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Hamin Pazhuhan Tis Institute[Affiliation] Publications | LitMetric

11 results match your criteria: "Hamin Pazhuhan Tis Institute[Affiliation]"

Rare bleeding disorders (RBDs) represent 3 to 5% of congenital bleeding disorders and are primarily inherited in an autosomal recessive manner, with increased prevalence in consanguineous populations. Clinically, RBDs can be accompanied by mild to severe bleeding episodes, often assessed using bleeding assessment tools (BATs) such as the International Society on Thrombosis and Hemostasis (ISTH)-BAT. However, the correlation between bleeding severity and coagulation factor activity levels remains inconsistent.

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Precision Medicine in Rare Bleeding Disorders.

Semin Thromb Hemost

December 2024

Department of Biomedical and Molecular Sciences, School of Medicine, Queen's University, Kingston, Ontario, Canada.

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Article Synopsis
  • - FXIII-A deficiency is a rare bleeding disorder linked to serious complications like brain hemorrhages and miscarriages, with a higher prevalence of a milder form (heterozygous deficiency) found in up to 3.5% of the population.
  • - Individuals with severe FXIII-A deficiency require consistent preventative treatment, while heterozygous individuals, especially women, may only need treatment during specific bleeding events.
  • - Personalized treatment strategies based on individual patient factors (e.g., age, weight, and clinical situation) are essential for optimizing the effectiveness of therapy for FXIII deficiencies.
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The History of Rare Bleeding Disorders.

Semin Thromb Hemost

November 2024

Department of Hematology and Blood Banking, School of Allied Medical Sciences, Shahid Beheshti University of Medical Sciences, Tehran, Iran.

Deficiencies in coagulation factors I (FI), FII, FV, combined FV and FVIII (CF5F8) and vitamin K-dependent coagulation factors FVII, FX, FXI, and FXIII have been referred to as rare bleeding disorders (RBDs), rare coagulation factor deficiencies (RCFDs), or recessively inherited coagulation disorders. Fibrinogen was most likely the first member of this group to be identified, with reports of its discovery spanning from 1859 to 1966. If not, then the first coagulation factor to be identified was prothrombin in 1894, and the last coagulation factor to be found was FX in 1956, about 60 years later.

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Article Synopsis
  • * The development of CAT is influenced by multiple factors, including the type of cancer, genetic predisposition, and elevated levels of coagulation factors.
  • * Various studies have explored the relationship between inherited thrombophilia (like prothrombin 20210 and factor V Leiden) and CAT, showing mixed results regarding their correlation.
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Article Synopsis
  • β-thalassemia major (β-TM) is a genetic blood disorder that leads to low production of β-globin chains, increasing the risk of complications like thrombosis in patients, especially those getting frequent blood transfusions.
  • * The study involved 118 β-TM patients who had received at least 20 blood transfusions and 120 healthy controls, measuring levels of natural anticoagulants.
  • * Results showed significantly lower levels of protein C and antithrombin in β-TM patients, suggesting that they are at a higher risk for thrombosis due to reduced levels of these anticoagulants.
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The prothrombin time (PT) test is commonly used to monitor deficiencies in coagulation factors. A prolonged PT may indicate a deficiency of factors II, V, VII, X, and fibrinogen, or the presence of an inhibitor. However, further tests are required to differentiate between a true factor deficiency and the presence of an inhibitor.

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COVID-19 vaccine-induced immune thrombotic thrombocytopenia: pathophysiology and diagnosis.

Ann Hematol

November 2023

Department of Medical Laboratory Sciences, School of Allied Medical Sciences, Arak University of Medical Sciences, Arak, Iran.

Coronavirus disease-19 (COVID-19) vaccine-induced immune thrombotic thrombocytopenia (VITT) is a rare but serious clinical condition with high mortality rate in apparently healthy individuals without noticeable risk factors. VITT typically arises due to the administration of vaccines that possess recombinant adenoviral vectors, including ChAdOx1 nCov-19 (AstraZeneca) and Ad26 COV2.S (Johnson & Johnson/Janssen).

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The clinical laboratory uses commercial products with limited shelf life or certain expiry dates requiring frequent lot changes. Prior to implementation for clinical use, laboratories should determine the performance of the new reagent lot to ensure that there is no significant shift in reagent performance or reporting of patient data. This guideline has been written on behalf of the International Council for Standardization in Haematology (ICSH) to provide the framework and provisional guidance for clinical laboratories for evaluating and verifying the performance of new lot reagents used for coagulation testing.

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Hypercoagulability is a prominent feature of coronavirus disease 2019 (COVID-19) and can lead to fatal consequences. Although the impact of COVID-19 on several disorders is well-established, its effect on congenital bleeding disorders (CBDs) is not well-documented. To address this ambiguity, a systematic review was conducted on the available studies to determine the impact of COVID-19 and vaccination aimed to prevent COVID-19 on patients with CBDs.

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