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Anti-phospholipid syndrome

Joshua T Byers, Xin Qing, Christopher Lo, Samuel W French, Ping Ji
Amyloidosis is a disorder characterized by the deposition of insoluble abnormal proteins in the extracellular space. It may occur as a localized lesion or as a systemic disease involving multiple organs and systems. Localized conjunctival amyloidosis is rare and is less frequently associated with systemic involvement. Although amyloidosis itself is a benign lesion involvement of multiple organs and systems is associated with poor prognosis. Diagnosis of amyloidosis is made on biopsy specimens with Congo red staining for the appearance of apple-green birefringence under polarized light microscopy...
March 15, 2018: Experimental and Molecular Pathology
Sebastian Vernal, Maria Jose Franco Brochado, Roberto Bueno-Filho, Paulo Louzada-Junior, Ana Maria Roselino
INTRODUCTION: Corticosteroids and/or thalidomides have been associated with thromboembolism events (TBE) in multibacillary (MB) leprosy. This report aimed to determine genetic and laboratory profiles associated with leprosy and TBE. METHODS: Antiphospholipid antibodies (aPL), coagulation-related exams, prothrombin and Leiden's factor V mutations, and ß2-glycoprotein-I (ß2GPI) Val247Leu polymorphism were assessed. RESULTS: Six out of seven patients with leprosy were treated with prednisone and/or thalidomide during TBE and presented at least one positive aPL...
January 2018: Revista da Sociedade Brasileira de Medicina Tropical
Cecilia Beatrice Chighizola, Francesca Pregnolato, Laura Andreoli, Caterina Bodio, Laura Cesana, Chiara Comerio, Maria Gerosa, Claudia Grossi, Rajesh Kumar, Maria Grazia Lazzaroni, Michael Mahler, Elena Mattia, Cecilia Nalli, Gary L Norman, Maria Gabriella Raimondo, Amelia Ruffatti, Marta Tonello, Laura Trespidi, Angela Tincani, Maria Orietta Borghi, Pier Luigi Meroni
Antibodies against β2 glycoprotein I (anti-β2GPI) have been identified as the main pathogenic autoantibody subset in anti-phospholipid syndrome (APS); the most relevant epitope is a cryptic and conformation-dependent structure on β2GPI domain (D) 1. Anti-β2GPI domain profiling has been investigated in thrombotic APS, leading to the identification of antibodies targeting D1 as the main subpopulation. In contrast, scarce attention has been paid to obstetric APS, hence this study aimed at characterizing the domain reactivity with regards to pregnancy morbidity (PM)...
February 14, 2018: Journal of Autoimmunity
Cecilia Beatrice Chighizola, Laura Andreoli, Maria Gerosa, Angela Tincani, Amelia Ruffatti, Pier Luigi Meroni
Anti-phospholipid syndrome (APS) is an acquired pro-thrombotic autoimmune disease that predisposes to thrombotic events and/or obstetric complications, in the persistent presence of anti-phospholipid antibodies (aPL). Life long moderate-intensity anticoagulation is the option of choice for aPL-positive patients with a previous thrombosis; critical issues concern the management of those with a history of arterial event due to the high rate of recurrence. Alternatives comprise anti-platelet agents and high-intensity anticoagulation...
February 12, 2018: Journal of Autoimmunity
Quentin Scanvion, Sandrine Morell-Dubois, Cécile M Yelnik, Johana Bene, Sophie Gautier, Marc Lambert
No abstract text is available yet for this article.
January 24, 2018: Rheumatology
Virginie Dufrost, Jessie Risse, Denis Wahl, Stéphane Zuily
No abstract text is available yet for this article.
January 24, 2018: Rheumatology
Olga Oskolkova, Nicolene Sarich, Yufeng Tian, Grzegorz Gawlak, Fanyong Meng, Valery N Bochkov, Evgeny Berdyshev, Anna A Birukova, Konstantin G Birukov
Correction of barrier dysfunction and inflammation in acute lung injury (ALI) represents an important problem. Previous studies demonstrate barrier-protective and anti-inflammatory effects of bioactive lipid prostacyclin and its stable analog iloprost (ILO). We generated a phospholipase resistant synthetic phospholipid with iloprost attached at the sn-2 position (ILO-PC) and investigated its biological effects. In comparison to free ILO, ILO-PC caused sustained endothelial cell (EC) barrier enhancement, linked to more prolonged activation of Rap1 and Rac1 GTPases and their cytoskeletal and cell junction effectors: cortactin, PAK1, p120-catenin and VE-cadherin...
January 17, 2018: Scientific Reports
Savino Sciascia, Massimo Radin, Giovanni Sanna, Irene Cecchi, Dario Roccatello, Maria Laura Bertolaccini
Objective: Recently, our group conceived a risk score for clinical manifestations of APS (the global APS score, or GAPSS) that takes into account the combination of independent cardiovascular risk factors and the aPL positivity profile. These include hyperlipidaemia, arterial hypertension, aCL, anti-β2 glycoprotein-I, aPS-PT and the LA. A complementary version, the adjusted GAPSS (aGAPSS), which excludes aPS-PT, was also designed. The aim of our study was to systematically review the literature to assess the clinical utility of the GAPSS and aGAPSS for risk stratification of any APS clinical manifestation...
January 12, 2018: Rheumatology
Pavol Mikolka, Jana Kopincova, Petra Kosutova, Maros Kolomaznik, Andrea Calkovska, Daniela Mokra
AIM: Meconium aspiration syndrome (MAS) is life-threatening respiratory failure of newborns which can be treated by exogenous surfactant. In response to meconium, increased levels of chemokine IL-8 (CXCL8) stimulate massive neutrophil infiltration of the lungs. Local accumulation and activation of neutrophils, on-going inflammation, lung edema, and oxidative damage contribute to inactivation of endogenous and therapeutically given surfactants. Therefore, we have hypothesized that addition of monoclonal anti-IL-8 antibody into exogenous surfactant can mitigate the neutrophil-induced local injury and the secondary surfactant inactivation and may finally result in improvement of respiratory functions...
January 11, 2018: Experimental Lung Research
E Campello, C M Radu, M Tonello, A Kuzenko, C Bulato, A Hoxha, E Mattia, L Spiezia, A Ruffatti, P Simioni
No abstract text is available yet for this article.
January 8, 2018: Scandinavian Journal of Rheumatology
Sja Johnsen, M B Lauvsnes, R Omdal
No abstract text is available yet for this article.
January 3, 2018: Scandinavian Journal of Rheumatology
Songwang Hou, Heike Fölsch, Ke Ke, Joan Cook Mills, Rosalind Ramsey-Goldman, Ming Zhao
Phosphatidylethanolamine (PE) is a major phospholipid species with important roles in membrane trafficking and reorganization. Accumulating clinical data indicate that the presence of circulating antibodies against PE is positively correlated with the symptoms of antiphospholipid syndromes (APS), including thrombosis and repeated pregnancy loss. However, PE is generally sequestered inside a normal resting cell, and the mechanism by which circulating anti-PE antibodies access cellular PE remains unknown. The studies presented here were conducted with synthetic PE-binding agents, plasma samples from patients with anti-PE autoimmunity, and purified anti-PE antibodies...
December 26, 2017: Proceedings of the National Academy of Sciences of the United States of America
Raida Ben Salah, Yosra Cherif, Faten Frikha, Dammak Chifaa, Mouna Snoussi, Moez Jallouli, Sameh Marzouk, Mhiri Chokri, Zouhir Bahloul
Background: Multiple sclerosis and other demyelinating processes are sometimes difficult to differentiate from the neurological involvement in autoimmune diseases. Distinguishing multiple sclerosis from other lesions due to autoimmune diseases is crucial to avoid unsuitable or delayed treatments. Methods: Charts of 6 patients diagnosed with mimicking multiple sclerosis between 1996 and 2014 were retrospectively assessed. Results: The mean age at diagnosis was 35±7 years...
2017: Caspian Journal of Internal Medicine
Cecilia Beatrice Chighizola, Yehuda Shoenfeld, Pier Luigi Meroni
PROBLEM: The association of low molecular weight heparin (LMWH) with low-dose aspirin (LDASA) provides the therapeutic cornerstone of obstetric anti-phospholipid syndrome (APS). This combo approach is not effective in all patients, and few women still experience recurrences. METHOD OF STUDY: In an elegant in vitro study, Chiombori Quao and colleagues demonstrated that anti-phospholipid antibodies (aPL) affect the functionality of endometrial endothelial cells interfering with angiogenesis...
March 2018: American Journal of Reproductive Immunology: AJRI
William E Winter, Sherri D Flax, Neil S Harris
Primary hemostasis begins with endothelial injury. VWF, produced by endothelial cells, binds to platelets and links them to subendothelial collagen. Platelet-derived ADP and thromboxane activate non-adhered platelets via their GPIIb/IIIa receptors, allowing these platelets to participate in platelet aggregation. Secondary hemostasis is initiated with the binding of factor VII to extravascular tissue factor (TF). Factors II, VII, IX and X are vitamin K-dependent factors. The role of vitamin K is to assist in the addition of gamma carboxylate groups to glutamic acids in the "GLA" domains of these factors...
November 8, 2017: Laboratory Medicine
Kayoko Kaneko, Shuko Mishima, Mikako Goto, Mari Mitsui, Shinji Tanigaki, Kenji Oku, Nobuaki Ozawa, Eisuke Inoue, Tatsuya Atsumi, Haruhiko Sago, Atsuko Murashima
OBJECTIVE: To elucidate clinical feature and anti-phospholipid antibody (aPL) profiles, including lupus anticoagulant (LA), anti-cardiolipin (CL) antibodies and anti-phosphatidylserine/prothrombin (PS/PT) antibodies, of pregnancy failure in patients with antiphospholipid antibody syndrome (APS) already treated with conventional therapy. MATERIALS AND METHODS: Thirty-four women with a history of pregnancy who were diagnosed with APS between 2008 and 2016 were included in the study...
October 25, 2017: Modern Rheumatology
Chun-Yu Lin, Chun-Ching Chiu, Ju Cheng, Chia-Yun Lin, Ya-Fang Shi, Chun-Chou Tsai, Bor-Show Tzang, Tsai-Ching Hsu
Mounting evidence suggests a connection between human parvovirus B19 (B19) and autoimmune diseases, and especially an association between the B19-VP1 unique region (VP1u) and anti-phospholipid syndrome (APS). However, little is known about the antigenicity of B19-VP1u in the induction of APS-like syndrome. To elucidate the antigenicity of B19-VP1u in the induction of APS, N-terminal truncated B19-VP1u (tVP1u) proteins were prepared to immunize Balb/c mice to generate antibodies against B19-tVP1u proteins. The secreted phospholipase A2 (sPLA2) activities and binding specificity of mice anti-B19-tVP1u antibodies with cardiolipin (CL) and beta-2-glycoprotein I (β2GPI) were evaluated by performing immunoblot, ELISA and absorption experiments...
January 1, 2018: Virulence
Masashi Deguchi, Hideto Yamada, Mayumi Sugiura-Ogasawara, Mamoru Morikawa, Daisuke Fujita, Akinori Miki, Shintaro Makino, Atsuko Murashima
The aim of this study was to understand the clinical features of antiphospholipid syndrome (APS)-complicated pregnancies and evaluate risk factors for the adverse pregnancy outcomes. This multicenter study evaluated live-birth rates according to therapy modality for APS and risk factors of pregnancy loss in 81 pregnancies. Risk factors for pregnancy complications, including premature delivery before 34 gestational weeks, hypertensive disorders of pregnancy, thrombocytopenia, and light-for-date neonate, were evaluated in 51 women who received low dose aspirin (LDA) plus unfractionated heparin (UFH) and delivered after 24 GW...
August 2017: Journal of Reproductive Immunology
Jean Amiral, Marie Peyrafitte, Claire Dunois, Anne Marie Vissac, Jerard Seghatchian
Anti-phospholipid syndrome is a complex and severe clinical situation, associated with symptoms such as recurrent thrombosis, arterial or venous, at any site, pregnancy loss, and other related syndromes. These clinical burdens, are highly variable from patient to patient, and are associated with biological abnormalities, such as the presence of the Lupus Anticoagulant or phospholipid dependent antibodies, confirmed on two occasions at least 12 weeks apart. From the diagnosis standpoint, both, functional (clotting) or immunological assays, are difficult to standardize and to optimize, due to the absence of reference material, or a characteristic clinical group, and international reference preparations...
July 15, 2017: Transfusion and Apheresis Science
Ahmadou M Jingi, Liliane Mfeukeu-Kuate, Aurel T Tankeu, Narcisse Assene Ateba, Edvine Wawo Yonta, Jean Jacques Noubiap
BACKGROUND: Anti-phospholipid syndrome (APLS) is a condition characterized by the presence of raised plasma levels of anti-phospholipid antibodies associated with thrombo-embolic disease and/or poor obstetrical outcomes in women. The epidemiology of APLS is unknown in most sub-Saharan African countries due to limited access to diagnosis tools. We report the case of APLS in a 29-year-old obese woman that was preceded by pre-eclampsia and fetal death. The diagnosis of APLS was made during a thrombo-embolic episode 4 years after the poor obstetrical outcome...
August 8, 2017: BMC Research Notes
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