Results: 44

Consensus of the Brazilian Association of Hematology, Hemotherapy and Cellular Therapy (ABHH) and the Brazilian Ministry of Health - General management of blood and blood products on the tests necessary for the release of exceptional medicines for sickle cell disease

Abstract To date, hydroxyurea is the only effective and safe drug that significantly reduces morbidity and mortality of individuals with Sickle cell disease. Twenty years of real-life experience has demonstrated that hydroxyurea reduces pain attacks, vaso-occlusive events, including acute chest syndrome,...

Sickle cell anemia: hierarchical cluster analysis and clinical profile in a cohort in Brazil

Abstract Introduction Sickle cell anemia is a monogenic disorder caused by a mutation in the β-hemoglobin gene, resulting in sickle hemoglobin that can polymerize. Presentation and clinical course have significant inter-individual variability and classifying these patients for severity is a challen...

Hiperpigmentação Mucocutânea Associada à Terapia de Hidroxiureia em Paciente com Trombocitemia Essencial: Relato de Caso
Mucocutaneous Hyperpigmentation Associated with Hydroxyurea Therapy in a Patient with Essential Thrombocythemia: Case Report

Introdução: A hiperpigmentação mucocutânea é uma condição dermatológica que pode estar relacionada a tratamentos quimioterápicos, a exemplo das terapias com uso de hidroxiureia (HU). A HU é um fármaco citostático de amplo uso nas doenças mieloproliferativas e compõe a principal linha de tr...

Follow-up of children with sickle cell anemia screened with transcranial Doppler and enrolled in a primary prevention program of ischemic stroke

ABSTRACT Background: Stroke is a serious complication of sickle cell anemia (SCA). The transcranial Doppler (TCD) is the risk-screening tool for ischemic strokes. The objective of the study was to describe the clinical progression of children with SCA who presented with high risk for stroke by TCD or re...

Guidelines on sickle cell disease: secondary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular guidelines project: Associação Médica Brasileira - 2022

Guidelines on sickle cell disease: primary stroke prevention in children and adolescents. Associação Brasileira de Hematologia, Hemoterapia e Terapia Celular Guidelines project: Associação Médica Brasileira - 2021

Effects of hydroxyurea on cytotoxicity, inflammation and oxidative stress markers in neutrophils of patients with sickle cell anemia: dose-effect relationship

ABSTRACT Introduction: Although the efficacy of hydroxyurea (HU) in inhibiting erythrocyte sickling has been well demonstrated, the action of this drug on human neutrophils and the mechanism by which it improves the manifestations of the disease have not been studied thoroughly. We aimed to investigate ...

Recambio de Hematíes en gestante con Anemia de Células Falciformes: reporte de un caso en el Hospital Regional de Talca

Rev. méd. Maule; 36 (2), 2021
Sickle cell anemia or sickle cell disease is an autosomal recessive disease, caused by a mutation in the hemoglobin gene, where glutamic acid is substituted for valine at position 6 of the beta chain of hemoglobin, resulting in hemoglobin S The diagnosis is made with electrophoresis. The clinical manifes...

Anemia falciforme: una revisión sobre el genotipo de la enfermedad, haplotipos, diagnóstico y estudios asociados

Rev. méd. Chile; 149 (9), 2021
Sickle cell anemia is a type of hemoglobinopathy characterized by a specific mutation in the beta globin gene with the consequent generation of an unstable hemoglobin that crystallizes in a state of hypoxia. This causes a change in the structure of the red blood cell, which ends up producing vaso-occlusi...