This manuscript presents many important clinical conditions that are uncommon and may go undetected by medical personnel. [pubmed.ncbi.nlm.nih.gov]

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]

Patients with congenital deficiency of alpha 2-antiplasmin may present with a severe hemorrhagic disorder due to impaired inactivation of plasmin and resulting premature lysis of the hemostatic fibrin plug. [rarecoagulationdisorders.org]

Case presentation An 11-year-old Sudanese boy presented with frequent bleeding diathesis (ecchymosis, epistaxis, and gingival bleeds) (Fig. 2). [jmedicalcasereports.biomedcentral.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]

In addition to routine coagulation studies, the workup for patients with possible alpha2-PI deficiency includes antigenic and functional alpha2-PI assays, which are performed in specialized laboratories (see Workup). [emedicine.medscape.com]

[…] 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]

Paving the way in PLGD treatment and research The IHTC has been a pioneer in the development of treatment and clinical studies for patients with plasminogen deficiency (PLGD). [ihtc.org]

Data of all reported cases of congenital alpha-2 antiplasmin deficiency were extracted and summarized for study setting, patient characteristics, and types of treatments. [pubmed.ncbi.nlm.nih.gov]

[…] 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]

[…] 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]

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]

Alpha 2-Antiplasmin Deficiency Disease Overview Background Etiology Epidemiology The congenital deficiency of alpha 2-antiplasmin (also referred to as alpha 2-plasmin inhibitor) was first described by Masateru Kohakura in 1969 in a child living on the [rarecoagulationdisorders.org]

They are organised into groups, and further divided into clinical, etiological or histopathological sub-types. [orpha.net]

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]

Alpha 2-Antiplasmin Deficiency Disease Overview Background Etiology Epidemiology The congenital deficiency of alpha 2-antiplasmin (also referred to as alpha 2-plasmin inhibitor) was first described by Masateru Kohakura in 1969 in a child living on the [rarecoagulationdisorders.org]

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]

[…] joints, similar to the manifestations seen in patients with hemophilia. [2] Gene knockout mouse models of alpha2-PI deficiency show the expected accelerated clot lysis, but the mice do not manifest the bleeding disorder that is seen in humans. [15] Epidemiology [emedicine.medscape.com]

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]

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]