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Antiphospholipid syndrome

Carlos Perez-Sánchez, Iván Arias-de la Rosa, María Ángeles Aguirre, María Luque-Tévar, Patricia Ruiz-Limón, Nuria Barbarroja, Yolanda Jiménez-Gómez, María Carmen Ábalos-Aguilera, Eduardo Collantes-Estévez, Pedro Segui, Francisco Velasco, María Teresa Herranz, Jesús Lozano-Herrero, María Julia Hernandez-Vidal, Constantino Martínez, Rocío González-Conejero, Massimo Radin, Savino Sciascia, Irene Cecchi, María José Cuadrado, Chary López-Pedrera
The present study aimed at identifying the plasma miRNA profile of antiphospholipid syndrome patients and to investigate the potential role of specific circulating miRNAs as non-invasive disease biomarkers. Ninety antiphospholipid syndrome patients and 42 healthy donors were recruited. Profiling of miRNAs by PCR-array in plasma of antiphospholipid syndrome patients allowed identifying a set of miRNAs differentially expressed and collectively involved in clinical features. Logistic Regression and ROC-curve analyses identified a signature of 10 miRNA ratios as biomarkers of disease...
March 15, 2018: Haematologica
Tatsuya Seki, Yasushige Shingu, Hiroshi Sugiki, Satoru Wakasa, Hiroki Katoh, Tomonori Ooka, Tsuyoshi Tachibana, Suguru Kubota, Yoshiro Matsui
Antiphospholipid syndrome (APS) is a complex autoimmune disease often related to systemic lupus erythematosus. Although adequate anticoagulation is important for APS patients during cardiopulmonary bypass, clotting tests can be potentially misleading due to antiphospholipid antibodies. We performed cardiac surgery safely in two APS patients under anticoagulation monitoring determined using preoperative heparin titration. We performed heparin titration for activated clotting time to determine the appropriate heparin concentration during cardiac surgery...
March 14, 2018: Journal of Artificial Organs: the Official Journal of the Japanese Society for Artificial Organs
Ilaria Cavazzana, Laura Andreoli, Maarteen Limper, Franco Franceschini, Angela Tincani
PURPOSE OF REVIEW: This review focuses on new clinical aspects of antiphospholipid syndrome (APS) in the last 5 years. RECENT FINDINGS: The pathogenesis of APS is related to endothelial activation by mechanisms other than autoantibody-mediated massive coagulation. These include Toll-like receptors, the m-TORC pathway, and neutrophil activation, inducing an uncontrolled inflammatory cascade. Given these new pathogenetic hypotheses, the treatment of APS could be directed towards a fine balance between anticoagulation and immunomodulation...
March 15, 2018: Current Rheumatology Reports
F Popescu, C R Jaslow, W H Kutteh
STUDY QUESTION: Will the addition of 24-chromosome microarray analysis on miscarriage tissue combined with the standard American Society for Reproductive Medicine (ASRM) evaluation for recurrent miscarriage explain most losses? SUMMARY ANSWER: Over 90% of patients with recurrent pregnancy loss (RPL) will have a probable or definitive cause identified when combining genetic testing on miscarriage tissue with the standard ASRM evaluation for recurrent miscarriage...
March 12, 2018: Human Reproduction
Dileep Unnikrishnan, Nasreen Shaikh, Ahmad Sharayah, Chandler Patton
A 47-year-old woman with a medical history of Raynaud's phenomenon presented with fever, cough and shortness of breath. She was found to have left lower lobe consolidation and pleural effusion and was treated as a case of pneumonia. During the hospital course, her respiratory status worsened, and she was intubated on the third hospital day. To investigate the high A-a gradient, a Computerized Tomographic Pulmonary Embolism (CTPE) study was done which identified a large left lower pulmonary artery embolism. She was also found to have a new murmur, and an echocardiogram demonstrated a large lesion on tricuspid valve...
March 13, 2018: BMJ Case Reports
K M J Devreese, T L Ortel, V Pengo, B de Laat
No abstract text is available yet for this article.
February 14, 2018: Journal of Thrombosis and Haemostasis: JTH
Luis J Jara, Gabriela Medina, Miguel A Saavedra
PURPOSE OF REVIEW: To provide an update about the interactions between infections and autoimmune diseases (AIDs), from the molecular perspective to the clinical spectrum and the differentiation between infection and disease activity. RECENT FINDINGS: Any kind of infection may modify the innate and adaptive immune response through the following mechanisms: molecular mimicry, superantigens, epitope spreading and B-cell activation. The consequence is the overproduction of antibodies shared with those found in AIDs...
March 9, 2018: Current Opinion in Rheumatology
M E Rodríguez-Almaraz, I Herraiz, P I Gómez-Arriaga, P Vallejo, E Gonzalo-Gil, A Usategui, E A López-Jiménez, A Galindo, M Galindo
OBJECTIVE: To evaluate the usefulness of the uterine artery mean pulsatility index (mPI-UtA) and the sFlt-1/PlGF ratio in women with systemic lupus erythematosus (SLE) or antiphospholipid syndrome (APS) for the prediction of placental dysfunction-related adverse outcomes (AO), namely pre-eclampsia (PE) and intrauterine growth restriction (IUGR), and for differential diagnosis between PE and SLE flares. STUDY DESIGN: Observational prospective cohort study of 57 pregnant women with SLE or APS...
January 2018: Pregnancy Hypertension
Nada Abdulaziz, Anjali R Shah, William J McCune
PURPOSE OF REVIEW: Antimalarial drugs including chloroquine, its less toxic quinolone-derivative hydroxychloroquine (HCQ), and quinacrine have become cornerstones in the treatment of autoimmune diseases including systemic lupus, rheumatoid arthritis, sarcoidosis, and Sjogren syndrome; cutaneous disorders, antiphospholipid syndrome, and have recently been employed at higher dioses in oncology. Benefits include anti-inflammatory effects, protection against thrombosis, and improved control of hyperglycemia and hyperlipidemia...
March 6, 2018: Current Opinion in Rheumatology
Sadaaki Horiguchi, Hisako Ono, Hiroyuki Shirato, Toshimitsu Kawakami, Shiho Yabuki, Naomi Morita, Nozomu Shirasugi
In our varicose vein center, on a trial basis, among the patients with asymptomatic calf deep vein thrombosis (CDVT) we carefully selected the patients for varicose vein surgery using the requirements as follows; 1) the patients had varicose veins with incompetent saphenous veins, 2) sequential examination including DUS confirmed stability and clinical insignificance of asymptomatic CDVT, 3) the patients do not have any risk factors for DVT such as a coagulation profile disorder (antithrombin deficiency, protein C deficiency, protein S deficiency, or antiphospholipid syndrome) or malignancies, 4) surgery is possible under local anesthesia alone, and 5) the patients can understand the concept of asymptomatic CDVT and undergo the surgery on their own will and informed consent...
December 25, 2017: Annals of Vascular Diseases
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
Chrisanna Dobrowolski, Doruk Erkan
Antiphospholipid syndrome (APS) is a systemic autoimmune disorder marked by thrombosis and/or pregnancy morbidity in the presence of antiphospholipid antibodies (aPL). At the present time, treatment is primarily focused on anticoagulation. However, there is increasing awareness of the mechanisms involved in APS pathogenesis, which has led to the trial of novel therapies targeting those mechanisms. Following a brief review of the etiopathogenesis of and current management strategies in APS, this paper focuses on the evidence for these potential, targeted APS treatments, e...
March 3, 2018: Clinical Immunology: the Official Journal of the Clinical Immunology Society
Ami Schattner, Shilo Voichanski, Livnat Uliel
A healthy 38-year-old woman developed sudden unilateral vision loss due to retrobulbar optic neuritis in the wake of varicella-zoster virus infection. She had no further central nervous system (CNS) lesions. Antinuclear antibodies (ANA) and anti-aquaporin 4 antibodies were found, consistent with neuromyelitis optica (NMO). Later, serial MRIs showed dynamic short-segment and long-segment myelitis lesions, ANA titre increased and additional autoantibodies were found including anti-dsDNA, anti-chromatin/nucleosome and antiphospholipid antibodies...
March 5, 2018: BMJ Case Reports
Anastasia Sacharidou, Ken L Chambliss, Victoria Ulrich, Jane E Salmon, Yu-Min Shen, Joachim Herz, David Y Hui, Lance S Terada, Philip W Shaul, Chieko Mineo
In the antiphospholipid syndrome (APS), antiphospholipid antibody (aPL) recognition of β2 glycoprotein (β2GPI) promotes thrombosis, and preclinical studies indicate that this is due to endothelial NO synthase (eNOS) antagonism via apoER2-dependent processes. How apoER2 molecularly links these events is unknown. Here we show that in endothelial cells the apoER2 cytoplasmic tail serves as a scaffold for aPL-induced assembly and activation of the heterotrimeric protein phosphatase PP2A. Dab2 recruitment to the apoER2 NPXY motif promotes the activating L309 methylation of the PP2A catalytic subunit by leucine methyl transferase-1...
March 2, 2018: Blood
Hiroyuki Nakamura, Kenji Oku, Yusuke Ogata, Kazumasa Ohmura, Yoko Yoshida, Etsuko Kitano, Yuichiro Fujieda, Masaru Kato, Toshiyuki Bohgaki, Olga Amengual, Shinsuke Yasuda, Yoshihiro Fujimura, Tsukasa Seya, Tatsuya Atsumi
INTRODUCTION: Although complement activation has been proposed as a possible thrombophilic mechanism in antiphospholipid syndrome (APS), the origin of complement activation in APS remains unclear. Here, we focused on complement regulatory factors (CRF), which control the complement system to prevent damage to host tissue. We evaluated the function of two major CRF, membrane cofactor protein (MCP) and factor H (FH), in APS patients. MATERIALS AND METHODS: In this study, we analyzed preserved serum samples from 27 patients with primary APS (PAPS), 20 with APS complicated with SLE (APS + SLE), 24 with SLE (SLE), and 25 with other connective tissue diseases (Other CTD)...
February 21, 2018: Thrombosis Research
Amelia Ruffatti, Marta Tonello, Ariela Hoxha, Savino Sciascia, Maria J Cuadrado, José O Latino, Sebastian Udry, Tatiana Reshetnyak, Nathalie Costedoat-Chalumeau, Nathalie Morel, Luca Marozio, Angela Tincani, Laura Andreoli, Ewa Haladyj, Pier L Meroni, Maria Gerosa, Jaume Alijotas-Reig, Sara Tenti, Karoline Mayer-Pickel, Michal J Simchen, Maria T Bertero, Sara De Carolis, Véronique Ramoni, Arsène Mekinian, Elvira Grandone, Aldo Maina, Fátima Serrano, Vittorio Pengo, Munther A Khamashta
The effect of additional treatments combined with conventional therapy on pregnancy outcomes was examined in high-risk primary antiphospholipid syndrome (PAPS) patients to identify the most effective treatment strategy. The study's inclusion criteria were (1) positivity to lupus anticoagulant alone or associated with anticardiolipin and/or anti-β2 glycoprotein I antibodies; (2) a history of severe maternal-foetal complications (Group I) or a history of one or more pregnancies refractory to conventional therapy leading to unexplained foetal deaths not associated with severe maternal-foetal complications (Group II)...
February 28, 2018: Thrombosis and Haemostasis
Ayumi Koga, Ken Okamoto, Hirokazu Tazume, Toshihiro Fukui
Antiphospholipid syndrome is a rare autoimmune disease with a hypercoagulable state causing vascular thrombosis. We present the case of a 42-year old female who underwent mitral valve replacement with a mechanical valve 15 months ago. The postoperative course was uneventful, and echocardiography performed 14 months postoperatively showed good valve function. The patient developed sudden dyspnea 15 months postoperatively and was referred to our hospital. Echocardiography revealed mitral stenosis with stuck leaflets...
January 31, 2018: Heart Surgery Forum
Gökhan Özgür, Cengiz Beyan
Catastrophic thrombotic syndrome is characterized by occurrence of several thromboembolic occlusions which affect a variety of vascular beds over a short period of time. Catastrophic Antiphospholipid syndrome (CAPS) (1%) is regarded as a rare but severe variant of Antiphospholipid Syndrome. Treatment with anticoagulants, steroids and therapeutic plasma exchange has been shown to give the best results. However, to treat CAPS, the best substitute for plasma is still a debatable issue because there are no standard methods...
February 20, 2018: Transfusion and Apheresis Science
Yu Zuo, Jennifer Fan, Ravi Sarode, Song Zhang, Una E Makris, David R Karp, Yu-Min Shen
The evaluation of thrombotic and pregnancy risks associated with antiphospholipid antibodies (aPL)in individual patients without antiphospholipid syndrome (APS) clinical manifestation is challenging. Our aim is to identify additional risk factors or potential candidate "second hits" for APS clinical events in a large cohort of ethnically diverse aPL-positive patients. We included 219 consecutive aPL-positive patients who attended clinic at our institution. All patients had at least 1 persistent high titer (≥99 percentiles) aPL...
January 1, 2018: Clinical and Applied Thrombosis/hemostasis
Loukman Omarjee, Vincent Jaquinandi, Audrey Camarzana, Frederic Rouleau, Tin-Hinan Mezdad, Thierry Le Tourneau, Guillaume Mahe, Cristina Belizna
No abstract text is available yet for this article.
February 22, 2018: Circulation Journal: Official Journal of the Japanese Circulation Society
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