Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

---

Query: EC:3.4.21.6 (thromboplastin)
13,278 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Special challenges exist in the management of patients with von Willebrand disease (VWD) because of limitations in diagnostic facilities and therapeutic options. However, even within these limitations, it is possible to establish comprehensive services for this condition. Our data show that among 202 patients with VWD, 107 were type 3, 62 were type 1, and the others different categories of type 2. Basic tests such as bleeding time and activated partial thromboplastin time with factor (F)VIII coagulant are able to diagnose most of those with severe disease. We have been able to adapt the specific tests such as von Willebrand factor (VWF) ristocetin cofactor and VWF antigen from the tedious batched manual methods to cost-effective automated methods on advanced coagulometers. Discriminatory tests such as VWF collagen binding, VWF:FVIIIB, ristocetin-induced platelet agglutination (RIPA) are done in batches. Therapeutic options and for the treatment of bleeding include desmopressin, cryoprecipitate, and intermediate purity VWF-containing clotting factor concentrates. Tranexamic acid is also widely used as well as hormonal therapy for menorrhagia. We have also shown that modest doses of intermediate purity FVIII (Koate DVI; Talecris Biotherapeutics, Raleigh, NC, USA) at 35 IU/kg preoperatively and 10 to 20 IU/kg after that are sufficient for surgical hemostasis in these patients.
...
PMID:Diagnosis and management of von Willebrand disease: a developing country perspective. 2210 4

Blood dose not normally coagulate in the blood vessels covered with endothelial cells, because these cells contain some substances responsible for antithrombotic action such as thrombomodulin, heparin-like substance, prostacyclin, nitric oxide and tissue plasminogen activator. Most important role of blood coagulation is hemostasis. Blood can coagulate in two ways: intrinsic coagulation pathway and extrinsic coagulation pathway that is activated by negatively charged substances and FVIIa-tissue-factor (TF) complex, respectively. Prothrombin time(PT) can represent extrinsic pathway, while activated partial thromboplastin time (APTT) can represent intrinsic pathway. PT is prolonged in such diseases as vitamin K deficiency, hepatic failure and warfarin intake, while APTT is prolonged such diseases as hemophilia A & B, von Willebrand disease and lupus anticoagulant. Cross mixing test is very useful to assess prolonged clotting time. FDP means fibrin/fibrinogen degradation products and D-dimer is the smallest products of fibrin degradation. These markers are often used to diagnose disseminated intravascular coagulation (DIC) and deep vein thrombosis (DVT). Thrombin-antithrombin complex (TAT) and plasmin-alpha2 plasmin inhibitor (PIC) can be used to evaluate the extent of coagulation and fibrinolysis activation, respectively. These two markers is essential for classify the pathophysiology of DIC: DIC with suppressed fibrinolysis, enhanced fibrinolysis or balanced fibrinolysis. In conclusion, exact interpretation of hemostatic and fibrinolytic markers is one of the most important abilities in clinical situation.
...
PMID:[Interpretation of hemostatic and fibrinolytic markers]. 2218 80

Bleeding disorders commonly result from deficiencies or defects in von Willebrand factor (VWF), platelets, coagulation factors, or fibrinolytic proteins. The primary goal of our study was to assess current North American coagulation laboratory practices for diagnosing bleeding disorders, using an on-line patterns-of-practice survey of diagnostic laboratory members of the North American Specialized Coagulation Laboratory Association. The survey examined laboratory approaches to evaluating bleeding disorders, with specific questions about the tests and test panels offered and compliance to recent guideline recommendations on diagnosing von Willebrand disease (VWD) and platelet function disorders. All laboratories responding to the survey performed a prothrombin time/international normalized ratio, an activated partial thromboplastin time, and coagulation factor assays, and many tested for VWD and platelet disorders. However, few laboratories had test panels that evaluated the more common bleeding disorders and few performed some assays, including VWF multimer assessments and assays for fibrinolytic disorders. Additionally, the cutoffs used by laboratories to diagnose type 1 VWD varied considerably, with only a minority following the National Heart Lung Blood Institute recommendations. In contrast, laboratories that tested for platelet function disorders mostly complied with aggregation testing recommendations, as published in the recent North American guidelines. Our results indicate that there are some gaps in the strategies used by laboratories to diagnose bleeding disorders that might be addressed by development of further guidelines and test algorithms that emphasize evaluations for common bleeding disorders. Laboratories may also benefit from guidelines on test interpretation, and external evaluation of their bleeding disorder testing strategies.
...
PMID:Approaches to investigating common bleeding disorders: an evaluation of North American coagulation laboratory practices. 2236 23

Keeping an updated registry of bleeding disorders is crucial for planning care and documenting prevalence. We aimed to assess the prevalence of various bleeding disorders including rare inherited coagulation and platelet disorders concerning their clinico-epidemiological, diagnostic data and bleeding manifestations severity. Patients suffering from manifestations of bleeding or coagulation disorders presented to Hematology Clinic during 16 years were included and prospectively followed up. Demographics, clinical characteristics, complete blood count, bleeding, prothrombin and activated partial thromboplastin times, platelet aggregation tests and bone marrow aspiration were recorded. Overall 687 patients with bleeding disorders from total 2949 patients were identified. Inherited coagulation defects were found in 27.2%; hemophilia A (70.6%), hemophilia B (13.9%), factor I deficiency (2.3%), factor V deficiency (1.6%), factor X deficiency (4.2%), factor VII deficiency (2.6%), factor XIII deficiency (1.1%), combined factor deficiency (2.1%) and unclassified coagulation disorders in 1.6% of studied patients. Overall 72.7% had diagnosed with platelet disorders; immune thrombocytopenia was the commonest (74.8%), and inherited conditions represent (25.2%) in the following order: Glanzman's thrombasthenia (11.2%), von Willebrand disease (6.6%), Bernard-Soulier syndrome (1%) and Chediak Higashi in 0.4% and unclassified in 6%. Median age of diagnosis of coagulation and platelet disorders were 33 and 72 months. Presenting symptoms of coagulation disorders were: 25.1% post circumcision bleeding, 22.5% ecchymosis, 20.9% hemoarthrosis and 15% epistaxis. Symptoms of rare coagulation disorders were postcircumcision bleeding (20%), bleeding umbilical stump (20%), epistaxis (12%), hemoarthrosis (8%) and hematomas (4%). Presenting symptoms in rare inherited platelet disorders were purpura, ecchymosis, epistaxis and bleeding gums, respectively. Analysis of the clinico-epidemiological data of patients with bleeding disorders is a useful tool for monitoring and improving their quality of care.
...
PMID:A longitudinal prospective study of bleeding diathesis in Egyptian pediatric patients: single-center experience. 2261 Jan 36

The pathogenesis of venous and arterial thrombosis is complex and multifaceted, entailing a multitude of risk factors, which only marginally overlap between the two vessels districts. Along with conventional and universally recognized risk factors, thrombosis might also develop as a consequence of unusual, atypical, unsuspected and even paradoxical conditions. Although the term "paradoxical embolism" is typically used to identify an embolization process that originates from the low-pressure venous system and may generate ischemic stroke or peripheral arterial occlusion through a cardiac or pulmonary shunt, there are additional clinical circumstances whereby the risk of thrombosis is surprising, unpredicted or even neglected. In this article we thereby analyze the prevalence, as well as the pathogenesis, of thrombosis associated with apparently paradoxical triggers such as during factor replacement therapy in haemophiliacs or in patients with von Willebrand disease; in patients with inherited clotting factor deficiencies especially involving factor XII, factor VII, fibrinogen; or in those with a prolonged activated partial thromboplastin time for the presence of lupus anticoagulant.
...
PMID:Paradoxical thrombosis part 1: factor replacement therapy, inherited clotting factor deficiencies and prolonged APTT. 2264 22

Patients suffering from hemorrhagic disorders often present with only minimal bleeding during surgery or injuries. However, some patients have life-threatening bleeding. Simple screening tests can be used to find the cause of the bleeding: patient and family histories provide information on whether the bleeding tendency is hereditary or acquired. Clinical examination can reveal the bleeding type. Measurement of platelet count can be used to exclude thrombocytopenia. Coagulation tests, such as prothrombin time (PT, Quick) and activated partial thromboplastin time (aPTT) can supply initial information concerning deficiency states of coagulation factors. Bleeding time is often prolonged in patients suffering from von Willebrand disease, thrombocytopenia or thrombocytopathy. If--due to the results of these screening tests-further testing of particular coagulation factors or platelet function is needed, then patients should be referred to a centre specialized in blood coagulation.
...
PMID:[Hemorrhagic disorders]. 2271 59

Bleeding disorder panels often include the prothrombin time (PT)/international normalized ratio (INR), activated partial thromboplastin time (APTT), fibrinogen level, and thrombin time (TT). We explored the detection of abnormalities from bleeding disorders by these tests among subjects referred for bleeding disorder assessments, using data from a bleeding disorder study to determine sensitivities and specificities. Among subjects referred to hematologists for bleeding disorder assessment, coagulation defects were uncommon and the APTT and TT detected many nonsignificant abnormalities. While all test and panel specificities were acceptable (88 to 100%), coagulation screening tests were less sensitive to clinically significant abnormalities (1.0 to 2.1%) than von Willebrand disease (VWD) screens (6.7%), and light transmission platelet aggregometry (LTA) (26%). Accordingly, panels comprising PT/INR, APTT, fibrinogen, and TT had lower sensitivity to bleeding disorders (3.7%) than panels expanded to include VWD screens (8.5%), or VWD screens and LTA (30%). These findings have important implications for bleeding disorder diagnosis.
...
PMID:Laboratory investigations for bleeding disorders. 2301 92

Global coagulation assays display variable effects at different concentrations of rivaroxaban. The aim of this study is to quantify the ex vivo effects of low-dose rivaroxaban on thrombophilia screening assays and coagulation factor activities based on the administration time, and to show how to mask possible interferences. Plasma samples from 40 patients receiving rivaroxaban 10 mg daily were investigated to measure activities of clotting factor II, V, VII, VIII, IX, XI, XII and XIII; protein C- and protein S-levels; lupus anticoagulants; anticardiolipin IgG and IgM; D-dimer, heparin-platelet factor 4 (HPF4) antibodies and screening tests for von Willebrand disease (VWD). Two hours after rivaroxaban administration, the activities of clotting factors were significantly decreased to different extents, except for factor XIII. Dilution of plasma samples resulted in neutralisation of these interferences. The chromogenic protein C activity assay was not affected by rivaroxaban. Depending on the timing of tablet intake in relation to blood sampling protein S activity was measured falsely high when a clotting assay was used. False-positive results for lupus anticoagulants were observed depending on the assay system used and the administration time of rivaroxaban. ELISA-based assays such as anticardiolipin IgG and IgM, D-dimer, HPF4-antibodies and the turbidimetric assays for VWD were not affected by rivaroxaban. Specific haemostasis clotting tests should be performed directly prior to rivaroxaban intake. Assay optimisation in the presence of rivaroxaban can be achieved by plasma dilution. Immunologic assays are not influenced by rivaroxaban, while chromogenic assays can be used, when they do not depend on factor Xa.
...
PMID:Ex vivo effects of low-dose rivaroxaban on specific coagulation assays and coagulation factor activities in patients under real life conditions. 2313 90

Menometrorrhagia is a common symptom in adolescents. It is idiopathic in most cases. In case of menometrorrhagia, it is necessary to exclude a pregnancy, a disorder of hemostasis, particularly the von Willebrand disease, as it represents the most common inherited disorder, and more rarely a chronic disease or an endocrinopathy. History of the bleedings, menstrual blood loss quantification by the Higham score and tolerance of the bleedings (blood pressure) should be evaluated. Laboratory testing includes hCG, ferritin level, a complete blood count, a prothrombin time, an activated partial thromboplastin. Management of menometrorrhagia is related to the severity of the blood loss. It associates antifibrinolytics or non-steroidal anti-inflammatory agents (NSAIDS) with hormonal treatments, such as estrogen-progestin oral contraceptive pill or cyclic oral progestins. Primary or functional dysmenorrhea concerns 40 to 90% of the teenagers and represents a frequent cause of school absenteeism. Management of primary dysmenorrhea is primarily based on a treatment by NSAIDS. In case of its inefficacy or if contraception is needed hormonal treatments, such as estrogen-progestin combined pill should be prescribed. It is very important when pelvic pain is chronic and not soothed by simple medications to look for a secondary dysmenorrhea, mainly endometriosis. In such cases, pelvic magnetic resonance imaging should be performed.
...
PMID:[Meno-metrorrhagia, dysmenorrhea in adolescents]. 2372 73

Menorrhagia is one of the gynecological complaints, seen in women of reproductive age. In majority of cases no organic pathology is found. To date there is no consensus on application of von Willebrand disease (vWD) testing as part of the routine investigations in menorrhagia. Diagnosis of vWD is challenging. It is complicated by intra-individual variations in von Willebrand antigen, activity, and factor VIII levels due to fluctuation of these factor levels during the menstrual cycle or hormonal therapy. The aim of this study is to detect vWD presenting with menorrhagia among Malays attending gynecology clinic by using a standard panel of haemostatic profiles. Thirty Malay patients attending gynecology clinic with unexplained menorrhagia were included in this study. Haemostatic profile such as platelet count, prothrombin time, activated partial thromboplastin time (APTT), factor VIII assay, von Willebrand factor antigen, and von Willebrand factor activity, and collagen binding assay were measured in all patients. Pre- and post hormonal haemostatic profiles were also performed in the patients diagnosed as vWD. All patients had normal APTT. Based on von Willebrand factor work-up, vWD was diagnosed in four patients (13.3%). Three of them were Type 1 and the other one was Type 2M. Investigation for vWD is essential in patients with menorrhagia and thus the laboratories performing vWD testing should provide a complete panel of diagnostic work-up in order to reduce the interpretation error. Screening for vWD should be performed before hormonal treatment as haemostatic profile post treatment could mask the diagnosis.
...
PMID:Single-Center Experience of von Willebrand Disease (vWD) Among Patients with Menorrhagia: A Diagnosis which could be Missed. 2399 52


<< Previous 1 2 3 4 5 6 7 8 9 10

---