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https://www.readbyqxmd.com/read/28072955/-clinical-analysis-of-12-patients-with-pediatric-antiphospholipid-syndrome-with-pulmonary-embolism
#1
J R Ma, H M Song, J Xiao, X Y Tang, Y Y He, M Wei
Objective: To identify the clinical and immunological characteristics of pediatric antiphospholipid syndrome (APS) patients with pulmonary embolism. Method: Among 47 pediatric APS patients from Peking Union Medical College Hospital during the year of 2000 to 2015, 12 patients were diagnosed of pulmonary embolism, who were investigated and compared with APS patients without pulmonary embolism. Result: Twelve patients (among whom 6 cases were primary and the other 6 were secondary APS)had pulmonary embolism and all of them were non-shock type, which was the first presenting manifestation in 6 of them...
January 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28065614/cerebral-venous-sinus-thrombosis-presenting-feature-of-systemic-lupus-erythematosus
#2
Rajesh Kumar Singh, Sanjeev Kumar Bhoi, Jayantee Kalita, Usha Kant Misra
BACKGROUND: CVST (cerebral venous sinus thrombosis) may sometimes be associated with autoimmune disorders that require specific treatment. The clinical and magnetic resonance imaging (MRI) findings of systemic lupus erythematosus (SLE) patients with CVST are presented and contrasted with CVST without SLE. METHODS: Consecutive patients with CVST admitted in neurology service during 2012-2016 were included. The diagnosis of CVST was confirmed by MR venography or digital subtraction angiography...
January 5, 2017: Journal of Stroke and Cerebrovascular Diseases: the Official Journal of National Stroke Association
https://www.readbyqxmd.com/read/28063132/evaluation-of-role-of-fv-fviii-and-aplas-in-the-pathogenesis-of-apcr-in-fv-leiden-negative-dvt-patients-a-study-in-india
#3
Amit Sharma, Kanwaljeet Singh, Arijit Biswas, Ravi Ranjan, Kamal Kishor, Ravi Kumar, Hareram Pandey, Vineet Kumar Kamal, Renu Saxena
Resistance to APC (APCR) is a very important cause of thrombophilia and most frequently caused by the Leiden mutation. APCR is also seen in the absence of FV Leiden and associated with elevated levels of factor V (FV), factor VIII (FVIII) and antiphospholipid antibodies (APLAs). The aim of this prospective case control study was to find out the frequency and role of FV, FVIII and APLAs in the pathogenesis of APCR in FV Leiden negative deep vein thrombosis (DVT) patients in India. A total 30 APCR positive and FV Leiden negative patients with DVT and similar number of age and sex matched healthy controls were recruited...
January 6, 2017: Journal of Thrombosis and Thrombolysis
https://www.readbyqxmd.com/read/28050648/diagnostic-value-of-antibodies-to-phosphatidylserine-prothrombin-complex-for-antiphospholipid-syndrome-in-chinese-patients
#4
Lei Zhu, Chun Li, Na Liu, Xin Yang, R L Jia, Rong Mu, Yin Su, Z G Li
To evaluate the diagnosis value of antibodies to phosphatidylserine/prothrombin complex (aPS/PT) in patients with antiphospholipid syndrome (APS) and to determine the clinical features of APS patients with avidity of aPS/PT. Serum samples were collected from 108 APS patients. Sixty patients with pregnancy morbidity, 37 patients with thrombosis without a history of autoimmune diseases, and 89 healthy blood donors were included as the control group. The enzyme-linked immunosorbent assay (ELISA) test was performed to detect the concentration of aPS/PT, including IgG/M, IgG, and IgM forms, in the same serum sample...
January 3, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28050066/antiphospholipid-syndrome-a-diagnostic-challenge
#5
REVIEW
R S Mallhi, Neerja Kushwaha, T Chatterjee, J Philip
The antiphospholipid syndrome (APS) is an acquired autoimmune thrombophilic disorder that is characterized by thrombosis (venous, arterial and microvascular) and obstetric morbidity due to a diverse family of antibodies against phospholipid-binding proteins present in plasma. The term antiphospholipid antibody is actually a misnomer as the antibodies are not against the phospholipid per se, but target the plasma protein co-factors, which bind to anionic PLs. The exact etiology has not been elucidated and is multifactorial...
December 2016: Medical Journal, Armed Forces India
https://www.readbyqxmd.com/read/28028988/direct-oral-anticoagulants-in-patients-with-thrombophilia-challenges-in-diagnostic-evaluation-and-treatment
#6
REVIEW
Anetta Undas, Tadeusz Góralczyk
Direct oral anticoagulants (DOACs) or non-vitamin K oral anticoagulants (NOACs) are increasingly used in the prevention of recurrent venous thromboembolism (VTE), including that associated with thrombophilia. The efficacy of DOACs in thrombophilic patients, especially those with severe trombophilia or triple positive antiphospholipid syndrome (APS) with arterial thromboembolic events, remains controversial. Most case reports and case series indicate that DOACs are an attractive therapeutic option in the vast majority of these patients at high risk of recurrent VTE with more concerns raised in high-risk APS patients and these deficient in protein S (PS)...
November 2016: Advances in Clinical and Experimental Medicine: Official Organ Wroclaw Medical University
https://www.readbyqxmd.com/read/28009646/lupus-anticoagulant-hypoprothrombinemia-syndrome-a-rare-cause-of-intracranial-bleeding
#7
Sreejesh Sreedharanunni, Jasmina Ahluwalia, Narender Kumar, Sunil K Bose, Rishi Dhawan, Pankaj Malhotra
We report a 14-year-old girl who presented with subdural hematoma and a deranged coagulation profile suggestive of an inhibitor. Investigations revealed prothrombin deficiency along with positivity for antiphospholipid antibodies, which improved with steroid therapy. Bleeding diathesis in children and adolescents commonly results from thrombocytopenia, platelet function disorders, or coagulation factor deficiency; whereas bleeding because of coagulation factor inhibitors are extremely rare in this age group...
December 22, 2016: Blood Coagulation & Fibrinolysis: An International Journal in Haemostasis and Thrombosis
https://www.readbyqxmd.com/read/28005581/retinal-deep-capillary-plexus-ischemia-in-a-case-with-antiphospholipid-syndrome
#8
Serra Arf, Isil Sayman Muslubas, Mumin Hocaoglu, Murat Karacorlu
PURPOSE: To report visualization of a case of retinal deep capillary plexus ischemia with antiphospholipid syndrome. METHODS: A 53-year-old woman was referred with a 1-week history of sudden onset of decreased vision in the right eye. Her symptoms were evaluated by clinical examination, infrared reflectance, fundus autofluorescence, fluorescein angiography, spectral-domain optical coherence tomography, microperimetry, and multifocal electroretinography. RESULTS: The patient's visual acuity on presentation was 0...
September 30, 2016: Retinal Cases & Brief Reports
https://www.readbyqxmd.com/read/28002027/coexistence-of-anti-%C3%AE-2-glycoprotein-i-domain-i-and-anti-phosphatidylserine-prothrombin-antibodies-suggests-strong-thrombotic-risk
#9
Jee-Soo Lee, JaYoon Gu, Hee Sue Park, Hyun Ju Yoo, Hyun Kyung Kim
BACKGROUND: Highly specific assays for measuring antiphospholipid antibodies (aPLs) are required for accurate assessment of thrombotic risk. aPLs against β2-glycoprotein I domain I (anti-β2GPIdI) and against prothrombin complexed with phosphatidylserine (anti-PS/PT) have been recently identified as being associated with a hypercoagulable state. This study evaluated the synergism between anti-β2GPIdI and anti-PS/PT for predicting thrombotic events. METHODS: A total of 180 patients with clinical suspicion of hypercoagulability were evaluated...
December 21, 2016: Clinical Chemistry and Laboratory Medicine: CCLM
https://www.readbyqxmd.com/read/27989533/the-use-of-direct-oral-anticoagulants-in-56-patients-with-antiphospholipid-syndrome
#10
Katarzyna Malec, Tadeusz Góralczyk, Anetta Undas
INTRODUCTION: Antiphospholipid syndrome (APS) is a common acquired thrombophilia associated with a high thrombotic risk, in which vitamin K antagonists (VKA) represent the mainstay of therapy. Case series involving up to 35 patients with APS suggested limited efficacy and safety of direct oral anticoagulants (DOACs). MATERIAL AND METHODS: In the prospective case series we followed 56 consecutive patients with APS (44 women and 12 men, aged from 22 to 64years), including 33 (60%) associated with systemic lupus erythematosus (SLE) and 16 (28...
December 14, 2016: Thrombosis Research
https://www.readbyqxmd.com/read/27988438/detection-of-lupus-anticoagulant-in-the-era-of-direct-oral-anticoagulants
#11
REVIEW
Ariela Hoxha, Alessandra Banzato, Amelia Ruffatti, Vittorio Pengo
Lupus anticoagulant (LAC) is an in vitro phenomenon determining a phospholipid-dependent elongation of clotting times. The presence of LAC associated with anticardiolipin (aCL) and anti-β2 glycoprotein I (anti-β2GPI) antibodies is strongly associated with thrombosis and pregnancy morbidity. Direct oral anticoagulants (DOACs) targeting thrombin and factor Xa are currently widely use to prevent and treat venous and arterial thromboembolism. Some concern has, however, been expressed about the possibility of false laboratory results during LAC assessment in patients taking these drugs...
December 15, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27981815/the-role-of-clinically-significant-antiphospholipid-antibodies-in-systemic-lupus-erythematosus
#12
M Taraborelli, M G Lazzaroni, N Martinazzi, M Fredi, I Cavazzana, F Franceschini, A Tincani
The objective is to investigate the role of clinically significant antiphospholipid antibodies (aPL) in a cohort of systemic lupus erythematosus (SLE) patients. All SLE patients followed for at least 5 years and with available aPL profile at the beginning of the follow-up in our center were studied. Clinically significant aPL were defined as: positive lupus anticoagulant test, anti-cardiolipin and/or anti- β2Glycoprotein I IgG/IgM >99th percentile on two or more occasions at least 12 weeks apart. Patients with and without clinically significant aPL were compared by univariate (Chi square or Fisher's exact test for categorical variables and Student's t or Mann-Whitney test for continuous variables) and multivariate analysis (logistic regression analysis)...
December 16, 2016: Reumatismo
https://www.readbyqxmd.com/read/27913748/a-3-year-follow-up-of-a-patient-with-acute-renal-failure-caused-by-thrombotic-microangiopathy-related-to-antiphospholipid-syndrome-case-report
#13
X-J Zhou, M Chen, S-X Wang, F-D Zhou, M-H Zhao
BACKGROUND: Microvascular manifestations of antiphospholipid antibody syndrome in the kidneys include acute renal failure, thrombotic microangiopathy and hypertension. Therapy has been largely empiric. CASE REPORT: A 49-year-old Chinese man presented with anuric acute renal failure without abundant proteinuria and heavy haematuria, but markedly low levels of urinary sodium, potassium and chlorine upon admission. On day 1 of hospitalization, his thrombocytopenia, anaemia and renal failure showed rapid progression...
December 2, 2016: Lupus
https://www.readbyqxmd.com/read/27913565/a-24-year-old-male-with-a-painful-and-cold-lower-extremity
#14
Eric A Robinson, Zain I Khalpey, Rajesh Janardhanan
CLINICAL INTRODUCTION: A 24-year-old male presented to the emergency department with intense pain in his right lower extremity. He has a medical history significant for systemic lupus erythematosus and antiphospholipid syndrome. He also had four prior episodes of deep venous thromboses on rivaroxaban. The patient stated that early in the morning, he started to feel intense pain that started from his knee and progressed to his calf, with associated numbness and paraesthesia. On physical examination, the limb felt cold with absent right popliteal and dorsalis pedis pulses...
December 2, 2016: Heart: Official Journal of the British Cardiac Society
https://www.readbyqxmd.com/read/27913551/what-is-the-role-of-hydroxychloroquine-in-reducing-thrombotic-risk-in-patients-with-antiphospholipid-antibodies
#15
Tzu-Fei Wang, Wendy Lim
A 35-year-old man presents with an acute unprovoked deep vein thrombosis of the left lower extremity. He is treated with anticoagulation and elects to discontinue treatment after 6 months. He subsequently develops polyarthralgias, fatigue, and a malar rash, and a diagnosis of systemic lupus erythematosus is made based on laboratory and clinical findings. Additional laboratory testing reveals persistent triple positive antiphospholipid antibodies, including lupus anticoagulant, high titer anticardiolipin antibodies, and anti-β2-glycoprotein I antibodies...
December 2, 2016: Hematology—the Education Program of the American Society of Hematology
https://www.readbyqxmd.com/read/27903029/-lupus-erythematosus-update-2016
#16
Martin Aringer, Reinhard Edmund Voll
Meanwhile, five years have passed since the approval of the anti-BAFF antibody belimumab as a first biological for SLE, but no further SLE drug candidate is even close to approval. There are still no clinical trial data available for the use of new oral anticoagulants in antiphospholipid syndrome. In spite of convincing evidence for the use of mycophenolate mofetil (MMF) in lupus nephritis, the German "Gemeinsame Bundesausschuss" (GBA) has not yet decided on its reimbursement. However, several of the ongoing clinical trials have potential to lead to important advances in SLE treatment in the future...
November 2016: Deutsche Medizinische Wochenschrift
https://www.readbyqxmd.com/read/27881058/relationship-between-systemic-lupus-erythematosus-activity-and-persistent-positive-antiphospholipid-antibodies
#17
Zhaleh Shariati Sarabi, Maryam Sahebari, Ali Etemad Rezaie, Mohammadtaghi Norouzi, Kamila Hashemzadeh
Systemic Lupus Erythematosus (SLE) is an autoimmune disorder, characterized by producing different auto-antibodies and multiorgan involvements. In this study we aimed to investigate the relationship between SLE activity and persistently positive antiphospholipid antibodies. Fifty-nine lupus patients (55 women and 4 men) who were assessed in two consecutive visits with 6 weeks interval were selected. Patients` clinical and laboratory data and serum antiphospholipid antibodies` values, were collected. Serum anticardiolipin antibodies and lupus anticoagulant were measured in two visits...
November 22, 2016: Current Rheumatology Reviews
https://www.readbyqxmd.com/read/27866289/echocardiographic-findings-in-asymptomatic-systemic-lupus-erythematosus-patients
#18
Abdel GaffarA Mohammed, Abdulaziz A Alghamdi, Mohammad A ALjahlan, Ibrahim A Al-Homood
The aim of this study is to use transthoracic echocardiographic (TTE) imaging methods to identify cardiac dysfunction in asymptomatic systemic lupus erythematosus (SLE) patients and to determine the association between echocardiographic findings and serology. This is a prospective cross-sectional study where 50 patients with confirmed diagnoses of SLE were recruited from rheumatology outpatient clinics. Clinical and serological evaluation to confirm the diagnosis of lupus was done in all patients. Fifty SLE patients, 46 (92%) females and 4 (8%) males, were recruited...
November 19, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27862892/lupus-anticoagulant-hypoprothrombinemia-syndrome-associated-with-severe-thrombocytopenia-in-a-child
#19
Aimee Foord, Nicole Baca, David Buchbinder, Arash Mahajerin
Lupus anticoagulant hypoprothrombinemia syndrome (LAHPS) comprises lupus anticoagulant, acquired hypoprothrombinemia, and often mild thrombocytopenia or normal platelets. It is usually associated with autoimmunity or postviral illness. We describe a case of a 10-year-old boy with oral bleeding and severe thrombocytopenia initially suggestive of immune thrombocytopenia. Secondary to bleeding, evaluation demonstrated prolonged coagulation tests and subsequently revealed the presence of lupus anticoagulant and hypoprothrombinemia, along with marked autoimmunity, suggestive of LAHPS...
November 15, 2016: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/27861383/living-donor-renal-transplantation-in-patients-with-antiphospholipid-syndrome-a-case-report
#20
Ji Yoon Choi, Joo Hee Jung, Sung Shin, Young Hoon Kim, Duck Jong Han
INTRODUCTION: Antiphospholipid syndrome (APS), autoantibodies directed against phospholipid-binding proteins are associated with cause vascular thrombosis. Patients with APS requiring renal transplantation are at risk of early graft loss due to arterial or venous thrombosis, or thrombotic microangiopathy (TMA). Here, we report 3 cases of successful renal transplantation in patients with APS. CLINICAL FINDINGS: A 53-year-old man with end-stage renal disease (ESRD) had experienced bilateral deep venous thrombosis (DVT) in the lower extremities 16 years ago and was administered warfarin...
November 2016: Medicine (Baltimore)
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