Constitutive hypercoagulability in pediatric sickle cell disease patients with hemoglobin SS genotype
CONCLUSION: Our results indicate that pediatric SCD patients with the HbSS genotype have constitutively activated hemostasis relative to HbSC patients and healthy children. It remains to be determined how treatments that improve clinical outcomes in SCD patients affect this constitutively hypercoagulable state.PMID:38605827 | PMC:PMC11004888 | DOI:10.1016/j.rpth.2024.102374 (Source: Thrombosis and Haemostasis)
Source: Thrombosis and Haemostasis - April 12, 2024 Category: Hematology Authors: Raizl G Sussman Joy Mburu MacGregor Steele Annie Bang Jeremy Friedman Ran Goldman Melanie Kirby Margaret L Rand Victor S Blanchette Fred G Pluthero Suzan Williams Walter H A Kahr Source Type: research

Bile Acid –Targeted Hyaluronic Acid Nanoparticles for Enhanced Oral Absorption of Deferoxamine
AbstractPatients with β-thalassemia and sickle cell disease often rely on blood transfusions which can lead to hemochromatosis and chronic oxidative stress in cells and tissues. Deferoxamine (DFO) is clinically approved to treat hemochromatosis but is suboptimal to patients due to its poor pharmacokinetics which require s long-term infusion regimens. Although the oral route is preferable, DFO has limited oral bioavailability. Studies have shown that hyaluronic acid (HA) and bile acid (BA) can enhance the oral absorption of poorly absorbed drugs. To improve upon the oral delivery of DFO, we report on the synthesis a nd cha...
Source: The AAPS Journal - April 12, 2024 Category: Drugs & Pharmacology Source Type: research

Prevalence and predictors of iron deficiency anaemia among children with sickle cell disease in Dodoma, Tanzania: a cross-sectional study
This study aimed to determine the prevalence of iron deficiency anaemia (IDA) and their predictors among children with SCD aged b... (Source: BMC Public Health)
Source: BMC Public Health - April 12, 2024 Category: Consumer Health News Authors: Asha O. Bossy, James J. Yahaya and Shakilu Jumanne Tags: Research Source Type: research

Assessing Patterns of Telehealth Use Among People with Sickle Cell Disease Enrolled in Medicaid During the Start of the COVID-19 Pandemic
Telemedicine and e-Health, Ahead of Print. (Source: Telemedicine and e-Health)
Source: Telemedicine and e-Health - April 11, 2024 Category: Information Technology Authors: Sarah L. Reeves Melissa Plegue Pooja N. Patel Susan T. Paulukonis Sophia S. Horiuchi Mei Zhou Brandon K. Attell Betty S. Pace Angela B. Snyder Allison P. Plaxco Ayesha Mukhopadhyay Matthew P. Smeltzer Chandy S. Ellimoottil Mary Hulihan Source Type: research

In-depth structure-function profiling of the complex formation between clotting factor VIII and heme
Blood disorders, such as sickle cell disease, and other clinical conditions are often accompanied by intravascular hemolytic events along with the development of severe coagulopathies. Hemolysis, in turn, leads to the accumulation of Fe(II/III)-protoporphyrin IX (heme) in the intravascular compartment, which can trigger a variety of proinflammatory and prothrombotic reactions. As such, heme binding to the blood coagulation proteins factor VIII (FVIII), fibrinogen, and activated protein C with functional consequences has been demonstrated earlier. (Source: Thrombosis Research)
Source: Thrombosis Research - April 11, 2024 Category: Hematology Authors: Marie-T. Hopp, Deniz Ugurlar, Behnaz Pezeshkpoor, Arijit Biswas, Anuradha Ramoji, Ute Neugebauer, Johannes Oldenburg, Diana Imhof Tags: Full Length Article Source Type: research

TSH Receptor Reduces Hemoglobin S Polymerization and Increases Deformability and Adhesion of Sickle Erythrocytes
Anemia. 2024 Apr 2;2024:7924015. doi: 10.1155/2024/7924015. eCollection 2024.ABSTRACTSCD is a hereditary disorder caused by genetic mutation in the beta-globin gene, resulting in abnormal hemoglobin, HbS that forms sickle-shaped erythrocytes under hypoxia. Patients with SCD have endocrine disorders and it was described that 7% of these patients have clinical hypothyroidism. Recent studies have shown that mature erythrocytes possess TSH receptors. Thus, we aimed to assess the effects of TSH on SCD erythrocytes. The experiments were conducted using different concentrations of TSH (1, 2, 3, and 5 mIU/L). In HbS polymerization...
Source: Anemia - April 10, 2024 Category: Hematology Authors: Evelyn Mendon ça-Reis Camila Cristina Guimar ães-Nobre Lyzes Rosa Teixeira-Alves Leandro Miranda-Alves Clemilson Berto-Junior Source Type: research