Sickle cell disease treatment

Bunn HF Pathogenesis and treatment of sickle cell disease. N Engl J Med 1997 337 762. 

Manning JM et aJ Normal and abnormal protein subunit interactions in hemoglobins.] Biol Chem 1998 273 19359-Mario N, Baudin B, Giboudeau J Qualitative and quantitative analysis of hemoglobin variants by capillary isoelectric focusing. J Chromatogr B Biomed Sci Appl 1998 706 123-Reed W, Vichinsky EP New considerations in the treatment of sickle cell disease. Annu Rev Med 1998 49 46l. 

Rosse WF et al New Views of Sickle Cell Disease Pathophysiology and Treatment. The American Society of Hematology. www.asheducationbook.org... 

Steinberg MH. Pathophysioiogically based drug treatment of sickle cell disease. Trends Pharmacol Sci 2006 27(4) 204-210. 

Hydroxy urea (HU), a hydroxylated derivative of urea, has long been used in the treatment of various forms of neoplastic disorders such as polycythemia vera, head and neck cancer, chronic granulocytic leukemia, and more recently in sickle cell disease, / thalassemia and HIV infection [1],... 

In conclusion, the use of specific endothelin antagonists must be considered in the treatment of sickle cell disease as an alternative to HU, whose potential longterm mutagenic/carcinogenic effect is still under debate [36], especially in children. 

Congress thus may constitutionally fund sickle-cell anemia treatment centers across the country, even though 97% of persons with the disease are African American (Lee, 2001). Because this is a disease-, not race-, specific... 

Another Allosteric Effector for Treatment of Sickle-cell Disease... 

ICA -17043 is a novel inhibitor of the Gardos channel (a calcium-dependent potassium channel). This drug is indicated for the treatment of a hematologic genetic disease (sickle cell disease) (Phase 11) (Figure 8.88). 

Unlabeled Uses Prevention of postoperative deep vein thrombosis (DVT), protection of aortocoronary bypass grafts, reduction of graft loss after renal transplant, treatment of intermittent claudication, sickle cell disease, subarachnoid hemorrhage, diabetic microangiopathy, ischemic heart disease... 

Hydroxyurea. - Hydroxyurea, H2NC(0)NH0H, is used in the treatment sickle cell disease and myeloproliferative disorders. The interaction of HU with Hb-Fe(Il)-02 results in the production of Hb-Fe(II)-NO, Hb-Fe(III) and NOu. The anti-proliferative action of HU results from its inhibition of ribonucleotide reducase (a key enzyme in DNA synthesis), which is believed to involve the reaction of NO with a tyrosyl radical formed during the reaction cycle. King et... 

A dominant theme in the treatment of sickle cell disease is the manipulation of the hemoglobin switch from fetal to adult hemoglobin. If the switch did not occur at all or if the y-globin gene did not completely switch to P-globin... 

De Franceschi L, Corrocher R Established and experimental treatments for sickle cell disease. Haematologica 89 348-356, 2004. 

Subbiah P, Wijdicks E, Muenter M et al (1996). Skin lesion with a fatal neurologic outcome (Degos disease). Neurology 46 636-640 Switzer JA, Hess DG, Nichols FT et al. (2006). Pathophysiology and treatment of stroke in sickle-cell disease present and future. Lancet Neurology 5 501-512... 

Sustained elevated lOPs require treatment, as does any elevated lOP associated with hyphema in a patient with sickle cell disease or trait. These patients have a higher incidence of increased lOP (sickled cells do not pass through trabecular meshwork as freely as normal red blood cells), optic atrophy, and secondary hemorrhage in the setting of tramnatic hyphema compared with non-sickle cell patients. 

Favorable results of exchange transfusion in a variety of diseases in adults, for example sickle cell disease, severe clotting disorders, hepatic failure, and acute hemolytic transfusion reactions, have been published (1). Today, however, machine apheresis procedures are more effective and safer for patients requiring exchange of cellular elements or plasma. Exchange transfusion is the most effective therapeutic procedure in the treatment of hemolytic disease of the newborn. Bilirubin removal prevents damage to the central nervous system caused by hyperbilirubinemia. In addition, sensitized erythrocytes are replaced by normally surviving cells and anemia is corrected. 

Treatment of patients with sickle cell anemia has led to disastrous sickling crises (99), and there are at present no clinical data to support the use of erythropoietin in sickle cell disease (100). 

Communicating ideas Learn about the latest advances in sickle cell disease research. Create a chart that shows major symptoms, their causes, and their treatment. Share your findings with other students in your class. 

---