Publications by authors named "R Bou-Fakhredin"
Article Synopsis
- Thalassaemia stems from over 250 mutations in the beta globin gene, impacting hematopoietic stem cell differentiation and causing ineffective red blood cell production.
- The traditional focus on managing symptoms with transfusions and iron chelation therapy has hindered progress toward developing cell-based treatments, despite advancements in understanding the disease since the identification of the beta039 mutation in 1979.
- Recent progress in treating hematopoietic stem cell disorders emphasizes a 'target cell strategy,' suggesting a shift toward innovative treatments for thalassaemia that identify suitable candidates through risk stratification, highlighting its nature as a congenital HSC disorder.
Article Synopsis
- β-thalassemia and sickle cell disease are linked to a hypercoagulable state, increasing the risk of thrombosis and organ complications.
- Factors like free heme, inflammation, and splenectomy, alongside the role of red blood cells, contribute to this thrombotic risk.
- Understanding these mechanisms can help improve prevention and treatment strategies for managing thrombosis in patients with these blood disorders.
Iron is an essential nutrient and a constituent of ferroproteins and enzymes crucial for human life. Generally, nonmenstruating individuals preserve iron very efficiently, losing less than 0.1% of their body iron content each day, an amount that is replaced through dietary iron absorption.
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