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Jul19
SICKLE CELL DISEASE & BUDD-CHIARI SYNDROME
The Budd-chiari syndrome is a heterogeneous group of disorders characterized by hepatic venous outflow obstruction at the level of the hepatic venules, the large hepatic veins, the IVC or the right atrium(1). Hyper coagulable states of the blood are the most common etiology of this disorder(2); amongst which sickle cell disease is a rare cause(2). This part of Orissa is having high incidence of SCD and trait. No such case report has been done earlier.
Sickle cell disease is a type of Hemoglobinopathy characterized by production of abnormal sickle Hemoglobin (Hbs). This abnormality is due to the substitution of valine for glutamic acid at the 6th position of  chain of globin(34). The abnormal HbS tends to polymerize on deoxygenation and the RBCs containing HbS become less pliable and consequently deform into the characteristic sickle shape, for which the disease is named so. This disorder is acquired by inheriting abnormal sickle genes from both the parents.


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