Presentation
This present study brings the frequency of heterozygous alpha 2-AP deficient patients with a bleeding tendency to 13 out of 59 heterozygotes reported in the literature. [unboundmedicine.com]
Heterozygotes usually do not present with bleeding disorders Homozygotes may present with moderate to severe bleeding Bleeding is usually posttraumatic rather than spontaneous Thrombotic risk Elevated levels of PAI-1 may be associated with venous or [arupconsult.com]
With her first pregnancy, she presented at 10-week gestation with recurrent nephrolithiasis and hematuria. [hindawi.com]
Entire Body System
- Non-Alcoholic Cirrhosis
Also, raised levels have been described in acute viral hepatitis, alcoholic cirrhosis and non-alcoholic cirrhosis [17] FVIII assays are of little help clinically for evaluating the coagulation defect in liver disease. [saudijgastro.com]
Musculoskeletal
- Short Arm
The gene for α 2 -PI [SERPINF2] maps to the short arm of chromosome 17 [17pter-p12] and contains 10 exons and 9 introns. The gene encodes a protein of 51kDa which is synthesised in the liver and circulates either free or bound to plasminogen. [practical-haemostasis.com]
Urogenital
- Uterine Hemorrhage
Uterine Hemorrhage Bleeding from blood vessels in the UTERUS, sometimes manifested as vaginal bleeding. [bioportfolio.com]
Workup
[…] may be necessary to definitively diagnose severe PAI-1 deficiency Plasminogen – activity testing Alpha-2-antiplasmin – activity testing tPA – antigen testing Genotyping (SERPINE1) Consider in patient with recurrent deep vein thrombosis and negative workup [arupconsult.com]
Differential Diagnoses Hemophilia A and B Pediatric Von Willebrand Disease Platelet dysfunction Other clotting factor deficiencies Uncommon coagulopathies Diagnostic Workup Laboratory studies for suspected hemophilia C should include the following: Complete [medcaretips.com]
Treatment
[…] of Venous Thromboembolic Disease Overview of Arterial Thrombotic Disorders Pathophysiology of Atherothrombosis Prevention of Thromboembolic Stroke in Patients with Acute Thrombolytic Therapy Unusual Sites of Arterial Occlusion Pathogenesis and Treatment [books.google.com]
Treatment with epsilon aminocaproic acid was associated with diminished bleeding and marked increase in the plasmin inhibitor level. [unboundmedicine.com]
Food and Drug Administration has chosen Wave Life Sciences’ planned phase 2/3 study of suvodirsen, its lead experimental therapy for the treatment of Duchenne muscular dystrophy, to break new ground. [globalgenes.org]
Abstract Patients with acute promyelocytic leukemia often develop bleeding diatheses during treatment. [annals.org]
Prognosis
[…] if ALK -) cutaneous T cell lymphoma CD4+ convoluted "cerebriform" lymphocytes advanced stage of mycosis fungicides malignant T cell (sezary cells) seed peripheral blood poor prognosis, rare erythroderma (beet red skin) small number of neoplastic cells [quizlet.com]
Low levels have been found in fulminant hepatic failure and a value of less than 20% is associated with poor prognosis [17]. However, FV may be raised in patients with acute infection. [saudijgastro.com]
hemolysis (high bilirubin, LDH, low haptoglobin), schistocytes); thrombocytopenia; renal failure (high BUN, creatine); stool culture - Stx gene detected by PCR Rx: supportive, RBC txfusions for anemia, NO platelet txfusions, dialysis, eculizumab Prognosis [flashcardmachine.com]
This is particularly true in cases of intracranial hemorrhage which represent a serious anticoagulant-related bleeding complication, even though there is no solid evidence from literature of an improvement in prognosis [ 116, 117 ]. [aneskey.com]
The prognosis of patients with reductions in antithrombin as part of other systemic disorders depends on the underlying disorder. The frequency of arterial thrombotic complications is low in patients with antithrombin deficiency. [emedicine.medscape.com]
Etiology
This practical text covers disorders of thrombosis and hemostasis in a logical and sequential manner: etiology, pathophysiology, clinical and laboratory diagnosis, and management. [books.google.com]
They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]
Increased destruction and/or decreased production may be etiological. [slideplayer.com]
Epidemiology
Management of Acute Hemorrhage Platelets Plasma and Specialized Coagulation Concentrate New Approaches for the Therapy of Bleeding Disorders Overview of Venous Thromboembolism Thrombophilia Genetics Epidemiology and Risk Factors for Venous Clinical Manifestations [books.google.com]
[…] platelet disorder Synonym(s): (no synonyms) Classification (Orphanet): - Rare genetic disease - Rare hematologic disease Classification (ICD10): - Diseases of the blood and blood-forming organs and certain disorders involving the immune mechanism - Epidemiological [csbg.cnb.csic.es]
Relevant External Links for SERPINF2 Genetic Association Database (GAD) SERPINF2 Human Genome Epidemiology (HuGE) Navigator SERPINF2 Atlas of Genetics and Cytogenetics in Oncology and Haematology: SERPINF2 No data available for Genatlas for SERPINF2 Gene [genecards.org]
Epidemiology of obstructive sleep apnea. A population health perspective. Am J Respir Crit Care Med. 2002; 165: 1217–1239. pmid:11991871 View Article PubMed/NCBI Google Scholar 7. [journals.plos.org]
Pathophysiology
This monograph owes its conception to a programme of work directed towards understanding the basic pathophysiology of atherosclerosis. [books.google.com]
SERPINE1) Consider in patient with recurrent deep vein thrombosis and negative workup for common defects Differential Diagnosis Factor deficiency Factor VIII or IX deficiency most common von Willebrand disease Functional platelet disorder Background Pathophysiology [arupconsult.com]
Semin Thromb Hemost 35(4):400–406 PubMed CrossRef Google Scholar Meeks SL, Abshire TC (2008) Abnormalities of prothrombin: a review of the pathophysiology, diagnosis, and treatment. [link.springer.com]
Prevention
Inherited deficiency of α 2 -antiplasmin results in tendency to severe bleeding, including extravasation into joints or their synovial cavities. an·ti·plas·min ( an'tē-plaz'min ), A substance that inhibits or prevents the effects of plasmin; found in [medical-dictionary.thefreedictionary.com]
[…] of Venous Thromboembolic Disease Treatment of Venous Thromboembolic Disease Overview of Arterial Thrombotic Disorders Pathophysiology of Atherothrombosis Prevention of Thromboembolic Stroke in Patients with Acute Thrombolytic Therapy Unusual Sites of [books.google.com]
In this way α 2 -PI prevents plasminogen binding to fibrin.. The gene for α 2 -PI [SERPINF2] maps to the short arm of chromosome 17 [17pter-p12] and contains 10 exons and 9 introns. [practical-haemostasis.com]