questions of clarification and their success in providing appropriate repair. It is argued that this behavior provides evidence for a speech monitor that detects errors and enables repair. Contrary to A. Karmiloff-Smith's (1992) claim, control over the production and comprehension of specific structures does not necessarily precede monitoring. Rather, metalinguistic abilities that are implicated in speech monitoring develop simultaneously with the acquisition of primary linguistic behavior and do not require awareness. It is claimed that such metaprocesses are fundamental to the use of language as a vehicle for the expression of intentional content.
Child Language↗
The effect of anthocyanosides in a multiple oral dose on night vision.
PURPOSE: In view of research demonstrating the ability of anthocyanosides in a multiple oral dose to improve night vision in normal individuals, we assessed their effect on three night vision tests: full-field absolute scotopic retinal threshold (SRT), dark adaptation rate (DAR) and mesopic contrast sensitivity (MCS). METHODS: In a double-masked, placebo-controlled, cross-over study, 18 young normal volunteers were randomly assigned to one of three different regimens of multiple oral administrations of 12 and 24 mg anthocyanosides, and a placebo, given twice daily for 4 days. A 2 week washout period was allowed between each 4 day treatment period. SRT, DAR and MCS was tested 1 day before and at days 1, 2, 3 and 4 during the treatment period. RESULTS: No significant effect was found on any of the three above-mentioned night vision tests. The study had a power of 0.95 to detect a 0.1 log unit improvement in SRT and 0.5 log unit improvement in MCS. CONCLUSIONS: Multiple oral administrations of 12 and 24 mg anthocyanosides twice a day appear to lack significant effect on night vision tests.
Adult↗
Atheroma: links with antiphospholipid antibodies, Hughes syndrome and lupus.
Antiphospholipid antibodies (aPL) are found in a variety of autoimmune diseases, and are thought to predispose to arterial and venous thrombosis. These antibodies, when investigated in different assays in vitro, activate endothelial cells and promote uptake of modified LDL to macrophages. These observations suggest that aPL can contribute to atheroma development by targeting some of the sequential steps that constitute early atherogenesis. If substantiated by large-scale clinical trials, the pro-atherogenic properties of aPL may merit screening and intervention programs in selected populations.
Antibodies, Antiphospholipid↗
Marked improvement of Churg-Strauss vasculitis with intravenous gammaglobulins.
Churg-Strauss vasculitis (CSV) is a systemic vasculitis usually treated with steroids and cytotoxic drugs. Herein, we describe the beneficial effect of intravenous immune globulin (IVIG) in a 50-year-old man with CSV. The IVIG therapy brought a marked clinical improvement, with impressive decline in the titers of antineutrophil cytoplasmic autoantibody.
Antibodies, Antineutrophil Cytoplasmic↗
Anti-endothelial cell antibodies in systemic sclerosis.
Explore the source record for details and available documents.
Autoantibodies↗
Serologic and clinical response to treatment of systemic vasculitis and associated autoimmune disease with intravenous immunoglobulin.
BACKGROUND: Autoimmune vasculitides cannot always be controlled by steroids and immunosuppressive drugs. Intravenous immunoglobulin (IVIg) treatment was found beneficial in several vasculitides including systemic and organ-specific diseases. In this article we tested whether the beneficial clinical response of IVIg treatment in vasculitides was accompanied by a decrease in vasculitis-associated autoantibody levels. METHODS: Ten patients diagnosed as having vasculitis were treated with high-dose (2 g/kg) IVIg monthly, in a 5-day schedule. In all the patients, other therapeutic measures failed to control disease progression prior to IVIg treatment. Each patient received between 1 and 6 treatment courses. All patients were evaluated for the levels of 5 autoantibodies (Abs) related to vasculitis before and after each treatment course. RESULTS: In 6 out of the 10 patients, a beneficial clinical response followed IVIg treatment. Moreover, no treatment-related adverse effects were observed in any of the patients. Anti-myeloperoxidase antibodies and cytoplasmic-antineutrophil cytoplasmic antibodies levels decreased concomitantly with the clinical improvement observed in the patients with Churg-Strauss vasculitis and Wegener's granulomatosis, respectively. Levels of cytoplasmic-antineutrophil cytoplasmic antibodies (ANCA) with specificity for bacteridial/permeability-increasing protein and human lysosomal-associated membrane protein increased after each treatment course, but returned to normal values before the following one. CONCLUSIONS: When other therapeutic measures, such as immunosuppressive therapy, fails to control disease manifestations in patients with vasculitides, IVIg is a possible effective intervention method with a high response rate. IVIg probably exerted its effects on disease progression via different mechanisms. Among these mechanisms, a decrease in relevant Ab levels is often found (probably by anti-idiotypes in IVIg), and thus ANCA levels are expected to associate with disease activity.
Adult↗
Enhancement of atherosclerosis in beta-2-glycoprotein I-immunized apolipoprotein E-deficient mice.
We have previously shown that low density lipoprotein receptor-deficient (LDL-RD) mice immunized with beta2-glycoprotein I (beta2GPI; a target of autoimmune anticardiolipin antibodies) developed enhanced early atherosclerosis, when fed a normal chow diet. The current study was undertaken to evaluate the effect of immunization with beta2GPI and the addition of a high fat diet on the progression of atherosclerosis in the apolipoprotein E (ApoE)-deficient mouse. Six-week-old female ApoE-deficient mice (n = 10) were immunized subcutaneously with either human beta2GPI or with ovalbumin, both emulsified in complete Freund's adjuvant and fed a high fat diet for 6 weeks. The beta2GPI-immunized mice were found to develop accelerated atherosclerosis when compared with their ovalbumin-immunized littermates (aortic lesion area of 137,500 +/- 13,801 vs. 72,444 +/- 14,465 microm2, respectively; p = 0.0067). The beta2GPI-immunized mice developed high titers of anti-beta2GPI antibodies, 10 days after the procedure, which were sustained until the sacrifice. LDL extracted from both study groups displayed similar susceptibility to ex vivo oxidation. These results confirm our previous study in which we found increased atherosclerosis in beta2GPI-immunized LDL-RD mice fed a chow diet. In the current study we show that the proatherogenic effect of beta2GPI immunization is maintained despite high cholesterol levels and is not associated with increased susceptibility of LDL to ex vivo oxidation.
Animals↗
A study of 20 SLE patients with intravenous immunoglobulin--clinical and serologic response.
OBJECTIVE: To test the clinical response of systemic lupus erythematosus (SLE) patients to intravenous immunoglobulins (IVIg), and whether the clinical response of IVIg treatment in SLE is accompanied by modification of SLE-associated autoantibodies/antibodies (Abs) and complement levels. METHODS: Twenty SLE patients were treated with high-dose (2 g/kg) IVIg monthly, in a 5-d schedule. Each patient received between 1-8 treatment courses. They were evaluated for the clinical response, Systemic Lupus Activity Measure (SLAM) score before and after IVIg, levels of antinuclear antibody (ANA), dsDNA (double-stranded DNA), SS-A or SS-B, ENA (extractable nuclear antigens), C3 and C4 levels before and after the treatment, and before and after each treatment course. RESULTS: A beneficial clinical response following IVIg treatment was noted in 17 out of 20 patients (85%). Few clinical manifestations responded more to treatment: arthritis, fever, thrombocytopenia, and neuropsychiatric lupus. In 9 patients evaluated before and after IVIg, mean SLAM score decreased from 19. 3+/-4.7 to 4+/-2.9 (P<0.0001). There was a tendency towards abnormal levels of complement and Abs before IVIg courses among the treatment responders compared with the non-responders, and similarly the former tended to have normalization of their abnormal levels more than the latter. These differences were found statistically significant only with respect to C4 and SS-A or SS-B levels before IVIg courses. CONCLUSION: IVIg has a high response rate among SLE patients. A combination of clinical manifestations, Abs and complement levels may aid in the future in predicting who among SLE patients will benefit more from IVIg treatment.
Adult↗