Sickle cell anemia is characterized by chronic hemolysis coupled with extensive vascular inflammation. This inflammatory state also mechanistically promotes a high risk of lethal, invasive pneumococcal infection. Current treatments to reduce vaso-occlusive complications include chronic hydroxyurea therapy to induce fetal hemoglobin. Because hydroxyurea also reduces leukocytosis, an understandin...

Hemoglobin SC disease is a very prevalent hemoglobinopathy; however, very little is known about this condition specifically. There appears to be an increased risk of thromboembolic events in hemoglobin SC disease, but studies evaluating the hemostatic alterations are lacking. We describe the findings of a cross-sectional observational study evaluating coagulation activation markers in adult pat...

OBJECTIVE Sickle cell anemia and the interaction S/Beta thalassemia differ in hematological values due to microcytosis and hypochromia caused by the thalassemic mutation. The clinical benefit of long-term hydroxyurea treatment is undeniable in sickle cell disease with monitoring of the biological action of the drug being by the complete blood count. The objective of this work is to compare chan...

UNLABELLED Sickle cell anemia is still a significant public health issue in underdeveloped and developing countries. Sickle cell disease is one of the most common inherited diseases in Brazil. It affects mainly the mixed race population. Approximately 1 African-Brazilian child is affected with sickle cell disease for every 37,400 children born alive. Hearing loss has been considered one of the ...

Sickle cell disease (SCD), an inherited hemoglobinopathy that causes anemia, severe pain, vaso-occlusive crisis (VOC), is currently recognized as a global public health concern, being the leading cause of pediatric stroke. Our aim was to synthesize evidence on efficacy and safety interventions for managing SCD in this population.

Herrick [1] was the first to discover sickle cell hemoglobin ( 2 2) with sickle-shaped erythrocytes. In 1910, he described the case of a young black student from the West Indies with severe anemia characterized by “peculiar elongated and sickle-shaped red blood corpuscles.” Herrick also noted a slightly increased volume of urine of low specific gravity and thus observed the most frequent featur...

Aim: To identify scientific evidence about families of children with sickle cell disease. Method: An integrative review of the search used the following descriptors: anemia sickle cell; hemoglobin SC disease; hemoglobin sickle; family; family relations; and, child. The search was carried out for literature in Portuguese, English, and Spanish. The search used databases such as MEDLINE, LILACS, C...

Background: Treatment of sickle cell anemia is a challenging task and despite the well understood genetic and biochemical pathway of sickle hemoglobin, current therapy continues to be limited to the symptomatic treatment of pain, supplemental oxygen, antibiotics, red blood cell transfusions and hydroxyurea. SANGUINATE is a carbon monoxide releasing molecule and oxygen transfer agent under clini...

Introduction: Our study focused on the evaluation of renal function in children with sickle cell disease compared to without at pediatric emergency unit Donka National Hospital and SOS Drepano-Guinea center. Patients Methods: This was a cross-sectional descriptive analytical lasting 3 months (October 1 December 31, 2020). Were included, all non-sickle aged 0 15 received an outpatient basis had ...