Hypoxia and hemoglobin S (HbSS) polymerization are the two cardinal triggers responsible for both erythrocyte sickling as well as of all clinical events in sickle cell anemia (SCA), a disease with many pathophysiological and molecular aspects that remain unclear. Despite being a permanent feature of SCA, there is still no consensus on the definition or management of hypoxia in sickle cell patients, and studies ...
A hidroxiuréia (HU) constitui o avanço mais importante no tratamento da anemia falciforme (AF) por prevenir complicações e aumentar a qualidade de vida dos pacientes. Entretanto, alguns aspectos do tratamento com HU permanecem obscuros, incluindo a sua ação e potencial toxicidade em outras células sanguíneas, tais como neutrófilos. Este estudo utilizou a mensuração da lactato desidrogenase (LDH) e do metil tiaz...
Falciform Anemia (FA) is a hereditary hemoglobinopathy resulting from a β-globin gene mutation (α2β26 GLU→ VAL) that originates a hemoglobin variant called S (HbS). Its polymerization promotes hemolytic and vaso-occlusive crises (VOC). Nowadays it is known that these reactions are initial FA events that unleash a chain reaction that ends with the generation of oxygen reactive types (ORT) nitric oxide (NO) bioav...
Background: Sickle cell disease is a hemoglobinopathy characterized by hemolytic anemia, increased susceptibility to infections and recurrent vaso-occlusive crises that reduces the quality of life of sufferers. Objective: To evaluate the correlation of the levels of lactate dehydrogenase, malonaldehyde and nitrite to fetal hemoglobin in patients with sickle cell disease not under treatment with hydroxyurea in o...