The antiphospholipid antibodies present in antiphospholipid syndrome (APS) are directed at a number of phospholipid-binding proteins: b 2 glycoprotein I (b 2 GPI), prothrombin, and so on. Antibodies directed at b 2 GPI are accepted as a classification criterion for APS, while the presence of antiprothrombin antibodies is not. In the present article, we investigated the possible role of antiphosphatidylserine/prothrombin antibodies (aPS/PT) as marker of APS on a cohort of 295 individuals with APS (95 primary APS and 45 secondary APS) and APS-related diseases. We found aPS/PT to be highly associated with venous thrombosis (immunoglobulin G [IgG] aPS/ PT odds ratio [OR], 7.44; 95% confidence interval [CI], 3.97-13.92 and IgM aPS/PT OR, 2.54; 95% CI, 1.35-4.77) and obstetric abnormalities (IgG aPS/PT OR, 2.37; 95% CI, 1.04-5.43), but not with arterial thrombosis. A very high degree of concordance between the concentration of aPS/PT and lupus anticoagulant activity was demonstrated. Therefore, we support the inclusion of aPS/PT determination as second-level assay to confirm APS classification.
The aim of this study was to determine if the measurement of anti-beta2-glycoprotein I antibodies (abeta2-GPI) in serum levels contributes to the better characterization of the clinical situation of patients with antiphospholipid syndrome (APS). For this purpose abeta2-GPI of both isotypes was measured in 42 patients with APS and 32 SLE patients without APS. Clinical records of all patients were thoroughly reviewed. The presence of abeta2-GPI was correlated with the clinical manifestations of APS and compared with the presence of anticardiolipin antibodies (aCL) and lupus anticoagulant (LA) activity. There was a positive correlation between levels of aCL and abeta2-GPI for both IgG and IgM isotypes (rho of Spearman=0.82 and 0. 64 respectively, P=0.0001). Both antibodies presented significantly higher titres in LA positive patients (P<0.05). The specificity for APS was 91% for IgG abeta2-GPI vs 75% for IgG aCL and 87% for IgM abeta2-GPI vs 81% for IgM aCL. 68% of patients with thrombosis of 100% of patients with thrombocytopenia showed positive tests for all three markers (aCL, LA, abeta2-GPI). Simultaneous presence of circulating LA and high titres of both aCL and abeta2-GPI identify a subset of patients with primary APS (PAPS) who have a more severe clinical course of the disease. Although the specificity of abeta2-GPI IgG is higher than that of aCL IgG, when all three tests are performed abeta2-GPI testing provides only additional information to that of aCL and LA. Therefore, we concluded that the abeta2-GPI test should not be considered as a substitute for conventional LA or aCL assays. However, performance of abeta2-GPI seems to be important in PAPS with high aCL titres, to alert the physician about the risk for the worst course of the illness.
Mitral valve prolapse (MVP) patients develop myocardial fibrosis that is not solely explained by volume overload, but the pathophysiology has not been defined. Mineralocorticoid receptor antagonists (MRAs) improve cardiac function by decreasing cardiac fibrosis in other heart diseases. We examined the role of MRA in myocardial fibrosis associated with myxomatous degeneration of the mitral valve. Myocardial fibrosis has been analyzed in a mouse model of mitral valve myxomatous degeneration generated by pharmacological treatment with Nordexfenfluramine (NDF) in the presence of the MRA spironolactone. In vitro, adult human cardiac fibroblasts were treated with NDF and spironolactone. In an experimental mouse, MRA treatment reduced interstitial/perivascular fibrosis and collagen type I deposition. MRA administration blunted NDF-induced cardiac expression of vimentin and the profibrotic molecules galectin-3/cardiotrophin-1. In parallel, MRA blocked the increase in cardiac non-fibrillar proteins such as fibronectin, aggrecan, decorin, lumican and syndecan-4. The following effects are blocked by MRA: in vitro, in adult human cardiac fibroblasts, NDF-treatment-induced myofibroblast activation, collagen type I and proteoglycans secretion. Our findings demonstrate, for the first time, the contribution of the mineralocorticoid receptor (MR) to the development of myocardial fibrosis associated with mitral valve myxomatous degeneration. MRA could be a therapeutic approach to reduce myocardial fibrosis associated with MVP.
The objective of this study was to determine: (1) the diagnostic value of antiperinuclear factor (APF), (2) the types of immunoglobulins involved in the reaction and (3) the presence of the antibody in paired samples of serum and synovial fluid (SF). We studied 408 serum samples from the following: healthy controls (n = 68), patients with rheumatoid arthritis RA; n = 160, 106 RF-positive and 54 RF-negative and patients with other rheumatic diseases (n = 180). We examined paired serum and SF samples in 27 patients (8 with RA and 19 with other rheumatic conditions). APF was determined by an indirect immunofluorescence assay. A group of 30 APF-positive serum samples was incubated with fluorescent-labelled antisera against IgG, IgM and IgA independently. APF was positive in 55.7% of patients with RF-positive RA, in 35.2% of patients with RF-negative RA, in 11.1% of patients with other rheumatic diseases and in 5.9% of healthy controls. Statistical differences were found between RF-positive RA and the other three groups (P = 0.02, P = 0.0001, P = 0.0001, respectively) and between RF-negative RA and the groups of other rheumatic diseases (P = 0.0001) and healthy controls (P = 0.005). The specificity of the test for RA was 90.2%. APF was present in three SF samples from RA patients (37.5%). The reaction was mediated by immunoglobulins of the IgG class in 100% of those tested, and, in addition, 30% were of IgA and 6.7% of IgM classes. We concluded that APF is a good diagnostic test that could be included in the classification criteria of RA, it can be present in SF and it is predominantly an antibody of the IgG class.
The renal vein thrombosis represents an etiologic and therapeutic problem in neonatal pathology. In our milieu RVT is more frequent than is reported in current bibliography. This high incidence is accompanied by a high mortality and by the development of chronic renal failure among survivors.This fact led us to try a thrombolytic treatment that could be effective with respect to life and renal function.In our study we describe seven newborn patients with radiologically checked RVT. All cases were secondary to hypernatremic dehydration syndrome due to hyperconcentrated feeding. Five patients were submitted to thrombolytic treatment with UK, one patient was treated with heparin and one patient died before any treatment could be tried.Among the five patients treated with UK, four showed a complete recanalization of the RVT, with recovery of their renal function without sequelae. One of the patients, in whom an intracraneal haemorrhage was clinically suspected, died during treatment.The dosage of UK during treatment was 1000 U/Kg/hour.The duration of treatment was individualized according to renal evolution.The description of treated cases will be done individually.
Background: inherited thrombophilias cause venous thrombotic events, however, their association with brain ischemia in adult patients is controversial. Our objective was to study the association between thrombophilia and cryptogenic stroke in patients under 55 years of age. Methods: prospective observational study of consecutive patients under 55 years of age who had had a brain ischemia (transient ischemic attack of brain infarction). The patients with cryptogenic brain ischemia were compared with the controls patients with brain ischemia of known cause. We examined the presence of thrombophilia (Factor V Leiden and prothrombin G20210A gene mutations; deficiencies in protein S, protein C and antithrombin levels; resistance to activated protein C) and patent foramen ovale (PFO) in all patients. Results: Two hundred fifty-four patients were included, 108 with cryptogenic brain ischemia and 146 controls patients with brain ischemia of known cause. Patients with cryptogenic brain ischemia were younger (mean age 42.4 vs. 45.6 years old, P=0.002). The frequency of thrombophilia was significantly higher among patients with cryptogenic brain ischemia than those with brain ischemia of known cause (22.2% vs. 6.8%, P<0.001). Taking into account each thrombophilic disorder separately, prothrombin G20210A mutation and protein C or S deficiency were significantly higher in the cryptogenic brain ischemia group than in the known cause group (10.2% vs. 2.7% and 8.3% vs. 2.1%, respectively, P<0.05) while Factor V Leiden mutation was similar in both groups (4.6% vs. 2.7%, P NS). The frequency of PFO and PFO plus thrombophilia were higher among patients with cryptogenic brain ischemia (35.2% vs. 12.3% and 8.4% vs. 0%, respectively, P<0.001). The PFO (+) cryptogenic brain ischemia patients showed higher frequency of thrombophilia than the other patients (23.7% vs. 11.6%, P=0.043), in particular prothrombin G20210A mutation (15.8% vs. 4.2%, P=0.014). Multivariate analysis adjusted confounding factors showed than the presence of thrombophilia was independently associated with cryptogenic brain ischemia (OR 3.9; 95% CI, 1.69 - 8.97; P=0.001). Conclusion: there is an association between thrombophilia and cryptogenic brain ichemia in patients under 55 years old. These data suggest that systemic thrombophilic disorders are cause of thromboembolic phenomena in brain arteries
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
hi@scite.ai
10624 S. Eastern Ave., Ste. A-614
Henderson, NV 89052, USA
Copyright © 2024 scite LLC. All rights reserved.
Made with 💙 for researchers
Part of the Research Solutions Family.